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Sample records for preventing radial spasms

  1. Intra-arterial vasodilators to prevent radial artery spasm: a systematic review and pooled analysis of clinical studies

    Energy Technology Data Exchange (ETDEWEB)

    Kwok, Chun Shing, E-mail: shingkwok@doctors.org.uk [Keele Cardiovascular Research Group, Keele University, Stoke-on-Trent (United Kingdom); Rashid, Muhammad [St. Helens & Knowsley Teaching Hospital (NHS) Trust, Whiston Hospital, Prescot (United Kingdom); Fraser, Doug [Manchester Heart Centre, Manchester Royal Infirmary (United Kingdom); Nolan, James [University Hospital of North Midlands, Stoke-on-Trent (United Kingdom); Mamas, Mamas [Keele Cardiovascular Research Group, Keele University, Stoke-on-Trent (United Kingdom); Farr Institute, Institute of Population Health, University of Manchester, Manchester (United Kingdom)

    2015-12-15

    Objectives: The aim of this study is to review the available literature on the efficacy and safety of agents used for prevention of RAS. Background: Different vasodilator agents have been used to prevent radial artery spasm (RAS) in patients undergoing transradial cardiac catheterization. Methods: We included studies that evaluated any intra-arterial drug administered in the setting cardiac catheterization that was undertaken through the transradial access site (TRA). We also compared studies for secondary outcomes of major bleeding, procedure time, and procedure failure rate in setting of RAS prevention, patent hemostasis and radial artery occlusion. Results: 22 clinical studies met the inclusion criteria. For placebo, RAS rate was 12% (4 studies, 638 participants), which was similar to 2.5 mg of verapamil 12% (3 studies, 768 participants) but greater than 5 mg of verapamil (4%, 2 studies, 497 participants). For nicorandil, there was a much higher RAS rate compared to placebo (16%, 3 studies, 447 participants). The lowest rates of RAS was found for nitroglycerin at both 100 μg (4%) and 200 μg (2%) doses, isosorbide mononitrate (4%) and nicardipine (3%). We found no information regarding the procedure failure rates, patent hemostasis, and radial artery occlusion in these studies. Conclusions: In this largest and up-to-date review on intra-arterial vasodilators use to reduce RAS, we have found that the verapamil at a dose of 5 mg or verapamil in combination with nitroglycerine are the best combinations to reduce RAS. - Highlights: • Radial artery spasm (RAS) causes procedural failure in transradial catheterization. • RAS may complicate 10–15% procedures undertaken through the radial approach. • We reviewed the efficacy of vasodilators that have been used to minimize RAS. • The pooled RAS rate was lowest with 5 mg of verapamil (4%) compared to placebo (12%). • The best combination of drugs to minimize RAS is nitroglycerine and verapamil.

  2. Balloon-Assisted Tracking to Overcome Radial Spasm during Transradial Coronary Angiography: A Case Report

    NARCIS (Netherlands)

    Verouden, N. J. W.; Kiemeneij, F.

    2014-01-01

    Spasm of the radial artery is the most important cause of failure to perform coronary angiography via the transradial approach. Spasmolytic cocktail may prevent radial artery spasm but is relatively contraindicated in patients with aortic stenosis or diminished left ventricular function. In this

  3. PROLONGED RADIAL ARTERY SPASM IN THE CATHETERIZATION LABORATORY - RELIEF BY PHARMACOLOGICAL INTERVENTION

    Directory of Open Access Journals (Sweden)

    Krishna Kumar

    2010-11-01

    Full Text Available Radial spasm is often very prolonged and painful to the patient. Here, we describe a novel way to deal with the same. The total spasm lasted over 4 hours. A 3.4 6 JR catheter was introduced via the femoral route and papav arine one ampoule was injected directly into the right subclavian artery. After about 10 min we were able to pull out the radial catheter. Radial angiography is a simple procedure with reportedly less complications 1,2. How ever ,it has one major complication radial spasm. We describe here a patient with radial spasm that persisted for more than 2 hours and how we dealt with it.

  4. Radial artery spasm occurred in transradial coronary intervention for coronary heart disease: its occurrence and predictors

    International Nuclear Information System (INIS)

    Zhong Jiming; Li Lang; Lu Yongguang; Zeng Shuyi

    2011-01-01

    Objective: To discuss the incidence and clinical predictors of radial artery spasm occurred in performing transradial coronary intervention for coronary heart disease. Methods: A total of 1020 patients, who underwent transradial coronary procedures for coronary heart disease during the period of May 2007 Jan 2010 in authors' hospital, were enrolled in this study. All clinical information and medication were recorded in detail. Arteriography via radial artery was performed in all patients. The diameter of the radial artery as well as the arterial anatomy, including arterial variations, were determined and observed, which was follow by coronary angiography or percutaneous coronary intervention. Multivariate Logistic regression analysis was adopted to evaluate the variables, such as clinical parameters, angiographic characteristics of the radial artery and procedure-related factors, in predicting the occurrence of radial artery spasm. Results: Radial artery spasm occurred in 209 (20.5%) patients. Multivariate Logistic regression analysis showed that the following eight factors were independently associated with the occurrence of radial artery spasm. These factors were as follows: female gender (OR=2.8, 95% CI 2.5-5.8; P=0.001), age (OR=0.68, 95% CI 0.60-0.92; P=0.003), smoking (OR=2.3, 95% CI 1.8-4.1; P=0.026), moderate-to-severe pain of forearm during radial artery cannulation (OR=3.0, 95% CI 2.3-4.8; P=0.006), radial artery anatomical abnormalities (OR=4.7, 95% CI 3.6-7.2; P=0.002), the ratio of radial artery diameter to patient's height (RAH) (OR=5.2, 95% CI 3.7-8.1; P=0.012), the ratio of radial artery diameter to outer diameter of the sheath (RAOD) (OR=5.8, 95% CI 4.2-6.9; P=0.006) and the number of catheter exchange (OR=2.3, 95% CI 1.4-4.3; P=0.038). Conclusion: Radial artery spasm occurred in performing transradial coronary intervention for coronary heart disease is frequently seen in clinical practice. Female gender, younger age, smoking, forearm pain during

  5. Radial artery spasm associated with transradial cardiovascular procedures: results from the RAS registry.

    Science.gov (United States)

    Goldsmit, Alejandro; Kiemeneij, Ferdinand; Gilchrist, Ian C; Kantor, Pablo; Kedev, Sasko; Kwan, Tak; Dharma, Surya; Valdivieso, Leon; Wenstemberg, Bernard; Patel, Tejas

    2014-01-01

    To report the incidence and predictors of moderate/severe radial artery spasm (RAS) in patients undergoing cardiovascular percutaneous procedures through a transradial approach (TRA) in centers with TRA expertise. Data regarding the actual rate of clinically meaningful RAS are limited due to difference in study designs and operator expertise. The RAS registry, an international (14 centers from Argentina, Chile, India, Indonesia, Macedonia, The Netherlands and United States of America) registry that included 1,868 patients undergoing TRA cardiovascular procedures (63.5% diagnostic and 56.5% therapeutic).All selected centers used TRA as default strategy in the cardiac catheterization laboratory. Throughout 2012, each center included all consecutive TRA cases (during a 2-month period) into a dedicated database covering clinical characteristics as well as procedural topics related to TRA patterns and RAS occurrence. The incidence of moderate/severe RAS was 2.7%. Only 0.7% of patients required crossover (8 to transfemoral and 5 to contralateral TRA). Patients with moderately/severe spasm were more frequently females, had a history of dyslipidemia, received more often a 7F sheath and more puncture attempts than patients without spasm. By multivariate analysis, the need for more than one attempt and the use of a 7 F sheath were independent predictors of the development of moderate/severe RAS. The incidence of moderate/severe RAS is low in centers with a default TRA. Its development appears to be strongly related to the numbers of puncture attempts and the use of large sheaths. Copyright © 2013 Wiley Periodicals, Inc.

  6. Prevention of infantile spasms relapse: Zonisamide and topiramate provide no benefit.

    Science.gov (United States)

    Rajaraman, Rajsekar R; Lay, Johnson; Alayari, Amethyst; Anderson, Kirsten; Sankar, Raman; Hussain, Shaun A

    2016-08-01

    There is scant evidence to guide the management of infantile spasms after successful response to initial therapies. There is significant risk of relapse, largely because effective pharmacologic treatments cannot be continued long term because of concern for significant adverse events. Zonisamide (ZNS) and topiramate (TPM) are commonly used to prevent relapse, and the purpose of this study was to specifically evaluate the efficacy of ZNS and TPM as agents for secondary prevention of infantile spasms. Patients with video-electroencephalography (EEG) confirmed resolution of infantile spasms were retrospectively identified. Relevant clinical data were systematically collected, including lead time from onset of spasms to successful treatment response, etiology of infantile spasms, number of treatment failures prior to response, timing of relapse, and detailed exposure data for ZNS and TPM. We identified 106 patients with response to hormonal therapy (n = 58), vigabatrin (n = 25), or surgery (n = 23). To prevent relapse of infantile spasms, 37 patients received ZNS, 34 received TPM, 3 received both ZNS and TPM, and 38 patients received neither ZNS nor TPM. There were 44 relapses, occurring a median of 6.9 (3.2-10.8) months after initial response. Time to relapse was not affected by treatment with ZNS or TPM. Relapse was less likely among patients who were older (hazard ratio 0.97 [per month], p = 0.036) and those who responded to surgical resection (hazard ratio = 0.28, p = 0.017). Of note, we identified a relatively refractory cohort with multiple treatment failures and long lead time to initial response. In this refractory cohort, neither ZNS nor TPM was successful in preventing relapse of infantile spasms, despite relatively high dosages. At this time, aside from surgical resection in eligible candidates, there is no known treatment that is efficacious in the prevention of relapse of infantile spasms. Wiley Periodicals, Inc. © 2016 International League Against

  7. Pressure-mediated versus pharmacologic treatment of radial artery spasm during cardiac catheterisation: a randomised pilot study.

    Science.gov (United States)

    Collet, Carlos; Corral, Juan M; Cavalcante, Rafael; Tateishi, Hiroki; Belzarez, Oward; Ribamar Costa, Jose; Costa, Ricardo; Chamié, Daniel; Onuma, Yoshinobu; de Winter, Robbert J; Abizaid, Alexandre; Serruys, Patrick W

    2017-04-07

    The aim of the study was to determine the effectiveness of a novel strategy to treat radial artery spasm (RAS). We conducted a prospective, randomised, single-centre, open-label trial comparing a novel strategy of pressure-mediated dilatation versus intra-arterial administration of a combination of nitroglycerine plus verapamil for the treatment of RAS. The primary endpoint was radial artery intraluminal diameter acute gain assessed by quantitative radial angiography. After screening two hundred and twenty consecutive cases, twenty patients presented with RAS and were randomised 1:1 to either strategy. Overall the mean age was 60.8±11.5 years and 53% were females. Pre-treatment angiographic characteristics were similar between the groups. The primary endpoint of radial artery acute gain was significantly greater in the pressure-mediated dilatation group (0.85±0.46 mm vs. 0.03±0.24 mm, p<0.001). Blood pressure drop was significantly lower in the pressure-mediated dilatation group (ΔBP -3.8±24 vs. -31.6±19 mmHg, p<0.001). There was one case of radial artery occlusion in the pressure-mediated dilatation group at follow-up. Short-duration pain was observed during the application of pressure. Pressure-mediated dilatation for the treatment of RAS was feasible, with superior angiographic results compared to a pharmacologic vasodilator strategy, with no impact on blood pressure. This novel approach proved to be safe and effective and should be tested in a large randomised trial.

  8. Neonatal estradiol stimulation prevents epilepsy in Arx model of X-linked infantile spasms syndrome.

    Science.gov (United States)

    Olivetti, Pedro R; Maheshwari, Atul; Noebels, Jeffrey L

    2014-01-22

    Infantile spasms are a catastrophic form of pediatric epilepsy with inadequate treatment. In patients, mutation of ARX, a transcription factor selectively expressed in neuronal precursors and adult inhibitory interneurons, impairs cell migration and causes a major inherited subtype of the disease X-linked infantile spasms syndrome. Using an animal model, the Arx((GCG)10+7) mouse, we determined that brief estradiol (E2) administration during early postnatal development prevented spasms in infancy and seizures in adult mutants. E2 was ineffective when delivered after puberty or 30 days after birth. Early E2 treatment altered mRNA levels of three downstream targets of Arx (Shox2, Ebf3, and Lgi1) and restored depleted interneuron populations without increasing GABAergic synaptic density. Postnatal E2 treatment may induce lasting transcriptional changes that lead to enduring disease modification and could potentially serve as a therapy for inherited interneuronopathies.

  9. Infantile Spasms

    Science.gov (United States)

    ... syndrome of infancy and childhood known as West Syndrome. West Syndrome is characterized by infantile spasms, developmental regression, ... syndrome of infancy and childhood known as West Syndrome. West Syndrome is characterized by infantile spasms, developmental regression, ...

  10. EPILEPTIC SPASMS

    Directory of Open Access Journals (Sweden)

    K. Yu. Mukhin

    2014-01-01

    Full Text Available Epileptic spasms are epileptic seizures with sudden flexion/extension or of the mixed flexion and extension type, mainly involving the proximal and truncal muscles, that are normally longer than myoclonic seizures but shorter than tonic seizures, and last for about 1 second. For diagnostics of epileptic spasms, it is necessary that they are combined with ictal and interictal epileptiform patterns on electroencephalography (EEG. The first detailed clinical description of seizures of the infantile spasms type was provided by English pediatrician W.J. West in 1841. The term of infantile spasms is limited with age and means epileptic spasms that occur to children in early infancy, usually up to 1 y.o. Infantile spasms cannot be synonymous to the West syndrome. Infantile spasms are a type of epileptic seizures and West syndrome is a form of epilepsy that is usually manifested through hypsarrhythmia on the EEG and mental retardation, apart from infantile spasms. Epileptic spasms is the term broader than infantile spasms. Committee of the International League Against Epilepsy (ILAE recommends exactly the “epileptic spasms” term, as this type of seizures is not a prerogative of the West syndrome and can be observed in children older than 1 y.o. and even in adults. The authors provided a detailed review of modern references devoted to epileptic spasms including the history of the issue, determination of the term, and position of epileptic spasms in modern classification systems, approaches to diagnostics including differential diagnosis, treatment, and prognosis.

  11. Esophageal Spasms

    Science.gov (United States)

    ... Symptom Checker Esophageal spasms Symptoms & causes Diagnosis & treatment Advertisement Mayo Clinic does not endorse companies or products. ... a Job Site Map About This Site Twitter Facebook Google YouTube Pinterest Mayo Clinic is a not- ...

  12. EPILEPTIC SPASMS

    OpenAIRE

    K. Yu. Mukhin; M. B. Mironov

    2014-01-01

    Epileptic spasms are epileptic seizures with sudden flexion/extension or of the mixed flexion and extension type, mainly involving the proximal and truncal muscles, that are normally longer than myoclonic seizures but shorter than tonic seizures, and last for about 1 second. For diagnostics of epileptic spasms, it is necessary that they are combined with ictal and interictal epileptiform patterns on electroencephalography (EEG). The first detailed clinical description of seizures of the infan...

  13. Neck pain or spasms -- self care

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/patientinstructions/000802.htm Neck pain or spasms - self care To use the sharing ... strengthening exercises and how to do them. Preventing Neck Pain If you work at a computer or a ...

  14. [Facial tics and spasms].

    Science.gov (United States)

    Potgieser, Adriaan R E; van Dijk, J Marc C; Elting, Jan Willem J; de Koning-Tijssen, Marina A J

    2014-01-01

    Facial tics and spasms are socially incapacitating, but effective treatment is often available. The clinical picture is sufficient for distinguishing between the different diseases that cause this affliction.We describe three cases of patients with facial tics or spasms: one case of tics, which are familiar to many physicians; one case of blepharospasms; and one case of hemifacial spasms. We discuss the differential diagnosis and the treatment possibilities for facial tics and spasms. Early diagnosis and treatment is important, because of the associated social incapacitation. Botulin toxin should be considered as a treatment option for facial tics and a curative neurosurgical intervention should be considered for hemifacial spasms.

  15. Vigabatrin for infantile spasms.

    Science.gov (United States)

    Pesaturo, Kimberly A; Spooner, Linda M; Belliveau, Paul

    2011-03-01

    Infantile spasms describe a pediatric epilepsy syndrome characterized by frequent clusters of brief symmetric muscle contractions; the condition is often associated with developmental delay. When infantile spasms are accompanied by hypsarrhythmia on electroencephalogram, the condition is labeled West syndrome. The mainstay of treatment for infantile spasms is adrenocorticotropic hormone; however, vigabatrin, a vinyl derivative of γ-aminobutyric acid, has been used for the treatment of infantile spasms in Europe since 1989. In 2009, vigabatrin was approved by the United States Food and Drug Adminstration (FDA) for use as monotherapy in the treatment of infantile spasms in patients aged 1 month-2 years when the benefits of treatment outweigh the risks. Results from numerous trials examining the role of vigabatrin in infantile spasms have been published; many of these trials were small, open-label, or noncontrolled. Although clinical trials have provided some insight into the utility of vigabatrin for the treatment of infantile spasms, these studies have notable limitations. In addition, vigabatrin is associated with a black-box warning that describes the potential for permanent bilateral concentric visual field defects. Currently, vigabatrin is available through a manufacturer-sponsored program in accordance with its FDA-approved Risk Evaluation and Mitigation Strategy. Although several guidelines recommend vigabatrin as a first-line therapy for infantile spasms, specifically infantile spasms related to tuberous sclerosis, it is still unclear whether vigabatrin should supersede hormone therapy as first-line therapy. Further research comparing the two therapies is needed.

  16. Management of infantile spasms.

    Science.gov (United States)

    Nelson, Gary Rex

    2015-10-01

    West syndrome, or infantile spasms syndrome is a frequently catastrophic infantile epileptic encephalopathy with a variety of etiologies. Despite the heterogeneous nature of causes of infantile spasms, a careful diagnostic evaluation can lead to diagnosis in many patients and may guide treatment choices. Magnetic resonance imaging (MRI) brain remains the highest yield initial study in determining the etiology in infantile spasms. Treatment of infantile spasms has little class I data, but adrenocorticotropic hormone (ACTH), prednisolone and vigabatrin have the best evidence as first-line medications. Other therapies including the ketogenic diet and other anti-epileptics medications may also prove useful in the treatment of infantile spasms. In general, more studies are needed to determine the best treatment regimen for this condition. Prognosis is generally poor, with the majority of patients having some or profound neurocognitive delays. Patients without delays at diagnosis and without an identifiable etiology, if treated appropriately, have the greatest likelihood of a normal outcome.

  17. Treatment of infantile spasms

    OpenAIRE

    2005-01-01

    The rationale, dosage, and side effects of ACTH treatment of infantile spasms are reviewed from the Department of Neurology, University of Southern California School of Medicine, and Children’s Hospital of Los Angeles, CA.

  18. Treatment of Symptomatic Infantile Spasms

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2013-05-01

    Full Text Available Investigators at Tokyo Women's Medical University studied the clinical, radiological, and EEG characteristics of 69 patients with infantile spasms (IS followed for 3-74 months (mean 18 months after initial cessation of epileptic spasms (ES.

  19. Genetic Classification of Infantile Spasms

    OpenAIRE

    J Gordon Millichap

    2012-01-01

    Researchers from University of Washington, Seattle, WA, and Washington University, St Louis, MO propose a genetic and biologic classification of infantile spasms. Infantile spasms are of 2 main groups: those with known or unknown predisposing genotypes.

  20. Treatment of Symptomatic Infantile Spasms

    OpenAIRE

    J Gordon Millichap

    2013-01-01

    Investigators at Tokyo Women's Medical University studied the clinical, radiological, and EEG characteristics of 69 patients with infantile spasms (IS) followed for 3-74 months (mean 18 months) after initial cessation of epileptic spasms (ES).

  1. Patent haemostasis prevents radial artery occlusion in patients with an acute coronary syndrome.

    Science.gov (United States)

    Wilson, Simon J; Mitchell, Andrew; Gray, Timothy J M; Loh, Hoe Jun; Cruden, Nick L

    2017-08-01

    A haemostatic technique that maintains radial artery flow ("patent haemostasis") following transradial catheterization reduces rates of radial artery occlusion (RAO) in patients with stable coronary disease. It is unclear whether this benefit extends to patients with an acute coronary syndrome (ACS). Patients undergoing inpatient transradial catheterization for an ACS were prospectively enrolled in a consecutive cohort study (n=300). Radial haemostasis was obtained using standard radial compression (cohort 1; n=150) or patent haemostasis (cohort 2; n=150). An end-of-case activated clotting time (ACT) was recorded and radial artery patency assessed within 24 hours of sheath removal by reverse Barbeau's test. The incidence of RAO was 16.0% following standard radial compression and 5.3% following patent haemostasis (p=0.003). Univariate predictors of RAO were patent haemostasis (OR 0.30; [0.13-0.68], p=0.004), hyperlipidaemia (OR 0.46; [0.21-0.98], p=0.04), history of current smoking (OR 2.86; [1.3-6.0], p=0.015) and longer procedure times (OR 1.03/additional minute; [1.01-1.05], p=0.003). There was no association between the end-of-case ACT and RAO (OR 1.00; [0.9-1.01] p=1.00). After adjusting for covariates, patent haemostasis reduced the risk of RAO by 70% compared to standard compression (OR 0.30; [0.12-0.77], p=0.12). The c-statistic for model discrimination was 0.79 (95% CI [0.71-0.86], ppatent haemostasis as an independent predictor of reduced RAO (OR 0.38 [0.15-0.95], p=0.039). Patent haemostasis is highly effective in preventing early RAO in patients with ACS. Copyright © 2017 Elsevier B.V. All rights reserved.

  2. Treatment of infantile spasms.

    Science.gov (United States)

    Hancock, Eleanor C; Osborne, John P; Edwards, Stuart W

    2013-06-05

    Infantile spasms (West's Syndrome) is a syndrome that includes a peculiar type of epileptic seizure-the spasms-and an electroencephalographic (EEG) abnormality often called hypsarrhythmia. Psychomotor retardation is frequently found at follow-up. Approximately two-thirds of affected infants will have a detectable underlying neurological abnormality, but still little is known about the pathophysiological basis for infantile spasms, and treatment remains problematic. To compare the effects of single pharmaceutical therapies used to treat infantile spasms in terms of control of the spasms, resolution of the EEG, relapse rates, psychomotor development, subsequent epilepsy, side effects, and mortality. To identify published data, we searched the Cochrane Epilepsy Group Specialised Register (October 2012), CENTRAL (The Cochrane Library 2012, Issue 9), MEDLINE (1946 to September Week 4, 2012), EMBASE (1980 to March 2003), and the reference lists of all retrieved articles.To identify unpublished data, we searched the ISRCTN Register (www.controlled-trials.com), corresponded with colleagues and drug companies, and made requests at international conferences. All randomised controlled trials (RCTs) of the administration of drug therapy to patients with infantile spasms. Data collection from all relevant publications was independently undertaken by three review authors (before 2010) or by two review authors using a standard proforma. Analysis included assessment of study quality and a search for sources of heterogeneity. We found 16 small RCTs (fewer than 100 patients enrolled) and 2 larger RCTs (more than 100 patients enrolled). These 18 studies looked at a total of 916 patients treated with a total of 12 different pharmaceutical agents. Overall methodology of the studies was poor, in part because of ethical dilemmas such as giving placebo injections to children. Two studies showed that placebo was not as good as active treatment in resolving the spasms. The strongest

  3. Recognition of Infantile Spasms Is Often Delayed: The ASSIST Study.

    Science.gov (United States)

    Hussain, Shaun A; Lay, Johnson; Cheng, Emily; Weng, Julius; Sankar, Raman; Baca, Christine B

    2017-11-01

    To characterize and quantify diagnostic and treatment delay among children with infantile spasms, and to estimate the developmental impact of this delay. In this cohort study, we surveyed the parents of 100 patients with infantile spasms about their experiences with diagnosis and treatment, and ascertained medical and sociodemographic factors potentially related to care of these infants. We specifically determined the latency to first visit an "effective provider," defined as a provider who identified infantile spasms, and prescribed an appropriate first-line treatment, namely adrenocorticotropic hormone, corticosteroids, or vigabatrin. Time to the first visit to an effective provider was evaluated using Cox proportional hazards regression. The median time from the onset of infantile spasms to first visit with an effective provider was 24.5 days. Only 29% of patients were evaluated by an effective provider within 1 week of infantile spasms onset. The time to first effective provider visit was associated with parental language preference, but with no other sociodemographic characteristics. Parents' suspicions that "something is wrong" were often discounted by healthcare providers, and survey respondents frequently reported that pediatricians and neurologists were unfamiliar with infantile spasms. This study demonstrates that substantial delay (ie, >1 week) in appropriate care is common, and suggests that the poor awareness of infantile spasms among healthcare providers is at least partly responsible for preventable and potentially significant delays in treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. Topiramate Monotherapy in Infantile Spasm

    Science.gov (United States)

    Jun, Yong-Hoon; Hong, Young-Jin; Son, Byong-Kwan

    2006-01-01

    Infantile spasm is an age-related refractory epilepsy. Topiramate is a new anticonvulsant with multiple mechanisms of action, and it may be effective for treating pediatric epilepsies. To evaluate the efficacy and tolerability of first-line topiramate treatment for infantile spasm, 20 patients received topiramate monotherapy during this study. They were treated with an initial dose of 1 mg/kg/day, with a progressive titration of 1 mg/kg a week until their spasms were controlled and a maximum dose of 12 mg/kg/day was achieved. The evaluation of the treatment efficacy was based on the spasm frequency data that was obtained by the scalp and video-EEG, and by the parental count of spasm. Thirty percent of the subjects became spasm-free during the study. Six of 20 subjects (30%) had cessation of spasm and disappearance of hypsarrhythmia as seen via the video EEG; four (50%) of eight idiopathic patients had a response, whereas two (17%) of 12 patients with symptomatic infantile spasm responded. Seventy of the patients, including the spasm-free patients, had a reduction in their seizure frequency of more than 50%, and 10% of the patients had a reduction in their seizure frequency of less than 50%. The clusters of spasm frequency decreased from 10.6 ± 8.5 to 3.5 ± 1.4 clusters/day. Topiramate is effective and tolerated in those patients suffering from infantile spasm. Our results suggest that this drug should be considered as a new first-line drug for treating infantile spasm. PMID:16941739

  5. Infantile spasms and pigmentary mosaicism

    DEFF Research Database (Denmark)

    Hansen, Lars K; Bygum, Anette; Krogh, Lotte N

    2010-01-01

    Summary We present a 3-year-old boy with pigmentary mosaicism and persistent intractable infantile spasms due to mosaicism of chromosome 7. Getting the diagnosis of pigmentary mosaicism in a child with infantile spasms may not be easy, as most diagnostic work-up is done in infancy, at a time when...

  6. Prevention of instability of the proximal end of the radius after radial head resection using an anconeus muscle flap.

    Science.gov (United States)

    Nishida, Kinya; Iwasaki, Norimasa; Funakoshi, Tadanao; Motomiya, Makoto; Minami, Akio

    2012-01-01

    Resection of the radial head frequently causes instability of the proximal end of the radius. To prevent this instability, we performed a stabilization technique using an anconeus muscle flap. Since 2003, six patients with radiocapitellar joint dysfunction have been treated with radial head resection combined with stabilizing its proximal end using an anconeus muscle flap. At a mean follow-up of 51 months, all patients were free from elbow pain and the mean Mayo Elbow Performance Score and the Disabilities of the Arm, Shoulder and Hand score significantly improved. Radiographic findings showed no apparent instability of the proximal radius. The anconeus is useful as a reliable muscle flap for preventing instability of the proximal radius after a radial head resection. This procedure does not require any microvascular techniques and makes it possible to apply a pedicled muscle flap using a relatively simple technique without any considerable risks of elbow dysfunction.

  7. Distal esophageal spasm.

    Science.gov (United States)

    Roman, Sabine; Kahrilas, Peter J

    2015-07-01

    Distal esophageal spasm (DES) is a rare esophageal motility disorder associated with dysphagia and chest pain. In 2011, the diagnosis of DES was refined based on the occurrence of premature (rather than rapid) contractions by high-resolution manometry. New therapeutic options have also been recently proposed. Thus, a review on DES incorporating publications since 2012 is timely because of these revisions in definition and management. DES remains a heterogeneous clinical disorder. Its pathophysiology is still debated and DES might be related to achalasia. Alternatively, it might be secondary to medications, especially opiates. Endoscopic ultrasound might be informative diagnostically by demonstrating muscularis propria hypertrophy and thickening. Botulinum toxin injection in the esophageal body has been shown superior to placebo to relieve symptoms associated with DES. Finally, per oral endoscopic myotomy is a promising therapeutic approach, but may be less effective in DES than in achalasia. The diagnosis of DES should lead to a systematic search for medication that might promote the occurrence of esophageal dysmotility. Endoscopic treatment of DES (botulinum toxin injection or per oral endoscopic myotomy) should be further evaluated in controlled studies using current diagnostic criteria by high-resolution manometry.

  8. Recent advances in the pharmacotherapy of infantile spasms.

    Science.gov (United States)

    Riikonen, Raili

    2014-04-01

    Adrenocorticotrophic hormone (ACTH), oral corticosteroids and vigabatrin are now first-line treatments for infantile spasms in the US and Europe. There is now increased knowledge regarding the role of ACTH, corticosteroids and vigabatrin (e.g. efficacy, doses, side effects, treatment in specific aetiological subtypes of infantile spasms), and other antiepileptic drugs (i.e. topiramate, valproate, zonisamide, sulthiame, levetiracetam, lamotrigine, pyridoxine, ganaxolone), as well as adjunctive flunarizine and novel drugs not yet in clinical use for infantile spasms (i.e. pulse rapamycin and melanocortin receptor agonists). The existence of a latent period, weeks to months following a precipitating brain insult, raises the possibility of preventive interventions. Recent experimental data emerging from animal models of infantile spasms have provided optimism that new and innovative treatments can be developed, and knowledge that drug treatment can affect long-term cognitive outcome is increasing. The aim of this article is to review recent developments in the pharmacotherapy of infantile spasms and to highlight the practical implications of the latest research.

  9. What Is a Coronary Artery Spasm?

    Science.gov (United States)

    ... pain (angina) and even a heart attack (myocardial infarction). These spasms are sometimes referred to as Prinzmetal's ... factors. Coronary artery spasms may be triggered by: Tobacco use Exposure to cold Extreme emotional stress Use ...

  10. Infantile Spasms: A Critical Review of Emerging Animal Models

    Science.gov (United States)

    Stafstrom, Carl E

    2009-01-01

    Infantile spasms is a developmental epilepsy syndrome with unique clinical and EEG features, a specific pattern of pharmacological responsiveness, and poor outcome in terms of cognition and epilepsy. Despite the devastating nature of infantile spasms, little is known about its pathogenesis. Until recently, there has been no animal model available to investigate the pathophysiology of the syndrome or to generate and test novel therapies. Now, several promising animal models have emerged, spanning the etiological spectrum from genetic causes (e.g., Down syndrome or Aristaless-related homeobox [ARX] mutation) to acquired causes (e.g., endogenous and exogenous toxins or stress hormones with convulsant activity or blockade of neural activity). These new models are discussed in this review, with emphasis on the insights each can provide for understanding, treating, and preventing infantile spasms. PMID:19471616

  11. MR imaging in infantile spasms

    Energy Technology Data Exchange (ETDEWEB)

    Yasujima, Miki; Konishi, Yukuo; Kuriyama, Masanori (Fukui Medical School (Japan)) (and others)

    1989-11-01

    CT and MRI in 13 patients with infantile spasms were analyzed. Cortical atrophy was found in 8 cases and ventricular dilatation in 9 cases. The patients with severe cortical atrophy or ventricular dilatation had poorer prognosis than the patients with normal CT findings. In addition to cortical atrophy and ventricular dilatation which were shown in CT, MRI revealed poor differentiation of gray and white matters in all cases and periventricular hyperintensity area in 9 patients. Six patients with the periventricular hyperintensity area of grade III and IV had severe developmental delay. With regard to the prognosis, MRI plays a more useful role in detecting intracranial pathology in infantile spasms. (author).

  12. Infantile spasms: A prognostic evaluation.

    Science.gov (United States)

    Iype, Mary; Saradakutty, Geetha; Kunju, Puthuvathra Abdul Mohammed; Mohan, Devi; Nair, Muttathu Krishnapanicker Chandrasekharan; George, Babu; Ahamed, Shahanaz M

    2016-01-01

    Few papers address the comprehensive prognosis in infantile spasms and look into the seizure profile and psychomotor outcome. We aimed to follow up children with infantile spasms to study: a) the etiology, demographics, semiology, electroencephalogram (EEG), and radiological pattern; b) seizure control, psychomotor development, and EEG resolution with treatment; c) the effects of various factors on the control of spasms, resolution of EEG changes, and psychomotor development at 3-year follow-up. Fifty newly diagnosed cases with a 1-12 month age of onset and who had hypsarrhythmia in their EEG were recruited and 43 were followed up for 3 years. Of the children followed up, 51% were seizure-free and 37% had a normal EEG at the 3-year follow-up. Autistic features were seen in 74% of the children. Only 22.7% among the seizure-free (11.6% of the total) children had normal vision and hearing, speech with narration, writing skills, gross and fine motor development, and no autism or hyperactivity. On multivariate analysis, two factors could predict bad seizure outcome - the occurrence of other seizures in addition to infantile spasms and no response to 28 days of adrenocorticotropic hormone (ACTH). No predictor could be identified for abnormal psychomotor development. In our study, we could demonstrate two factors that predict seizure freedom. The cognitive outcome and seizure control in this group of children are comparable to the existing literature. However, the cognitive outcome revealed by our study and the survey of the literature are discouraging.

  13. Atonic elements combined or uncombined with epileptic spasms in infantile spasms.

    Science.gov (United States)

    Xue, Jiao; Qian, Ping; Li, Hui; Yang, Haipo; Liu, Xiaoyan; Zhang, Yuehua; Yang, Zhixian

    2017-01-01

    To study the atonic elements combined or uncombined with epileptic spasms in infantile spasms. The demographic data, clinical characteristics, electroencephalogram (EEG), and polyelectromyography (PEMG) features were analyzed in 12 infantile spasm patients with atonic elements. A total of 29 EEGs were recorded. Hypsarrhythmia or hypsarrhythmia variants were identified during interictal EEG. Insular or clustered epileptic spasms occurred in all. Three subtypes of atonic elements combined or uncombined with epileptic spasms (spasm-atonic, pure atonic, and atonic-spasm seizures) were observed electroclinically, which could present insularly or in cluster or altered with epileptic spasms in the same cluster. The ictal EEG showed generalized high-amplitude slow waves presenting alone or combined with other patterns. The corresponding PEMG showed an obvious electrical silence alone or preceding or following a crescendo-decrescendo pattern generated from myoelectric burst. Atonic elements combined or uncombined with epileptic spasms was a newly noticed phenomenon in infantile spasms, which was artificially divided into three subtypes here. It might be a variant of epileptic spasms or a unique seizure type. Atonic elements combined or uncombined with epileptic spasms was a previously ignored phenomenon in infantile spasms, which should be seriously considered in clinical practice. Copyright © 2016 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  14. Infantile spasms: A prognostic evaluation

    Directory of Open Access Journals (Sweden)

    Mary Iype

    2016-01-01

    Full Text Available Background: Few papers address the comprehensive prognosis in infantile spasms and look into the seizure profile and psychomotor outcome. Objective: We aimed to follow up children with infantile spasms to study: a the etiology, demographics, semiology, electroencephalogram (EEG, and radiological pattern; b seizure control, psychomotor development, and EEG resolution with treatment; c the effects of various factors on the control of spasms, resolution of EEG changes, and psychomotor development at 3-year follow-up. Materials and Methods: Fifty newly diagnosed cases with a 1-12 month age of onset and who had hypsarrhythmia in their EEG were recruited and 43 were followed up for 3 years. Results: Of the children followed up, 51% were seizure-free and 37% had a normal EEG at the 3-year follow-up. Autistic features were seen in 74% of the children. Only 22.7% among the seizure-free (11.6% of the total children had normal vision and hearing, speech with narration, writing skills, gross and fine motor development, and no autism or hyperactivity. On multivariate analysis, two factors could predict bad seizure outcome — the occurrence of other seizures in addition to infantile spasms and no response to 28 days of adrenocorticotropic hormone (ACTH. No predictor could be identified for abnormal psychomotor development. Discussion and Conclusion: In our study, we could demonstrate two factors that predict seizure freedom. The cognitive outcome and seizure control in this group of children are comparable to the existing literature. However, the cognitive outcome revealed by our study and the survey of the literature are discouraging.

  15. Infantile spasms: A prognostic evaluation

    Science.gov (United States)

    Iype, Mary; Saradakutty, Geetha; Kunju, Puthuvathra Abdul Mohammed; Mohan, Devi; Nair, Muttathu Krishnapanicker Chandrasekharan; George, Babu; Ahamed, Shahanaz M.

    2016-01-01

    Background: Few papers address the comprehensive prognosis in infantile spasms and look into the seizure profile and psychomotor outcome. Objective: We aimed to follow up children with infantile spasms to study: a) the etiology, demographics, semiology, electroencephalogram (EEG), and radiological pattern; b) seizure control, psychomotor development, and EEG resolution with treatment; c) the effects of various factors on the control of spasms, resolution of EEG changes, and psychomotor development at 3-year follow-up. Materials and Methods: Fifty newly diagnosed cases with a 1-12 month age of onset and who had hypsarrhythmia in their EEG were recruited and 43 were followed up for 3 years. Results: Of the children followed up, 51% were seizure-free and 37% had a normal EEG at the 3-year follow-up. Autistic features were seen in 74% of the children. Only 22.7% among the seizure-free (11.6% of the total) children had normal vision and hearing, speech with narration, writing skills, gross and fine motor development, and no autism or hyperactivity. On multivariate analysis, two factors could predict bad seizure outcome — the occurrence of other seizures in addition to infantile spasms and no response to 28 days of adrenocorticotropic hormone (ACTH). No predictor could be identified for abnormal psychomotor development. Discussion and Conclusion: In our study, we could demonstrate two factors that predict seizure freedom. The cognitive outcome and seizure control in this group of children are comparable to the existing literature. However, the cognitive outcome revealed by our study and the survey of the literature are discouraging. PMID:27293335

  16. Dedicated radial ventriculography pigtail catheter

    Energy Technology Data Exchange (ETDEWEB)

    Vidovich, Mladen I., E-mail: miv@uic.edu

    2013-05-15

    A new dedicated cardiac ventriculography catheter was specifically designed for radial and upper arm arterial access approach. Two catheter configurations have been developed to facilitate retrograde crossing of the aortic valve and to conform to various subclavian, ascending aortic and left ventricular anatomies. The “short” dedicated radial ventriculography catheter is suited for horizontal ascending aortas, obese body habitus, short stature and small ventricular cavities. The “long” dedicated radial ventriculography catheter is suited for vertical ascending aortas, thin body habitus, tall stature and larger ventricular cavities. This new design allows for improved performance, faster and simpler insertion in the left ventricle which can reduce procedure time, radiation exposure and propensity for radial artery spasm due to excessive catheter manipulation. Two different catheter configurations allow for optimal catheter selection in a broad range of patient anatomies. The catheter is exceptionally stable during contrast power injection and provides equivalent cavity opacification to traditional femoral ventriculography catheter designs.

  17. Infantile Spasm: A Review Article

    Science.gov (United States)

    TAGHDIRI, Mohammad Mahdi; NEMATI, Hamid

    2014-01-01

    Objective Infantile spasm (IS) is a convulsive disease characterized by brief, symmetric axial muscle contraction (neck, trunk, and/or extremities). IS is a type of seizure that was first described by West in 1841, who witnessed the seizure in his own son. West’s syndrome refers to the classic triad of spasms, characteristic EEG, and neurodevelopmental regression. Most cases involve flexors and extensors, but either of the types may be involved independently. IS, as its name implies, most often occurs during the first year of life with an incidence of approximately 1 per 2000-4000 live births. Most, but not all, patients with this disorder have severe EEG abnormalities; this pattern was originally referred to as hypsarrhythmia by Gibbs and Gibbs. Cases with known etiology or signs of brain damage are considered as symptomatic. The Overall prognosis of the disease is poor. Peak onset age of the epileptic syndrome is 3 to 7 months, which mainly occurs before 2 years of age in 93% of patients. Hypsarrhythmia is the EEG hallmark of IS, which comprised a chaotic, bilaterally asynchronous high-voltage polyspike, and slow wave discharges interspersed with multifocal spikes and slow waves. Etiological classification is as follows: 1) Symptomatic: with identifiable prenatal, perinatal, and postnatal causes with developmental delay at the presentation time; 2) Cryptogenic: unknown underlying cause, normal development at the onset of spasms, normal neurological exam and neuroimaging, and no abnormality in the metabolic evaluation; 3) Idiopathic: pure functional cerebral dysfunction with complete recovery, no residual dysfunction, normal neuroimaging and normal etiologic evaluation, and normal neurodevelopment. PMID:25143766

  18. Hemifacial spasm. Study by magnetic resonance angiography

    International Nuclear Information System (INIS)

    Bittar, Miriam Salvadori; Staut, Claudio Cesar Vilela; Barbosa, Egberto Reis; Bacheschi, Luiz Alberto; Magalhaes, Alvaro Cebrian de Almeida

    1995-01-01

    Nine patients with idiopathic hemifacial spasm were evaluated with cranial magnetic resonance imaging and angiography. Alterations of the posterior fossa vasculature, possibly related to the facial nerve irritation, were found in 8 patients (88%). Magnetic resonance angiography is a noninvasive procedure and appears to be a sensitive method to evaluate hemifacial spasm etiology. (author)

  19. Practice Parameter: Medical Treatment of Infantile Spasms

    Science.gov (United States)

    Mackay, M.T.; Weiss, S.K.; Adams-Webber, T.; Ashwal, S.; Stephens, D.; Ballaban-Gill, K.; Baram, T.Z.; Duchowny, M.; Hirtz, D.; Pellock, J.M.; Shields, W.D.; Shinnar, S.; Wyllie, E.; Snead, O.C.

    2010-01-01

    Objective To determine the current best practice for treatment of infantile spasms in children. Methods Database searches of MEDLINE from 1966 and EMBASE from 1980 and searches of reference lists of retrieved articles were performed. Inclusion criteria were the documented presence of infantile spasms and hypsarrhythmia. Outcome measures included complete cessation of spasms, resolution of hypsarrhythmia, relapse rate, developmental outcome, and presence or absence of epilepsy or an epileptiform EEG. One hundred fifty-nine articles were selected for detailed review. Recommendations were based on a four-tiered classification scheme. Results Adrenocorticotropic hormone (ACTH) is probably effective for the short-term treatment of infantile spasms, but there is insufficient evidence to recommend the optimum dosage and duration of treatment. There is insufficient evidence to determine whether oral corticosteroids are effective. Vigabatrin is possibly effective for the short-term treatment of infantile spasm and is possibly also effective for children with tuberous sclerosis. Concerns about retinal toxicity suggest that serial ophthalmologic screening is required in patients on vigabatrin; however, the data are insufficient to make recommendations regarding the frequency or type of screening. There is insufficient evidence to recommend any other treatment of infantile spasms. There is insufficient evidence to conclude that successful treatment of infantile spasms improves the long-term prognosis. Conclusions ACTH is probably an effective agent in the short-term treatment of infantile spasms. Vigabatrin is possibly effective. PMID:15159460

  20. Diagnosing infantile spasms: Accuracy of the internet.

    Science.gov (United States)

    Krag, Amara; Holmes, Gregory L

    2016-11-01

    Infantile spasms, one of the catastrophic epilepsies, can be a diagnostic challenge since the clinical manifestations may be subtle and may mimic benign conditions. Because of the rarity of the condition, primary care physicians and pediatricians may never see a case of infantile spasms during their career and may be unfamiliar with the seizure semiology. This is a serious issue since there is evidence that early diagnosis and treatment may improve outcome. Patients and families are increasingly using the internet more than their physician as a source of medical information about epilepsy. The reliance on using the internet for diagnostic information raises concerns about the accuracy of posted material that has not undergone professional review. To assess the quality of information being obtained about infantile spasms, we reviewed written and video content addressing infantile spasms on the internet. A total of 135 websites and 156 videos were reviewed for accuracy. Using the search terms Infantile Spasms and West Syndrome, we report that the majority of the written and video information provided was accurate and provided important and pertinent information. We conclude that internet searches provide accurate and pragmatic information about infantile spasms that has the potential for reducing the delay between the onset of the spasms and medical intervention. Copyright © 2016. Published by Elsevier Inc.

  1. Infantile Spasms Respond Poorly to Topiramate.

    Science.gov (United States)

    Weber, Amanda; Cole, Justin W; Mytinger, John R

    2015-08-01

    Infantile spasms are seizures typical of an age-related epileptic encephalopathy. Although evidence supporting topiramate for infantile spasms is lacking, many clinicians use it for this indication. The aim of this study was to determine the rate of infantile spasm remission with topiramate at our institution. A low rate of infantile spasm remission was hypothesized. This was a single-center retrospective medical record review of patients treated with topiramate for infantile spasms between January 2009 and September 2013. Records were reviewed for accuracy of diagnosis and outcome. Clinical remission of infantile spasms was defined as resolution for at least 28 days at any time during treatment with topiramate. For patients with clinical remission, posttreatment electroencephalographs were reviewed to assess for electrographic remission. To assess for confounding variables affecting remission rate, demographics and outcomes were compared with patients treated with adrenocorticotropic hormone within the same period using the same criteria for remission. Three of 31 (9.7%) patients achieved clinical remission with topiramate, two of whom also experienced electrographic remission. The third patient had electrographic remission with previous adrenocorticotropic hormone treatment but infantile spasm remission only after receiving topiramate. All three of these patients experienced subsequent electroclinical relapse during topiramate therapy. Although there were no significant demographic differences between the topiramate and adrenocorticotropic hormone cohorts, more adrenocorticotropic hormone patients achieved clinical remission (9.7% versus 56%; P infantile spasms with topiramate was uncommon and no patient experienced persistent electroclinical remission. These findings suggest that infantile spasms respond poorly to topiramate. Copyright © 2015 Elsevier Inc. All rights reserved.

  2. [Clinical and polyneuroelectrophysiological characteristics of infantile spasm].

    Science.gov (United States)

    Wang, Bo; Cai, Fang-cheng

    2007-02-01

    To explore the characteristics of various seizure types in infantile spasm (IS) and to recognize the clinical and electrophysiological differences among spasm, myoclonic and tonic seizures. Totally 681 seizures of 8 infants with IS were analyzed, including 20 episodes of non-cortical myoclonus which were finally ruled out by video-electroencephalogram-electromyogram polygraphic recordings (VEEG-EMG) and off-line analysis of jerk-locked back averaging (JLA). As a control, the data of 58 myoclonic seizures collected from an infant with Aicardi syndrome within two months before his typical clinical presentations of IS were also analyzed. Three types of seizures were recorded from the 8 infants, including spasm, myoclonic and tonic seizures with the incidence of 94.4%, 4.5%, and 1.1%, respectively. Spasms were mostly presented as body muscle contraction axially, which often occurred in clusters and evolved in a crescendo-decrescendo manner; 85.7% of them lasted for 0.4 - 3.0 s and 14.3% for 3 - 7 s. In addition, there were 273 seizures which were identified as subtle spasms according to their ictal EEG with high voltage slow wave (HVS) and fast wave bursts in most. There was no constantly time-locked EEG correlating to spasms even when JLA was applied for analysis. Myoclonic seizures were shock-like muscle constraction lasting for less than 400 ms with or without visible epileptic discharges in its ictal EEG. However, there was a time-locked cortical discharge discerned by JLA in epileptic myoclonus. Tonic seizures were consisted of sustained muscle contractions involving limbs and trunk, lasting for more than 3 s. Its ictal EEGs were more likely low amplitude fast waves and medium amplitude theta activities. Some spasms, named as tonic spasm, could be distinguished from tonic seizure according to the seizure duration which was always less than 2 s in tonic spasms and their different EEG patterns. There were various seizure types in IS but spasm was the predominant one

  3. Linear Nevus Sebaceum Syndrome and Infantile Spasms

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2008-03-01

    Full Text Available Two infants with linear nevus sebaceum syndrome and infantile spasms are reported from Safra Childrens Hospital, Sheba Medical Center, Tel Hashomer, Israel; and Hospital for Sick Children, Toronto, Canada.

  4. Focal Cortical Hypometabolism and Infantile Spasms

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2002-06-01

    Full Text Available The occurrence and prognostic significance of focal defects in cerebral cortical glucose metabolism were evaluated in infants with newly diagnosed symptomatic and cryptogenic infantile spasms examined at Turku and Helsinki Universities, Finland.

  5. Infantile Spasms Treated with Intravenous Methypredinsolone Pulse.

    Science.gov (United States)

    Hassanzadeh Rad, Afagh; Aminzadeh, Vahid

    2017-01-01

    Infantile spasms is diagnosed late even by expert pediatricians. Late diagnosis (later than 3 weeks) can have a negative effect on the long-term prognosis. We aimed to investigate infantile spasms treated with intravenous methylprednisolone pulse. In this case series study, 20 infants with infantile spasms in 17-Shahrivar Hospital, Rasht, Iran were enrolled. Drugs were administered based on Mytinger protocol that included 3 days of methylprednisolone pulse and 56 days of oral prednisolone. The control of spasms and the omission of hypsarrhythmia in infants follow-up were the primary and secondary outcomes, respectively. Remission was indicated if the caregivers mentioned no spasms or >50% decrease regarding drug initiation for at least 5 consecutive days and the electroencephalography during sleep period noted the omission of hypsarrhythmia. Eleven female (55%) and 9 male (45%) patients with the mean age of 4.95±1.39 months were enrolled. Mean rapid remission was noted as 4.41±1.50 days. Twelve patients (60%) noted early remission. seizure was controlled in 3(15%) patients completely after 24 months. Five (25%) occasional seizures were noted controlled by routine anticonvulsant drugs after 24 months and 12 (60%) no response was mentioned. Most of the patients (65%) had cryptogenic etiology for infantile spasms. Uncontrolled seizure was mentioned after initial remission. Methyl prednisolone is an appropriate drug based on easy administering, low cost, and its accessibility.

  6. Clinical spectrum of infantile spasm at presentation.

    Science.gov (United States)

    Malik, Muhammad Akbar; Tarrar, Muhammad Arif; Qureshi, Ahmad Osaid; Zia-Ur-Rehman, Muhammad

    2012-01-01

    To determine the clinical and EEG findings in children with infantile spasms at their initial presentation to the Neurophysiology Department, Children's Hospital, Lahore, Pakistan. Observational study. The Neurophysiology Department, Children's Hospital, Lahore, Pakistan, from January 2008 to December 2010. Children aged infantile spasms. Clinical manifestation, EEG finding and anti-epileptic drugs being administered on presentation were analyzed by the paediatric neurologists. Among the total 2050, 410 children (20%) had infantile spasms. Mean age at presentation was 4.6 + 3.5 months. Three hundred and twenty eight presented due to infantile spasms / seizures (80%) and 82 due to psychomotor delay / regression (20%). Seventy-two percent patients presented at the age Spasm types were mixed (56%), flexors (24%), extensor (12%) and asymmetric (8%). Etiology classification was symptomatic in 58% and cryptogenic in 42%. Autonomic disturbance, impaired consciousness and abnormal eye movements were the dominant initial clinical presentations. EEG records showed hypsarrhythmic/modified hypsarrhythmic in 82% and other forms of epileptic discharges in 18%. Hormonal therapy was being administered in 12%, 40% were receiving Phenobarbitone and 34% were not being treated with any anti-epileptic agent. Patients with infantile spasms have abnormal EEG findings predominantly the hypsarrhythmic modified hypsarrhythmic discharge. To avoid improper treatment, such patients should be referred to the specialized centres.

  7. Carisbamate acutely suppresses spasms in a rat model of symptomatic infantile spasms.

    Science.gov (United States)

    Ono, Tomonori; Moshé, Solomon L; Galanopoulou, Aristea S

    2011-09-01

    Infantile spasms are the signature seizures of West syndrome. The conventional treatments for infantile spasms, such as adrenocorticotropic hormone (ACTH) and vigabatrin, are not always effective, especially in symptomatic infantile spasms (SIS). We tested the efficacy of carisbamate, a novel neurotherapeutic drug, to suppress spasms in the multiple-hit rat model of SIS, and compared it with phenytoin to determine if its effect is via sodium-channel blockade. Sprague-Dawley rats received right intracerebral infusions of doxorubicin and lipopolysaccharide at postnatal day 3 (PN3) and intraperitoneal p-chlorophenylalanine at PN5. A single intraperitoneal injection of carisbamate was administered at PN4, after the onset of spasms, at the following doses: 10 mg/kg (CRS-10), 30 mg/kg (CRS-30), and 60 mg/kg (CRS-60), and was compared to vehicle-injected group (VEH). Video-monitoring of PN6-7 CRS-60 or VEH-injected pups was also done. Carisbamate acutely reduced both behavioral spasms (CRS-30 and CRS-60 groups only) and electroclinical spasms during the first 2-3 postinjection hours, without detectable toxicity or mortality. In contrast, phenytoin (20 or 50 mg/kg) failed to suppress spasms. Our findings provide preclinical evidence that carisbamate displays acute anticonvulsive effect on spasms through a sodium channel-independent mechanism. Because spasms in the multiple-hit rat model are refractory to ACTH and transiently sensitive to vigabatrin, carisbamate may constitute a candidate new therapy for SIS, including the ACTH-refractory spasms. Further confirmation with clinical studies is needed. Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.

  8. The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study.

    Science.gov (United States)

    O'Callaghan, Finbar J K; Lux, Andrew L; Darke, Katrina; Edwards, Stuart W; Hancock, Eleanor; Johnson, Anthony L; Kennedy, Colin R; Newton, Richard W; Verity, Christopher M; Osborne, John P

    2011-07-01

    Infantile spasms is a severe infantile seizure disorder. Several factors affect developmental outcome, especially the underlying etiology of the spasms. Treatment also affects outcome. Both age at onset of spasms and lead time to treatment (the time from onset of spasms to start of treatment) may be important. We investigated these factors. Developmental assessment using Vineland Adaptive Behaviour Scales (VABS) at 4 years of age in infants enrolled in the United Kingdom Infantile Spasms Study. Date of or age at onset of spasms was obtained prospectively. Lead time to treatment was then categorized into five categories. The effects of lead time to treatment, age of onset of spasms, etiology, and treatment on developmental outcome were investigated using multiple linear regression. Age of onset ranged (77 infants) from treatment was 7 days or less in 11, 8-14 days in 16, 15 days to 1 month in 8, 1-2 months in 15, >2 months in 21 and not known in 6. Each month of reduction in age at onset of spasms was associated with a 3.1 [95% confidence interval (CI) 0.64-5.5, p = 0.03] decrease, and each increase in category of lead time duration associated with a 3.9 (95% CI 7.3-0.4, p = 0.014) decrease in VABS, respectively. There was a significant interaction between treatment allocation and etiology with the benefit in VABS in those allocated steroid therapy being in children with no identified etiology (coefficient 29.9, p=0.004). Both prompt diagnosis and prompt treatment of infantile spasms may help prevent subsequent developmental delay. Younger infants may be more at risk from the epileptic encephalopathy than older infants. Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.

  9. Coronary spasm induced by dipyridamole

    International Nuclear Information System (INIS)

    Wartski, M.; Caussin, C.; Lancelin, B.

    2001-01-01

    A 59 years old man was admitted at hospital for recurrent instable angina 1 month after coronary artery bypass surgery. Coronary artery disease started with a transmural antero-septo-apical myocardial infarction without thrombolysis and a percutaneous angioplasty with endo-prothesis on proximal left anterior descendant artery (LAD) is performed Because of recurrent rest angina and subacute stent thrombosis, a coronary artery bypass surgery (CABG) is performed with anastomosis of the left internal thoracic artery on LAD. The patient is admitted for recurrent rest angina one month after CABG. On ECG performed during chest pain, a ST-T segment elevation occurred on inferior leads. Coronary angiography showed no significant stenosis on endo-prothesis and no bypass graft dysfunction. Dipyridamole scintigraphy was realized. 2 minutes after the beginning of Dipyridamole infusion, a ST-T elevation occurred on inferior leads and two marked antero-septal and inferior defects were noticed on myocardial scintigraphy. Images at rest showed a clear improvement in the anterior wall and the inferior wall became normally perfused Patient was treated with anti-spastic drugs and a new coronarography with methyl-ergotamine test was performed inducing chest pain, ST-T elevation on inferior leads and tri-truncular coronary spasm. Patient's treatment was then modified with introduction of Nifedipine. The patient did not experienced new recurrent chest pain and remained totally asymptomatic few months later. (authors)

  10. Current trends in the treatment of infantile spasms

    Directory of Open Access Journals (Sweden)

    Chang-Yong Tsao

    2009-05-01

    Full Text Available Chang-Yong TsaoClinical Pediatrics and Neurology, The Ohio State University, College of Medicine, Columbus, Ohio, USAAbstract: Infantile spasms are an epilepsy syndrome with distinctive features, including age onset during infancy, characteristic epileptic spasms, and specific electroencephalographic patterns (interictal hypsarrhythmia and ictal voltage suppression. Adrenocorticotropic hormone (ACTH was first employed to treat infantile spasms in 1958, and since then it has been tried in prospective and retrospective studies for infantile spasms. Oral corticosteroids were also used in a few studies for infantile spasms. Variable success in cessation of infantile spasms and normalization of electroencephalograms was demonstrated. However, frequent significant adverse effects are associated with ACTH and oral corticosteroids. Vigabatrin has been used since the 1990s, and shown to be successful in resolution of infantile spasms, especially for infantile spasms associated with tuberous sclerosis. It is associated with visual field constriction, which is often asymptomatic and requires perimetric visual field study to identify. When ACTH, oral corticosteroids, and vigabatrin fail to induce cessation of infantile spasms, other alternative treatments include valproic acid, nitrazepam, pyridoxine, topiramate, zonisamide, lamotrigine, levetiracetam, felbamate, ganaxolone, liposteroid, thyrotropin-releasing hormone, intravenous immunoglobulin and a ketogenic diet. Rarely, infantile spasms in association with biotinidase deficiency, phenylketonuria, and pyridoxine-dependent seizures are successfully treated with biotin, a low phenylalanine diet, and pyridoxine, respectively. For medically intractable infantile spasms, some properly selected patients may have complete cessation of infantile spasms with appropriate surgical treatments.Keywords: infantile spasms, adrenocorticotropic hormone, oral corticosteroids, vigabatrin

  11. Epileptic spasms in tuberous sclerosis complex.

    Science.gov (United States)

    Hsieh, David T; Jennesson, Melanie M; Thiele, Elizabeth A

    2013-09-01

    To characterize epileptic spasms (ES) occurring after the age of two years in patients with tuberous sclerosis complex (TSC), particularly treatment response to vigabatrin (VGB), which is extremely effective for infantile spasms (IS) in TSC. The authors retrospectively reviewed 19 patients with TSC and ES. Medical records were assessed for clinical and treatment data, neurocognitive, EEG, MRI data, and genetic analyses. Of 391 patients with TSC, 19 (4.8%) had ES. Of those with detailed clinical data, six had infantile spasms that persisted after 2 years old, six recurred after an initial remission of infantile spasms (range 2-24 years old), and four occurred de novo over the age of two (range 2-20 years old). All concurrently had other seizure types. One had hypsarrhythmia on EEG. All had brain MRI stigmata typical of TSC. Thirteen had a mutation in TSC2, and one in TSC1. Six patients became spasm-free with medication treatment, including four with VGB, one with VGB in combination with the low glycemic index dietary treatment, and one with felbamate. Five became spasm-free after epilepsy surgery. VGB was not effective for seven patients. The majority continued to have refractory epilepsy. ES are not uncommon in patients with TSC, especially those with TSC2 mutations. ES in TSC occur in the setting of other seizure types and refractory epilepsy. Hypsarrhythmia is rare. VGB can be effective, but the success of VGB for ES in TSC is not equivalent to that of IS in TSC. Copyright © 2013 Elsevier B.V. All rights reserved.

  12. Ulnar Artery Compression: A Feasible and Effective Approach to Prevent the Radial Artery Occlusion after Coronary Intervention

    Directory of Open Access Journals (Sweden)

    Jun Tian

    2015-01-01

    Full Text Available Background: Radial artery (RA occlusion (RAO is not rare in patients undergoing coronary intervention by transradial approach (TRCI. Predictors of and prevention from RAO have not been systematically studied. This study aimed to analyze the risk factors of the weakness of RA pulsation (RAP and its predictive value for RAO after TRCI, and simultaneously to describe a feasible and effective approach to maintain RA patency. Methods: Between June 2006 and March 2010, all patients who underwent TRCI were classified according to the weakness of RAP after removing compression bandage with confirmation by Doppler ultrasound for the first 30 consecutive patients. Among a total of 2658 patients studied, 187 (7% patients having a weaker RAP were prospectively monitored. At 1 h after bandage removal, the ulnar artery in puncture side of all patients was blocked with manual compression to favor brachial and collateral artery blood flow through the RA until a good RAP was restored. The primary analysis was the occurrence of RAO. Results: Doppler ultrasound demonstrated the significant reduction of both systolic velocity (61.24 ± 3.95 cm/s vs. 72.31 ± 3.57 cm/s and diastolic velocity (1.83 ± 0.32 cm/s vs. 17.77 ± 3.97 cm/s in RA at access side as compared to the contralateral RA (all P < 0.001, but these velocities in ipsilateral ulnar artery (81.2 ± 2.16 cm/s and 13.1 ± 2.86 cm/s, respectively increased profoundly. The average time of ulnar artery compression was 4.1 ± 1.2 h (ranged 2.5-6.5 h. There were two patients experienced persistent RAO with a success rate of 98.9% and RAO in 0.075% of patients after ulnar artery compression was applied. The pulsation of the ulnar artery after compression was removed had not been influenced by the compression. Conclusions: After intervention using TRCI approach, the presence of a weaker RAP is an indicator of imminent RAO. The continuing compression of ipsilateral ulnar artery is an effective approach to

  13. Periodic Eye Movements and Epileptic Spasms in West Syndrome.

    Science.gov (United States)

    Kakisaka, Yosuke; Kobayashi, Tomoko; Hino-Fukuyo, Naomi; Uematsu, Mitsugu; Numata, Yurika; Mori, Masato; Kure, Shigeo

    2013-11-01

    In addition to the typical infantile spasm symptoms, several other symptoms, such as eye movements, have been reported to be associated with infantile spasms, although the relationship between the typical spasms and these other events is not fully understood. Here we present a case with West syndrome. We observed the appearance of periodic eye movements followed by the onset of typical spasms and the appearance/disappearance of periodic eye movements during withdrawal/increases of vigabatrin. We believe that the case strongly supports the notion that periodic eye movements and typical spasms represent a spectrum of symptoms related to the same phenomenon of West syndrome.

  14. Serotonin, atherosclerosis, and collateral vessel spasm

    Science.gov (United States)

    Hollenberg, N.

    1988-01-01

    Studies on animal models demonstrate that platelet products contribute to vascular spasm in ischemic syndromes and that this is reversible with administration of ketanserin and thromboxane synthesis inhibitors. Laboratory animals (dogs, rabbits, and rats) that had femoral artery ligations exhibited supersensitivity to serotonin within days in their collateral blood vessels. This supersensitivity lasted at least 6 months. The response to serotonin was reversed by ketanserin, but not by 5HT-1 antagonists. Supersensitivity does not extend to norepinephrine, and alpha blockers do not influence the response to serotonin. It appears that platelet activation by endothelial injury contributes to ischemia through blood vessel occlusion and vascular spasm. When platelet activation occurs in vivo, blood vessel occlusion and vascular spasm are reversible in part by using ketanserin or agents that block thromboxane synthesis or its action. Combining both classes of agents reverses spasm completely. These findings support existing evidence that platelet products contribute to vascular disease, and provide an approach to improved management with currently available pharmacologic agents.

  15. PROLONGED MULTIPLE SPASMS OF SMOOTH CORONARY ARTERIES PRESENTING AS ACUTE MIOCARDIAL INFARCTION, COMPLETE AV BLOCK AND SYNCOPE

    Directory of Open Access Journals (Sweden)

    Franci Cesar

    2004-11-01

    Full Text Available Background. A variant form of angina pectoris (VAP is caused by coronary vessel spasm and occures in patients with and without varying degrees of obstructive coronary artery disease. Although the prognosis of VAP without significant organic stenosis is generally good, multivessel spasm is associated with a high risk of life-threatening abnormalities of rhythm and conduction.Patient and methods. We describe a patient who presented with prolonged chest pain, associated with hypotension, lost of consciousness, complete AV block and widespread ST segment elevations consistent with inferoanterior acute myocardial infarction. Urgent selective coronary angiography revealed spasms in right coronary artery and in left circumflex artery that were relieved by intracoronary injection of nitroglycerin. All coronary arteries were otherwise patient, without signs of atherosclerosis. The patient was treated with diltiazem and nitrates. She made a complete recovery and resumed her normal activities.Conclusions. Simultaneous multiple spasms of native coronary arteries represent a rare syndrome characterized by significantly higher incidence of potentially life-threatening arrhythmia. Less commonly, prolonged coronary spasm may mimic acute myocardial infarction. Modern management of acute coronary syndromes, including urgent coronarography, enables a prompt differentiation between prolonged coronary spasm and atherosclerotic coronary disease, warranting different treatment strategies. Medical treatment with nitrates and calcium channel blockers in most cases prevents recurrence of vasospasms and arrhythmias.

  16. Association between infantile spasms and nonaccidental head injury.

    Science.gov (United States)

    Birca, Ala; D'Anjou, Guy; Carmant, Lionel

    2014-05-01

    Infantile spasms constitute a severe epileptic encephalopathy of infancy with poor long-term developmental outcome. Many diverse etiologies have been associated with infantile spasms, but the pathophysiological process is still not fully understood. We describe 2 cases of previously healthy 1- and 3-month-old infants who suffered a nonaccidental head injury with extensive cerebral lesions. Both presented with acute focal seizures rapidly controlled with phenobarbital. Nevertheless, they developed infantile spasms after a latency period of 3-4 months. Spasms were rapidly controlled with vigabatrin. Both children manifested with developmental delay, either exacerbated (case 1) or elicited (case 2) by infantile spasms. Our report highlights nonaccidental head injury as a risk factor for developing infantile spasms following a seizure-free latency period. A better understanding of the pathophysiology linking accidental brain trauma with infantile spasms could lead to more effective neuroprotective strategies. In the meantime, increased awareness and follow-up are warranted.

  17. Infraslow EEG changes in infantile spasms.

    Science.gov (United States)

    Myers, Kenneth A; Bello-Espinosa, Luis E; Wei, Xing-Chang; Scantlebury, Morris H

    2014-12-01

    Infantile spasms (IS) are a devastating epileptic encephalopathy syndrome of infancy. Analysis of infraslow EEG activity (ISA) has shown potential in the presurgical evaluation of patients with epilepsy and in differentiating between focal and generalized epilepsy syndromes. Infraslow EEG activity analysis may provide insights into the pathophysiology of some difficult-to-treat epilepsy syndromes, such as IS. To our knowledge, there are no published reports describing ISA in patients with IS. The purpose of this study was to describe ictal patterns of ISA in patients with IS and to correlate with clinical data. EEG recordings of all cases of IS in the past 10 years at the Alberta Children's Hospital were reviewed. Inclusion criteria were a technically adequate video EEG recording that captured at least one spasm. For each patient, the first 10 confirmed spasms were examined. Spasms were evaluated for changes in ISA, which were either generalized, lateralized, or absent ISA (g-ISA, l-ISA, or n-ISA, respectively). Results were correlated with treatments, clinical course, and information pertinent to likely etiology of the IS. A total of 77% of spasms were associated with ISA; 57% with g-ISA, 20% l-ISA, and 21% n-ISA. All patients with exclusively g-ISA showed at least a partial response to initial therapy, while this was the case in 66.7% of those with at least some l-ISA and 50% of those with exclusively n-ISA. Other seizure types occurred in 60% of patients with exclusively g-ISA versus 83% with some l-ISA and all patients with exclusively n-ISA. Ictal ISA was observed in the majority of IS. Trends were observed suggesting that the presence of exclusive g-ISA changes may be a positive prognostic factor in IS.

  18. Validation of the rat model of cryptogenic infantile spasms

    Science.gov (United States)

    Chachua, Tamar; Yum, Mi-Sun; Velíšková, Jana; Velíšek, Libor

    2011-01-01

    Purpose To determine whether a new model of cryptogenic infantile spasms consisting of prenatal priming with betamethasone and postnatal trigger of spasms by N-methyl-D-aspartic acid responds to chronic ACTH treatment, and has similar EEG signature, efficacy of treatments, and behavioral impairments as human infantile spasms. Methods Rats prenatally primed with betamethasone on gestational day 15 were used. Spasms were triggered with N-methyl-D-aspartic acid between postnatal days (P) 10-15 in a single session or in multiple sessions in one subject. The expression of spasms was compared to prenatally saline-injected controls. Effects of relevant treatments (ACTH, vigabatrin, methylprednisolone, rapamycin) were determined in betamethasone-primed rats. In the rats after spasms, behavioral evaluation was performed in the open field and and elevated plus maze on P20-22. Key Findings NMDA at P10-15 (the rat “infant” period) triggers the spasms significantly earlier and in greater numbers in the prenatal betamethasone-exposed brain compared to controls. Similar to human condition, the spasms occur in clusters. Repeated trigger of spasms is associated with ictal EEG electrodecrements and interictal large-amplitude waves, a possible rat variant of hypsarrhythmia. Chronic ACTH treatment in a randomized experiment, and chronic pretreatment with methylprednisolone significantly suppress number of spasms similar to human condition. Pretreatment with vigabatrin, but not rapamycin, suppressed the spasms. Significant behavioral changes occurred following multiple bouts of spasms. Significance The model of infantile spasms has remarkable similarities with the human condition in semiology, EEG, pharmacological response, and long-term outcome. Thus, the model can be used for search of novel and more effective treatments for infantile spasms. PMID:21854372

  19. Seizure outcome in infantile spasms--a retrospective study.

    Science.gov (United States)

    Mohamed, Basheer Peer; Scott, Rod C; Desai, Nivedita; Gutta, Pranathi; Patil, Shekhar

    2011-04-01

    Prior to the United Kingdom Infantile Spasms Study (UKISS), our practice was to initiate vigabatrin for infantile spasms. However, since then we tend to use steroids as first-line agent for infantile spasms. Herein we compare seizure-free outcomes in children with infantile spasms on steroid therapy or vigabatrin therapy. This was a retrospective case study over 8 years of children with infantile spasms who were treated at our center. A positive response to therapy was defined as a two-week spasm-free interval. Of the 98 children presenting to us, 75 were included for this study. The ratio of cryptogenic to symptomatic spasms was 24:51. The response rate for steroid therapy was 61.1% and 42.5% for vigabatrin. Cessation of spasms was achieved faster in the group receiving steroids. Both groups had similar relapse rates. Steroids had significantly better response in the cryptogenic group, whereas in the symptomatic group both the medications were equally effective. Cryptogenic spasms have a better neurodevelopmental outcome. Early introduction of therapy for spasms did not predict a good neurodevelopmental outcome. Seventy-eight percent of children with spasms had seizures of other types at 12 months follow-up. At our center, steroids are now the preferred choice for initial therapy of infantile spasms. This is likely to have been a beneficial change, particularly for children with cryptogenic spasms. Spasms in 25% of the patients tend to be refractory, and the majority of patients from the cohort continue to have epilepsy with motor and cognitive disabilities. Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.

  20. Estradiol does not affect spasms in the betamethasone-NMDA rat model of infantile spasms.

    Science.gov (United States)

    Chachua, Tamar; Di Grazia, Paola; Chern, Chian-Ru; Johnkutty, Meenu; Hellman, Benjamin; Lau, Ho An; Shakil, Faariah; Daniel, Margaret; Goletiani, Cezar; Velíšková, Jana; Velíšek, Libor

    2016-08-01

    This study attempted to validate the effects of neonatal estradiol in ameliorating the spasms in the prenatally betamethasone-primed N-methyl-d-aspartate (NMDA) model of infantile spasms in rats as shown previously in a mouse Arx gene knock-in expansion model of infantile spasms. Neonatal rats prenatally exposed to betamethasone (on day 15 of pregnancy) were treated with subcutaneous 40 ng/g estradiol benzoate (EB) between postnatal days (P)3-P10 or P0-P5. A synthetic estrogen analogue, diethylstilbestrol, was used between P0 and P5 (2 μg per rat, s.c.). On P12, P13, and P15, the rats were subjected to NMDA-triggered spasms, and latency to onset and number of spasms were evaluated. Rats with EB on P3-P10 were tested after spasms in the open field, novel object recognition, and elevated plus maze to determine effects of treatment on behavior. Additional rats with P3-P10 or P0-P5 EB were investigated for γ-aminobutyric acid (GABA)ergic neurons (glutamate decarboxylase [GAD]67 expression) in the neocortex. As a positive control, a group of rats received either subcutaneous adrenocorticotropic hormone (ACTH) (2 × 0.3 mg/kg on P12 and 3 × 0.3 mg/kg on P13 and P14) or vehicle after the first episode of spasms on P12. Neither EB treatment nor diethylstilbestrol consistently affected expression of spasms in this model, although we found a significant increase in GAD67-immunopositive cells in the neocortex after P3-P10 and P0-P5 EB treatment, consistent with a study in mice. Behavioral tests showed increase in lateralization in male rats treated with P3-P10 EB, a behavioral trait usually associated with female sex. Diethylstilbestrol treatment in male rats resulted in arrested pubertal descent of testes. ACTH had robust effects in suppressing spasms. Treatment of infantile spasms (IS) using neonatal EB may be justified in those cases of IS that present with detectable deficits in GABAergic neurons. In other types of IS, the efficacy of neonatal EB and its analogues is

  1. Proteomic analysis on infantile spasm and prenatal stress.

    Science.gov (United States)

    Wang, Jing; Wang, Juan; Zhang, Ying; Yang, Guang; Shang, Ai-Jia; Zou, Li-Ping

    2014-09-01

    Infantile spasms (IS) are an age-dependent epileptic encephalopathy with severe cognitive dysfunction. Prenatal stress (PS) has been reported to increase the risk for IS through clinical and animal studies. We aim to investigate the mechanism of brain damage caused by IS and the effect of PS. Animals were divided into 4 groups: PS-spasm model, PS-saline control, NS-spasm model, and saline control. N-methyl-d-aspartate (NMDA) was used to induce spasm and swimming in cold water was used to induce PS. A proteomics-based approach was used to compare the NS-spasm model vs. saline control, and PS-spasm model vs. NS-spasm model. Gel image analysis was followed by mass spectrometric protein identification and bioinformatics analysis. We observed an increased spasm frequency (t=8.65, Pspasm model vs. the NS-spasm model. In the NS-spasm model vs. saline control, the main differentially expressed proteins were CFL1, PKM2, PRPS2, DLAT, CKB, DPYSL3, and SNAP25. In the PS-spasm model vs. NS-spasm model, MDH1 and YWHAZ were differentially expressed. YWHAZ was directly connected with CFL1 in protein networks. YWHAZ and CFL1 were further validated by Western blot analysis. The biological function of differentially expressed proteins indicates the pathogenesis of IS maybe relevant to energy metabolism, brain development, and neural remodeling. PS aggravated seizures in the NMDA-induced spasm model, YWHAZ, and CFL1 may be involved. Copyright © 2014 Elsevier B.V. All rights reserved.

  2. Origin and Propagation of Epileptic Spasms Delineated on Electrocorticography

    Science.gov (United States)

    Asano, Eishi; Juhász, Csaba; Shah, Aashit; Muzik, Otto; Chugani, Diane C.; Shah, Jagdish; Sood, Sandeep; Chugani, Harry T.

    2005-01-01

    Summary Purpose Ictal electrographic changes were analyzed on intracranial electrocorticography (ECoG) in children with medically refractory epileptic spasms to assess the dynamic changes of ictal discharges associated with spasms and their relation to interictal epileptiform activity and neuroimaging findings. Methods We studied a consecutive series of 15 children (age 0.4 to 13 years; nine girls) with clusters of epileptic spasms recorded on prolonged intracranial subdural ECoG recordings, which were being performed for subsequent cortical resection, and in total, 62 spasms were analyzed by using quantitative methods. Results Spasms were associated with either a “leading” spike followed by fast-wave bursts (type I: 42 events analyzed quantitatively) or fast-wave bursts without a “leading” spike (type II: 20 events analyzed quantitatively). Twenty-three of the 42 type I spasms but none of the 20 type II spasms were preceded by a focal seizure. A “leading” spike had a focal origin in all 42 type I spasms and involved the pre- or postcentral gyrus within 0.1 s in 37 of these spasms. A leading spike was associated with interictal spike activity >1/min in 40 of 42 type I spasms and originated within 2 cm from a positron emission tomography glucose hypometabolic region in all but two type I spasms. Failure to resect the cortex showing a leading spike was associated with poor surgical outcome (p = 0.01; Fisher’s exact probability test). Fast-wave bursts associated with spasms involved neocortical regions extensively at least in two lobes within 1.28 s in all 62 spasms and involved the pre- or postcentral gyrus in 53 of 62 spasms. Conclusions Epileptic spasms may be triggered by a focal neocortical impulse in a subset of patients, and a leading spike, if present, might be used as a marker of the trigger zone for epileptic spasms. Rapidly emerging widespread fast-wave bursts might explain the clinical semiology of epileptic spasms. PMID:16026561

  3. Infantile Spasms: Little Seizures, BIG Consequences

    Science.gov (United States)

    Shields, W Donald

    2006-01-01

    Infantile spasms is one of the “catastrophic childhood epilepsies” because of the difficulty in controlling seizures and the association with mental retardation. However, early recognition, a careful diagnostic evaluation, and proper treatment may allow some children to attain seizure control and to achieve a normal, or at least much improved, level of development. Thus, there is the opportunity to have an important impact in the lives of these unfortunate children and their families. PMID:16761063

  4. Risk factors affecting prognosis in infantile spasm.

    Science.gov (United States)

    Gul Mert, Gulen; Herguner, Mihriban Ozlem; Incecik, Faruk; Altunbasak, Sakir; Sahan, Duygu; Unal, Ilker

    2017-11-01

    To assess risk factors that affect epilepsy prognosis and neurodevelopmental outcome and response to treatment in patients diagnosed with infantile spasm. In this study, demographics, treatment modalities, etiologies, risk factors affecting neurodevelopmental outcome and epilepsy prognosis were assessed retrospectively at the end of a minimum 24-months follow-up of 104 patients diagnosed with infantile spasm from May 2012 to October 2015. Neonatal seizure during neonatal period, abnormal head circumference, young age at the time of presentation and early gestational age, symptomatic etiology, abnormal initial examination and abnormal development test at the time of diagnosis, consanguinity, the medical center where treatment was started in the second center or beyond and magnetic resonance imaging finding were found to be statistically significant for poor prognosis in terms of neurodevelopment (p Infantile spasm is an age-related epileptic encephalopathy, and it was observed that it is still catastrophic, and that the most important factor affecting prognosis of epilepsy is etiology, age at the time of presentation and the medical center where treatment was started in the second center or beyond.

  5. Current trends in the treatment of infantile spasms

    OpenAIRE

    Tsao, Chang-Yong

    2009-01-01

    Chang-Yong TsaoClinical Pediatrics and Neurology, The Ohio State University, College of Medicine, Columbus, Ohio, USAAbstract: Infantile spasms are an epilepsy syndrome with distinctive features, including age onset during infancy, characteristic epileptic spasms, and specific electroencephalographic patterns (interictal hypsarrhythmia and ictal voltage suppression). Adrenocorticotropic hormone (ACTH) was first employed to treat infantile spasms in 1958, and since then it has been tried in pr...

  6. Clinical profile and response to oral prednisolone in infantile spasm.

    Science.gov (United States)

    Noureen, Nuzhat; Rana, Muhammad Tariq

    2010-03-01

    To evaluate the clinical profile and response to oral prednisolone in infantile spasms. Case series. Neurology Department, The Children Hospital and Institute of Child Health, Multan, from July 2005 to June 2007. Fifty patients of infantile spasms were studied. Age, gender, age at onset of seizures, type of spasms (flexor, extensor or mixed), history of intrapartum asphyxia, developmental history, dysmorphic facial features, any hypopigmented/ hyperpigmented skin lesions, computed tomogram and electroencephalogram findings and response to oral prednisolone was noted. Data was analyzed statistically by SPSS 10. Descriptive statistics was used to find out frequencies and percentages of all above mentioned variables. Chi-square test was applied to determine the association between these variables and response to treatment. P-value of less than 0.05 was taken significant. Male to female ratio was 2.1:1. Mean age of babies was 6.5+/-3.35 months. Mean age at onset of seizures was 5.35+/-3.52 months. Flexor spasms was seen in 32 (64%), extensor spasms in 8 (16%) and mixed spasms in 10 babies (20%). Symptomatic infantile spasms were noted in 48 (96%) babies while two babies (4%) were having cryptogenic infantile spasm. History of intrapartum asphyxia was noted in 54% of symptomatic cases. Favourable response to oral prednisolone was seen in 27 babies (54%). Except male gender, none of the other variable reached the statistical significance for favourable response to treatment. Infantile spasms were found more common in males, flexor spasms were the commonest type noted. Symptomatic spasm was noted in 96% of cases and intrapartum asphyxia was the commonest cause of symptomatic group. Response to oral prednisolone was noted in more than half of cases of infantile spasms.

  7. [Unstable angina pectoris--combination of an epicardial stenosis and a Prinzmetal spasm].

    Science.gov (United States)

    Bentz, K; Ong, P; Sechtem, U

    2013-12-01

    A 61-year-old man presented with recurrent angina pectoris at rest for 3 days. The medical history revealed hypertension and an elevated cholesterol level as cardiovascular risk factors. The physical examination revealed no pathological findings. ECG at admission showed no signs of ischemia, while high-sensitive Troponin T was slightly elevated. Echocardiography showed diastolic dysfunction and biatrial dilatation. During another episode of angina at rest, ECG showed T-wave inversion in lead aVL. Therefore, coronary angiography was performed. At the beginning of the examination the patient complained of angina at rest and ECG showed ST-elevation in the inferior leads. Coronary angiography revealed a subtotal stenosis in the middle part of the RCA. After intracoronary nitroglycerin injection there was a high-grade stenosis in this region. An intracoronary acetycholin provocation test was performed which reproduced a focal spasm in the area of the RCA stenosis with simultaneous changes in the ECG and reproduction of the patient's unusual angina. After implantation of a bare metal stent a subsequent ACH-test did not elicit any further coronary spasm. Four weeks after the procedure the patient had no further complaints under medical treatment. This case illustrates a patient with a focal Prinzmetal-type spasm with ST-elevation on top of a high-grade stenosis of the right coronary artery as an explanation for the unstable angina. Coronary spasm of the Prinzmetal-type can occur in vessels with epicardial stenosis as well as in vessels without stenosis. In some cases focal coronary spasm can be prevented by the implantation of a stent. © Georg Thieme Verlag KG Stuttgart · New York.

  8. Infantile Spasms and Cytomegalovirus Infection: Antiviral and Antiepileptic Treatment

    Science.gov (United States)

    Dunin-Wasowicz, Dorota; Kasprzyk-Obara, Jolanta; Jurkiewicz, Elzbieta; Kapusta, Monika; Milewska-Bobula, Bogumila

    2007-01-01

    From 1 January 1995 to 31 December 2004, 22 patients (13 males, nine females; age range 2-12mo) with infantile spasms and cytomegalovirus (CMV) infection were treated with intravenous ganciclovir (GCV) and antiepileptic drugs. GCV was given for 3 to 12 weeks with a 1-month interval (one, two, or three courses). Epileptic spasms occurred before…

  9. Part Two: Infantile Spasms--The New Consensus

    Science.gov (United States)

    Pellock, John M.; O'Hara, Kathryn

    2011-01-01

    This article presents the conclusion made by the consensus group regarding infantile spasms. The consensus group concluded that "infantile spasms are a major form of severe epileptic encephalopathy of early childhood that results in neurodevelopmental regression and imposes a significant health burden." The entire group agrees that the best…

  10. Detailed Magnetic Resonance Imaging (MRI) Analysis in Infantile Spasms.

    Science.gov (United States)

    Harini, Chellamani; Sharda, Sonal; Bergin, Ann Marie; Poduri, Annapurna; Yuskaitis, Christopher J; Peters, Jurriaan M; Rakesh, Kshitiz; Kapur, Kush; Pearl, Phillip L; Prabhu, Sanjay P

    2018-01-01

    To evaluate initial magnetic resonance imaging (MRI) abnormalities in infantile spasms, correlate them to clinical characteristics, and describe repeat imaging findings. A retrospective review of infantile spasm patients was conducted, classifying abnormal MRI into developmental, acquired, and nonspecific subgroups. MRIs were abnormal in 52 of 71 infantile spasm patients (23 developmental, 23 acquired, and 6 nonspecific) with no correlation to the clinical infantile spasm characteristics. Both developmental and acquired subgroups exhibited cortical gray and/or white matter abnormalities. Additional abnormalities of deep gray structures, brain stem, callosum, and volume loss occurred in the structural acquired subgroup. Repeat MRI showed better definition of the extent of existing malformations. In structural infantile spasms, developmental/acquired subgroups showed differences in pattern of MRI abnormalities but did not correlate with clinical characteristics.

  11. A resorbable antibiotic-eluting polymer composite bone void filler for perioperative infection prevention in a rabbit radial defect model.

    Directory of Open Access Journals (Sweden)

    Benjamin D Brooks

    Full Text Available Nearly 1.3 million total joint replacement procedures are performed in the United States annually, with numbers projected to rise exponentially in the coming decades. Although finite infection rates for these procedures remain consistently low, device-related infections represent a significant cause of implant failure, requiring secondary or revision procedures. Revision procedures manifest several-fold higher infection recurrence rates. Importantly, many revision surgeries, infected or not, require bone void fillers to support the host bone and provide a sufficient tissue bed for new hardware placement. Antibiotic-eluting bone void fillers (ABVF, providing both osteoconductive and antimicrobial properties, represent one approach for reducing rates of orthopedic device-related infections. Using a solvent-free, molten-cast process, a polymer-controlled antibiotic-eluting calcium carbonate hydroxyapatite (HAP ceramic composite BVF (ABVF was fabricated, characterized, and evaluated in vivo using a bacterial challenge in a rabbit radial defect window model. ABVF loaded with tobramycin eliminated the infectious burden in rabbits challenged with a clinically relevant strain of Staphylococcus aureus (inoculum as high as 10⁷ CFU. Histological, microbiological, and radiographic methods were used to detail the effects of ABVF on microbial challenge to host bone after 8 weeks in vivo. In contrast to the HAP/BVF controls, which provided no antibiotic protection and required euthanasia 3 weeks post-operatively, tobramycin-releasing ABVF animals showed no signs of infection (clinical, microbiological, or radiographic when euthanized at the 8-week study endpoint. ABVF sites did exhibit fibrous encapsulation around the implant at 8 weeks. Local antibiotic release from ABVF to orthopedic sites requiring bone void fillers eliminated the periprosthetic bacterial challenge in this 8-week in vivo study, confirming previous in vitro results.

  12. [Approaches to radial shaft].

    Science.gov (United States)

    Bartoníček, J; Naňka, O; Tuček, M

    2015-10-01

    . During release and retraction of the supinator posterolaterally, it is beneficial to supinate the proximal fragment of the shaft as much as possible, preferably by K-wire drilled perpendicular into the anterior surface of the fragment and rotated externally. As a result, canalis supinatorius is moved posteriorly which reduces the risk of injury to the deep branch of the radial nerve. The supinator is released always from distal to proximal. Approximately at the level of the biceps brachii tendon, it is usually necessary to identify and ligate the radial recurrent artery and vein which prevent retraction of the radial vessels medially. After detachment of the whole supinator, a small Hohmann elevator is carefully inserted between the muscle and the bone. If necessary, it is now possible to open the anterior surface of the joint capsule and revise the humeroradial joint.

  13. UK Infantile Spasms Study: Effect of Time to Treatment and Age at Onset on Developmental Outcome

    OpenAIRE

    J Gordon Millichap

    2011-01-01

    The effects of lead time to treatment (time from onset of spasms to start of treatment), age at onset of spasms, etiology, and treatment on developmental outcome at 4 years were investigated using multiple linear regression in 77 infants with spasms treated in the UK Infantile Spasms Study (UKISS).

  14. Evidence-based guideline update: Medical treatment of infantile spasms

    Science.gov (United States)

    Go, C.Y.; Mackay, M.T.; Weiss, S.K.; Stephens, D.; Adams-Webber, T.; Ashwal, S.; Snead, O.C.

    2012-01-01

    Objective: To update the 2004 American Academy of Neurology/Child Neurology Society practice parameter on treatment of infantile spasms in children. Methods: MEDLINE and EMBASE were searched from 2002 to 2011 and searches of reference lists of retrieved articles were performed. Sixty-eight articles were selected for detailed review; 26 were included in the analysis. Recommendations were based on a 4-tiered classification scheme combining pre-2002 evidence and more recent evidence. Results: There is insufficient evidence to determine whether other forms of corticosteroids are as effective as adrenocorticotropic hormone (ACTH) for short-term treatment of infantile spasms. However, low-dose ACTH is probably as effective as high-dose ACTH. ACTH is more effective than vigabatrin (VGB) for short-term treatment of children with infantile spasms (excluding those with tuberous sclerosis complex). There is insufficient evidence to show that other agents and combination therapy are effective for short-term treatment of infantile spasms. Short lag time to treatment leads to better long-term developmental outcome. Successful short-term treatment of cryptogenic infantile spasms with ACTH or prednisolone leads to better long-term developmental outcome than treatment with VGB. Recommendations: Low-dose ACTH should be considered for treatment of infantile spasms. ACTH or VGB may be useful for short-term treatment of infantile spasms, with ACTH considered preferentially over VGB. Hormonal therapy (ACTH or prednisolone) may be considered for use in preference to VGB in infants with cryptogenic infantile spasms, to possibly improve developmental outcome. A shorter lag time to treatment of infantile spasms with either hormonal therapy or VGB possibly improves long-term developmental outcomes. PMID:22689735

  15. Treatment of infantile spasms with sodium valproate followed by benzodiazepines.

    Science.gov (United States)

    Auvichayapat, Narong; Tassniyom, Sompon; Treerotphon, Sutthinee; Auvichayapat, Paradee

    2007-09-01

    To review the result of the infantile spasms' treatment with sodium valproate followed by nitrazepam or clonazepam. Descriptive retrospective study. Srinagarind Hospital, Department of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand. Twenty-four infantile spasms admitted between January 1994 and December 2003 were analyzed. The inclusion criteria were the patients with infantile spasms clinically diagnosed by the pediatric neurologist, having hypsarrhythmic pattern EEG, and receiving sodium valproate with or without nitrazepam or clonazepam. The patients who had an uncertain diagnosis, incomplete medical record, or that were incompletely followed up were excluded. Data were collected on sex, age at onset of seizure, type of infantile spasms, associated type of seizure, predisposing etiological factor, neuroimaging study, and the result of treatment including cessation of spasms, subsequent development of other seizure types, quantitative reduction of spasms, relapse rates of spasms, psychomotor development, and adverse effects of AEDs. The mean age at onset was 177 days. The male-to-female ratio was 1:1.2. There were 13 cryptogenic (54.2%) and 11 symptomatic (45.8%) infantile spasms. The most common predisposing etiological factors in symptomatic cases were hypoxic ischemic encephalopathy (45.5%) and microcephaly (36.4%), respectively. Ten patients received sodium valproate (41.7%), another 10 received sodium valproate with clonazepam (41.7%), and four received sodium valproate with nitrazepam (16.7%). Both, the complete cessation rate and the 50% reduction of spasms rate were 45.8%. The duration to complete cessation was 70 days. The relapse rate was 18.2%. The rate of delayed psychomotor development was 83.3%. The mean duration of follow-up was 49.6 months. The authors propose to use sodium valproate concomitantly with benzodiazepines, especially clonazepam, in situations such as unavailability, intolerability, or adverse effects of

  16. Laryngeal spasm after general anaesthesia due to Ascaris Lumbricoides

    OpenAIRE

    Finsnes, K D

    2013-01-01

    Postoperative upper airway obstruction during recovery from general anaesthesia may have several causes. This is a report of a young girl who developed laryngeal spasm as a result of an ectopic roundworm Ascaris lumbricoides.

  17. Latest American and European updates on infantile spasms.

    Science.gov (United States)

    Lux, Andrew L

    2013-03-01

    Infantile spasms remain a challenging condition to study and treat, and although they form the commonest epilepsy syndrome with onset in infancy, the challenge is broadened by the wide range of potential underlying causes. The field of study remains dynamic, with debates relating to case definitions and organising structures for classification of seizures and epilepsies in general, and a newly proposed genetic and biologic classification specifically for infantile spasms. There have been recent consensus statements, a Delphi process eliciting prioritised quality-of-care indicators, systematic reviews of treatment, and a survey of clinical practice in the USA. There is increasing evidence that longer duration of spasms is associated with poorer neurodevelopmental outcomes. It has taken many years to develop an animal model that reasonably represents infantile spasms, but there are now several animal models, and they are leading to innovative and valuable studies that suggest novel treatments.

  18. Role of ARX Gene in Infantile Spasms and Dystonia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2007-08-01

    Full Text Available The role of ARX gene in a syndrome of infantile spasms with generalized dystonia was investigated in 6 boys from 4 families at the University of Florence, Italy, and other centers in Italy, Japan, and USA.

  19. Improving Outcomes in Infantile Spasms: Role of Pharmacotherapy.

    Science.gov (United States)

    Iyer, Anand; Appleton, Richard

    2016-10-01

    Infantile spasms, and specifically within the context of West syndrome , is one of the most common epileptic encephalopathies to occur in early infancy. Early recognition and treatment can improve neurodevelopmental outcome in some cases, although the underlying aetiology is probably the most important prognostic factor in both spasm suppression and developmental outcome. Corticosteroids, either adrenocorticotrophic hormone (ACTH) or prednisolone, and vigabatrin are currently the preferred first-line treatment options. Vigabatrin is the treatment of choice when the underlying cause is tuberous sclerosis complex (TSC). Emerging evidence suggests that a combination of steroid and vigabatrin may be more effective in the suppression of spasms and resolution of hypsarrhythmia, the electro-encephalographic signal of spasms. Several other anti-epileptic drugs (AEDs) (levetiracetam, nitrazepam, sodium valproate, topiramate, zonisamide) are usually used as add-on or adjunctive treatment in refractory cases. Pyridoxine (or pyridoxal phosphate) and the ketogenic diet are established treatment options in refractory cases. There is some evidence that neuro-active steroids, including ganaxolone, may be effective; however, clinical trials undertaken intermittently for over a decade have yet to prove their efficacy, not only for the suppression of infantile spasms but also for the resolution of hypsarrhythmia, which may be as important as seizure control in developmental outcome in these children. Insights into developing novel treatment options have emerged from rodent models of infantile spasms, and research is continuing into the efficacy of rapamycin in improving outcomes in infantile spasms. This review provides a brief overview of the existing scientific literature around treatment options and outlines emerging newer treatment options in infantile spasms.

  20. Structural brain alterations in hemifacial spasm: A voxel-based morphometry and diffusion tensor imaging study.

    Science.gov (United States)

    Tu, Ye; Yu, Tian; Wei, Yongxu; Sun, Kun; Zhao, Weiguo; Yu, Buwei

    2016-02-01

    Hemifacial spasm (HFS) is characterized by involuntary, irregular clonic or tonic movement of muscles innervated by the facial nerve. We evaluated structural reorganization in brain gray matter and white matter and whether neuroplasticity is linked to clinical features in HFS patients. High-resolution structural magnetic resonance imaging and diffusion tensor imaging data were acquired by 3.0 T MRI from 42 patients with HFS and 30 healthy subjects. The severity of the spasm was assessed according to Jankovic disability rating scale. Voxel-based morphometry (VBM) and tract-based spatial statistics (TBSS) analysis were performed to identify regional grey matter volume (GMV) changes and whole-brain microstructural integrity disruption measured by fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD). The VBM analysis showed that patients with HFS reduced GMV in the right inferior parietal lobule and increased GMV in the cerebellar lobule VIII, when compared with healthy subjects. Furthermore, within the HFS disease group, GMV decreased with the disease duration in the right inferior parietal lobule. TBSS did not identify group differences in diffusivity parameters. While no white matter integrity disruption was detected in the brain of patients with HFS, our study identified evident GMV changes in brain areas which were known to be involved in motor control. Our results suggest that HFS, a chronic neurovascular conflict disease, is related to structural reorganization in the brain. Copyright © 2015 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  1. Neonatal hypoglycemic brain injury is a cause of infantile spasms.

    Science.gov (United States)

    Yang, Guang; Zou, Li-Ping; Wang, Jing; Shi, Xiuyu; Tian, Shuping; Yang, Xiaofan; Ju, Jun; Yao, Hongxiang; Liu, Yujie

    2016-05-01

    Neonatal hypoglycemic brain injury is one of the causes of infantile spasms. In the present study, the clinical history and auxiliary examination results of 18 patients who developed infantile spasms several months after neonatal hypoglycemia were retrospectively analyzed. Among the 666 patients with infantile spasms admitted to two pediatric centers between January 2008 and October 2012, 18 patients developed infantile spasms after being diagnosed with neonatal hypoglycemia, defined as a whole blood glucose concentration of infantile spasms from between 2 and 10 months (mean, 4.9 months) following the diagnosis of neonatal hypoglycemia. All 18 patients had abnormal electroencephalographic findings with either classical or modified hypsarrhythmia. Upon examination using brain magnetic resonance imaging (MRI), 10 patients (55.6%) exhibited abnormalities. The MRI results principally showed a disproportional involvement of parietal and occipital cortices and sub-cortical white matter lesions. In conclusion, the results of this study indicate that neonatal hypoglycemic brain injury is associated with the subsequent development of infantile spasms.

  2. A genetic and biologic classification of infantile spasms

    Science.gov (United States)

    Paciorkowski, Alex R.; Thio, Liu Lin; Dobyns, William B.

    2011-01-01

    Infantile spasms are an age-dependent epilepsy that are highly associated with cognitive impairment, autism, and movement disorders. Previous classification systems have focused on a distinction between symptomatic and cryptogenic etiologies, and have not kept pace with the recent discoveries of mutations in genes in key pathways of central nervous system development in patients with infantile spasms. Children with certain genetic syndromes are much more likely to have infantile spasms, and we review the literature to propose a genetic classification of these disorders. Children with these genetic associations with infantile spasms also have phenotypes beyond epilepsy that may be explained by recent advances in the understanding of underlying biological mechanisms. We therefore also propose a biologic classification of the genes highly associated with infantile spasms, and articulate models for infantile spasms pathogenesis based on that data. The two best described pathways of pathogenesis are abnormalities in the gene regulatory network of GABAergic forebrain development, and abnormalities in molecules expressed at the synapse. We intend for these genetic and biologic classifications to be flexible, and hope that they will encourage much needed progress in syndrome recognition, clinical genetic testing, and ultimately the development of new therapies that target specific pathways of pathogenesis. PMID:22114996

  3. Neurodevelopmental and epilepsy outcome in children aged one to five years with infantile spasms--a North Indian cohort.

    Science.gov (United States)

    Sehgal, Rachna; Gulati, Sheffali; Sapra, Savita; Tripathi, Manjari; Kabra, Madhulika; Pandey, Ravinder Mohan

    2014-03-01

    The present study was planned as there is paucity of outcome data of children with infantile spasms, from India where profile of patients is different from the western world. Moreover, most previous studies have either not used strict inclusion criteria or standardized psychometric tests for developmental outcome. Ninety-five children, aged one-to-five years under follow up for more than six months in Pediatric Neurology Clinic of a tertiary care hospital with the diagnosis of infantile spasm were enrolled in this cross-sectional study if they had completed one or more years after the onset of spasms. The study period was January-December 2011. Neurodevelopment of each child was assessed using Development Profile 3 and Gross Motor Function Classification System. History regarding epilepsy frequency and control in the last one year was taken. Perinatal asphyxia was the commonest etiology in 43/95 children (45.2%). Favorable neurodevelopmental outcome was observed in 8/95 patients. Favorable epilepsy outcome in 58/95 (61.1%) patients was associated with treatment lag≤3 months between apparent onset of spasms and institution of therapy {OR 2 (1.1-3.8)} and response to first line antiepileptic drug {5 (2.6-10)}. The commonest etiology was potentially preventable perinatal cause. Early appropriate treatment may have a favorable epilepsy outcome. Copyright © 2014 Elsevier B.V. All rights reserved.

  4. Why West? Comparisons of clinical, genetic and molecular features of infants with and without spasms

    Science.gov (United States)

    Koh, Sookyong; Grinspan, Zachary M.; Shellhaas, Renée A.; Saneto, Russell P.; Wirrell, Elaine C.; Coryell, Jason; Chu, Catherine J.; Mytinger, John R.; Gaillard, William D.; Valencia, Ignacio; Knupp, Kelly G.; Loddenkemper, Tobias; Sullivan, Joseph E.; Poduri, Annapurna; Millichap, John J.; Keator, Cynthia; Wusthoff, Courtney; Ryan, Nicole; Dobyns, William B.; Hegde, Madhuri

    2018-01-01

    Infantile spasms are the defining seizures of West syndrome, a severe form of early life epilepsy with poorly-understood pathophysiology. We present a novel comparative analysis of infants with spasms versus other seizure-types and identify clinical, etiological, and molecular-genetic factors preferentially predisposing to spasms. We compared ages, clinical etiologies, and associated-genes between spasms and non-spasms groups in a multicenter cohort of 509 infants (spasms and non-spasms groups were compared. Spasms onset age was similar in infants initially presenting with spasms (6.1 months) versus developing spasms as a later seizure type (6.9 months) but lower in the non-spasms group (4.7 months, pspasms onset-age (r = -0.29, pspasm seizure age. Spasms were significantly preferentially associated with broad developmental and regulatory pathways, whereas motor functions and pathways including cellular response to stimuli, cell motility and ion transport were preferentially enriched in non-spasms. Neuronal cell-body organelles preferentially associated with spasms, while, axonal, dendritic, and synaptic regions preferentially associated with other seizures. Spasms are a clinically and biologically distinct infantile seizure type. Comparative clinical-epidemiological analyses identify the middle of the first year as the time of peak expression regardless of etiology. The inverse association with gestational age suggests the preterm brain must reach a certain post-conceptional, not just chronological, neurodevelopmental stage before spasms manifest. Clear differences exist between the biological pathways leading to spasms versus other seizure types and suggest that spasms result from dysregulation of multiple developmental pathways and involve different cellular components than other seizure types. This deeper level of understanding may guide investigations into pathways most critical to target in future precision medicine efforts. PMID:29518120

  5. CPP-115, a vigabatrin analogue, decreases spasms in the multiple-hit rat model of infantile spasms.

    Science.gov (United States)

    Briggs, Stephen W; Mowrey, Wenzhu; Hall, Charles B; Galanopoulou, Aristea S

    2014-01-01

    Infantile spasms (IS) have poor outcomes and limited treatment options, including vigabatrin, a γ-aminobutyric acid (GABA) aminotransferase inactivator. Vigabatrin has been associated with retinal toxicity. A high affinity vigabatrin analogue (CPP-115; Catalyst Pharmaceutical Partners, Inc., Coral Gables, FL, U.S.A.) has shown lower risk of retinal toxicity. Here, we test the efficacy of CPP-115 in reducing spasms and its tolerability in the multiple-hit rat model of IS, in which daily vigabatrin reduced spasms for only one day, but was not well tolerated. Male rats were treated with the protocol of the multiple-hit model of IS on postnatal day 3 (PN3). Using a randomized, blinded, vehicle-controlled, dose-response study design, CPP-115 (0.1, 1, or 5 mg/kg intraperitoneally [i.p.]) or vehicle was given daily (PN4-12) or as a single injection (PN7) after spasm onset. Intermittent video- or video-electroencephalography (EEG) monitoring was done. Secondary end points included the following: daily weights, survival, performance on open field activity, surface righting time, and negative geotaxis (PN3-20), horizontal bar (PN13-20), and Barnes maze (PN16-19). Statistics used a linear mixed model of raw or normalized log-transformed data, taking into account the repeated observations on each animal. The lower CPP-115 doses (0.1-1 mg/kg/day, PN4-12) reduced spasms between PN6 and 7 without increasing mortality. CPP-115 at 5 mg/kg/day (PN4-12) reduced spasms earlier (PN5), but was eventually lethal. A single CPP-115 injection (1 mg/kg, i.p.) decreased electroclinical spasms acutely but transiently. CPP-115 transiently improved the probability to >50% reduction of spasms, but did not accelerate spasm cessation. CPP-115 did not alter neurodevelopmental outcomes or visuospatial learning. We provide proof-of-concept evidence that CPP-115, a vigabatrin analogue, decreases spasms in the multiple-hit rat model of IS at considerably lower and better tolerated doses than vigabatrin

  6. Increased precipitation of spasms in an animal model of infantile spasms by prenatal stress exposure.

    Science.gov (United States)

    Shi, Xiu-Yu; Ju, Jun; Zou, Li-Ping; Wang, Juan; Shang, Ning-Xiu; Zhao, Jian-Bo; Wang, Jing; Zhang, Jun-Yan

    2016-05-01

    Infantile spasms (IS) represent a serious epileptic syndrome, called West syndrome (WS) that occurs in the early infantile age. Although several hypotheses and animal models have been proposed to explain the pathogenesis of IS, the pathophysiology of IS has not been elucidated. Recently, we proposed a hypothesis for IS under prenatal stress exposure (also called Zou's hypothesis) by correlating diverse etiologies and prenatal stresses with IS development. This research aims to determine the mechanism through which prenatal stress affects the offspring and establish the potential underlying mechanisms. Pregnant rats were subjected to forced swimming in cold water. Rat pups exposed to prenatal stress were administered with N-methyl-D-aspartate (NMDA). Exposure to prenatal stress sensitized the rats against development of NMDA-induced spasms. However, this phenomenon was altered by administering adrenocorticotropin. Prenatal stress exposure also altered the hormonal levels and neurotransmitter receptor expression of the developing rats as well as influenced the tissue structure of the brain. These findings suggest that maternal stress could alter the level of endogenous glucocorticoid, which is the basis of IS, and cerebral dysplasia, hypoxic-ischemic encephalopathy (HIE), inherited metabolic diseases, and other factors activated this disease in developmental brain. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Populations of Radial Glial Cells Respond Differently to Reelin and Neuregulin1 in a Ferret Model of Cortical Dysplasia

    Science.gov (United States)

    2010-10-28

    disruption of radial glia in rats. Developmental neuroscience 19(6): 521–528. 6. Ross ME (2002) Brain malformations, epilepsy, and infantile spasms ...linked with an increase of their BLBP expression. BLBP expressing radial glia are distinguished by being both less affected by MAM treatment and by...less affected by MAM treatment and by attempts at repair. We further investigated the effects induced by reelin and found that signaling was mediated

  8. The genetic landscape of infantile spasms.

    Science.gov (United States)

    Michaud, Jacques L; Lachance, Mathieu; Hamdan, Fadi F; Carmant, Lionel; Lortie, Anne; Diadori, Paola; Major, Philippe; Meijer, Inge A; Lemyre, Emmanuelle; Cossette, Patrick; Mefford, Heather C; Rouleau, Guy A; Rossignol, Elsa

    2014-09-15

    Infantile spasms (IS) is an early-onset epileptic encephalopathy of unknown etiology in ∼40% of patients. We hypothesized that unexplained IS cases represent a large collection of rare single-gene disorders. We investigated 44 children with unexplained IS using comparative genomic hybridisation arrays (aCGH) (n = 44) followed by targeted sequencing of 35 known epilepsy genes (n = 8) or whole-exome sequencing (WES) of familial trios (n = 18) to search for rare inherited or de novo mutations. aCGH analysis revealed de novo variants in 7% of patients (n = 3/44), including a distal 16p11.2 duplication, a 15q11.1q13.1 tetrasomy and a 2q21.3-q22.2 deletion. Furthermore, it identified a pathogenic maternally inherited Xp11.2 duplication. Targeted sequencing was informative for ARX (n = 1/14) and STXBP1 (n = 1/8). In contrast, sequencing of a panel of 35 known epileptic encephalopathy genes (n = 8) did not identify further mutations. Finally, WES (n = 18) was very informative, with an excess of de novo mutations identified in genes predicted to be involved in neurodevelopmental processes and/or known to be intolerant to functional variations. Several pathogenic mutations were identified, including de novo mutations in STXBP1, CASK and ALG13, as well as recessive mutations in PNPO and ADSL, together explaining 28% of cases (5/18). In addition, WES identified 1-3 de novo variants in 64% of remaining probands, pointing to several interesting candidate genes. Our results indicate that IS are genetically heterogeneous with a major contribution of de novo mutations and that WES is significantly superior to targeted re-sequencing in identifying detrimental genetic variants involved in IS. © The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  9. Long-term outcomes of infantile spasms

    Directory of Open Access Journals (Sweden)

    Seak Hee Oh

    2010-01-01

    Full Text Available Purpose : The aims of this study were to investigate the long-term outcomes in children with infantile spasms (IS and to identify the prognostic factors influencing their neurodevelopment. Methods : We retrospectively evaluated seventy two children over five years old who were treated for IS at Asan Medical Center, Seoul, Korea, between 1994 and 2007. Forty-three children were contacted by telephone or medical follow-up to assess their current neurodevelopmental status. Multiple logistic regression was used to calculate odds ratios (ORs and 95% confidence interval (95% CIs of risk factors for unfavorable outcomes. Results : The mean follow-up duration for these 43 children was 7.2¡?#?.5 ;years (range, 4.5 to 13.0 years. Of these, 13 (30.2% had cryptogenic and 30 (69.8% had symptomatic IS. Eleven (25.6% children were initially treated with adrenocorticotrophic hormone (ACTH therapy, with a mean treatment lag of 1.3¡?#?.9 ;months (range; 0.1 to 7.0 months. Eighteen (41.8% children clinically responded to initial treatment, as shown by EEG response. Overall, 22 (51.2% children had at least moderate neurodevelopmental disorders and 2 (4.8% died. In univariate analysis, etiology (symptomatic and poor electroclinical response to initial treatment were related to long-term unfavorable outcomes. In multivariate analysis, response to primary treatment was the sole significant independent risk factor with a high OR. Conclusion : Overall prognosis of children with IS was poor. Electroclinical non-responsiveness to initial treatment was related to unfavorable long-term outcomes, indicating that initial control of seizures may be important in reducing the likelihood of poor neurodevelopment.

  10. Association analysis of polymorphisms of the CRHR1 gene with infantile spasms

    OpenAIRE

    YANG, GUANG; ZOU, LI-PING; WANG, JING; SHI, XIU-YU; YANG, XIAO-FAN; WANG, BIN; LIU, YU-JIE; SUN, YAN-HONG; JIA, FEI-YONG

    2015-01-01

    While >200 types of etiologies have been shown to be involved in the pathogenesis of infantile spasms, the pathophysiology of infantile spasms remains largely elusive. Pre-natal stress and hypothalamic-pituitary-adrenal axis dysfunction were shown to be involved in the development of infantile spasms. To test the genetic association between the CRHR1 gene, which encodes the corticotrophin-releasing hormone (CRH) receptor, and infantile spasms, five single nucleotide polymorphisms (SNPs) in th...

  11. Preclinical Screening for Treatments for Infantile Spasms in the Multiple Hit Rat Model of Infantile Spasms: An Update.

    Science.gov (United States)

    Galanopoulou, Aristea S; Mowrey, Wenzhu B; Liu, Wei; Li, Qianyun; Shandra, Oleksii; Moshé, Solomon L

    2017-07-01

    Infantile spasms are the typical seizures of West syndrome, an infantile epileptic encephalopathy with poor outcomes. There is an increasing need to identify more effective and better tolerated treatments for infantile spasms. We have optimized the rat model of infantile spasms due to structural etiology, the multiple-hit rat model, for therapy discovery. Here, we test three compounds administered after spasms induction in the multiple hit model for efficacy and tolerability. Specifically, postnatal day 3 (PN3) male Sprague-Dawley rats were induced by right intracerebral injections of doxorubicin and lipopolysaccharide. On PN5 p-chlorophenylalanine was given intraperitoneally (i.p.). Daily monitoring of weights and developmental milestones was done and rats were intermittently video monitored. A blinded, randomized, vehicle-controlled study design was followed. The caspase 1 inhibitor VX-765 (50-200 mg/kg i.p.) and the GABA B receptor inhibitor CGP35348 (12.5-100 mg/kg i.p.) each was administered in different cohorts as single intraperitoneal injections on PN4, using a dose- and time-response design with intermittent monitoring till PN5. 17β-estradiol (40 ng/g/day subcutaneously) was given daily between PN3-10 and intermittent monitoring was done till PN12. None of the treatments demonstrated acute or delayed effects on spasms, yet all were well tolerated. We discuss the implications for therapy discovery and challenges of replication trials.

  12. Brainstem evoked potentials in infantile spasms; Comparison with MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Miyazaki, Masahito; Hashimoto, Toshiaki; Murakawa, Kazuyoshi; Tayama, Masanobu; Kuroda, Yasuhiro (Tokushima Univ. (Japan). School of Medicine)

    1992-08-01

    In ten patients with infantile spasms, brainstem evoked potentials and MRI examinations were performed to evaluate the brainstem involvement. The result of short latency somatosensory evoked potentials (SSEP) following the right median nerve stimulation revealed abnormal findings including the absence or low amplitudes of the waves below wave P3 and delayed central conduction time in 7 of the ten patients. The result of auditory brainstem responses (ABR) revealed abnormal findings including low amplitudes of wave V, prolonged interpeak latency of waves I-V and absence of the waves below wave IV in 5 of the ten patients. The result of the MRI examinations revealed various degrees of the brainstem atrophy in 6 of the ten patients, all of whom showed abnormal brainstem evoked potentials. The result of this study demonstrates that patients with infantile spasms are frequently associated with brainstem dysfunction and raises the possibility that brainstem atrophy might be a cause of infantile spasms. (author).

  13. Radial head button holing: a cause of irreducible anterior radial head dislocation

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Su-Mi; Chai, Jee Won; You, Ja Yeon; Park, Jina [Seoul National University Seoul Metropolitan Government Boramae Medical Center, Department of Radiology, Seoul (Korea, Republic of); Bae, Kee Jeong [Seoul National University Seoul Metropolitan Government Boramae Medical Center, Department of Orthopedic Surgery, Seoul (Korea, Republic of)

    2016-10-15

    ''Buttonholing'' of the radial head through the anterior joint capsule is a known cause of irreducible anterior radial head dislocation associated with Monteggia injuries in pediatric patients. To the best of our knowledge, no report has described an injury consisting of buttonholing of the radial head through the annular ligament and a simultaneous radial head fracture in an adolescent. In the present case, the radiographic findings were a radial head fracture with anterior dislocation and lack of the anterior fat pad sign. Magnetic resonance imaging (MRI) clearly demonstrated anterior dislocation of the fractured radial head through the torn annular ligament. The anterior joint capsule and proximal portion of the annular ligament were interposed between the radial head and capitellum, preventing closed reduction of the radial head. Familiarity with this condition and imaging findings will aid clinicians to make a proper diagnosis and fast decision to perform an open reduction. (orig.)

  14. Epidermoid cyst causing hemifacial spasm epidermoid cyst in cerebellopontine angle presenting with hemifacial spasm

    Directory of Open Access Journals (Sweden)

    Murat Alemdar

    2012-01-01

    Full Text Available Hemifacial Spasm (HS occurs idiopathically or secondary to the lesions compressing the root exit zone of the facial nerve symptomatically. Symptomatic HS is generally due to vascular compression. We report on a 23-year-old male with right sided HS for a month. Magnetic resonance imaging (MRI of the brain revealed a well-demarcated epidermoid cyst in the right cerebellopontine cistern. It was hypointense on T1-weighted imaging, hyperintense on T2-weighted imaging without contrast enhancement, hyperintense on DWI, and slightly hypointense on ADC relative to the brain. Although it caused shifting of the pons and medulla to the left side and compression of the right cerebellar peduncles and fourth ventricle, the sole symptom of the patient was HS. Clinicians are advised to request MRI/scan for brainstem lesions from the patients with HS. Epidermoid cysts in cerebellopontine cistern may present with HS as the sole symptom.

  15. Computerized tomography of brain in infantile spasms (West syndrome).

    Science.gov (United States)

    Mahdi, A H; Yohannan, M D; Patel, P J; Malabarey, T M; Kolawole, T M

    1990-01-01

    Computerized tomographic scanning of the brain was performed in 26 infants with Infantile spasms. Majority of the patients, 18 (69%) had some abnormality. Changes noted were cerebral atrophy in 12, calcifications in 5 and dysgenesis of the corpus callosum in 3 patients. One infant each had porencephaly, hydrocephalus and cavum septum pellucidum. Five patients had more than one abnormality simultaneously. Three infants had progressively worsening atrophy on ACTH therapy. Patients with infantile spasms, without any physical or neurological abnormality, are unlikely to have any abnormalities on brain CT scanning (p = less than 0.005).

  16. Laryngeal spasm after general anaesthesia due to Ascaris lumbricoides.

    Science.gov (United States)

    Finsnes, K D

    2013-08-01

    Postoperative upper airway obstruction during recovery from general anaesthesia may have several causes. This is a report of a young girl who developed laryngeal spasm as a result of an ectopic roundworm Ascaris lumbricoides. © 2013 The Acta Anaesthesiologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

  17. Pelvic floor spasm as a cause of voiding dysfunction.

    Science.gov (United States)

    Kuo, Tricia L C; Ng, L G; Chapple, Christopher R

    2015-07-01

    Pelvic floor disorders can present with lower urinary tract symptoms, bowel, sexual dysfunction, and/or pain. Symptoms of pelvic muscle spasm (nonrelaxing pelvic floor or hypertonicity) vary and can be difficult to recognize. This makes diagnosis and management of these disorders challenging. In this article, we review the current evidence on pelvic floor spasm and its association with voiding dysfunction. To distinguish between the different causes of voiding dysfunction, a video urodynamics study and/or electromyography is often required. Conservative measures include patient education, behavioral modifications, lifestyle changes, and pelvic floor rehabilitation/physical therapy. Disease-specific pelvic pain and pain from pelvic floor spasm needs to be differentiated and treated specifically. Trigger point massage and injections relieves pain in some patients. Botulinum toxin A, sacral neuromodulation, and acupuncture has been reported in the management of patients with refractory symptoms. Pelvic floor spasm and associated voiding problems are heterogeneous in their pathogenesis and are therefore often underrecognized and undertreated; it is therefore essential that a therapeutic strategy needs to be personalized to the individual patient's requirements. Therefore, careful evaluation and assessment of individuals using a multidisciplinary team approach including a trained physical therapist/nurse clinician is essential in the management of these patients.

  18. Decompression of the facial nerve in cases of hemifacial spasm

    Directory of Open Access Journals (Sweden)

    Karsten Kettel

    1954-12-01

    Full Text Available Among 11 patients a complete cure was obtained in one case, a fair result in 4 cases, while in 6 cases the effect of the operation has only been temporary and full recurrence has taken place. Even if decompression has thus resulted in a few recoveries and improvements, the results in the majority of cases have been disappointing. Everything points to hemifacial spasm being due to a disorder of the lower motor neuron. Intracranial lesions in the vicinity of the facial nerve are known to have resulted in irritation and spasm. It may be perfectly true that the majority of cases of hemifacial spasm are due to a lesion, the nature of which may vary, in the Fallopian canal near the stylomastoid foramen, not least the postparalytic following Bell's palsy. But the disappointing results of decompression seems to indicate that at the time of operation irreparable damage to the nerve has in the majority of cases been already done. Consequently I gave up decompression in cases of hemifacial spasm some years ago. Good results from injections of alcohol into the nerve have been reported13 but I prefer selective sections of the branches to the muscles involved as described by German and Greenwood8.

  19. Role of Muscle Relaxant (Tizanidine) In Painful Muscle Spasm ...

    African Journals Online (AJOL)

    More prolonged or regular cramps may be treated with drugs. Tizanidine which is an agonist at á ... Inclusion criteria included all the patients suffering from painful muscle spasm in back, neck, shoulder, knee or other anatomical sites with onset not more than two days prior to presentation. The patients suffering from ...

  20. Part One: Infantile Spasms--The New Consensus

    Science.gov (United States)

    Pellock, John

    2011-01-01

    Infantile spasms (IS, West syndrome) represent a difficult to treat and sometimes not immediately recognized form of epilepsy which is relatively rare. West Syndrome or IS is one of the most recognized types of epileptic encephalopathy, a form of epilepsy usually associated with developmental regression and delay, frequently difficult to treat and…

  1. Response to treatment in a prospective national infantile spasms cohort.

    Science.gov (United States)

    Knupp, Kelly G; Coryell, Jason; Nickels, Katherine C; Ryan, Nicole; Leister, Erin; Loddenkemper, Tobias; Grinspan, Zachary; Hartman, Adam L; Kossoff, Eric H; Gaillard, William D; Mytinger, John R; Joshi, Sucheta; Shellhaas, Renée A; Sullivan, Joseph; Dlugos, Dennis; Hamikawa, Lorie; Berg, Anne T; Millichap, John; Nordli, Douglas R; Wirrell, Elaine

    2016-03-01

    Infantile spasms are seizures associated with a severe epileptic encephalopathy presenting in the first 2 years of life, and optimal treatment continues to be debated. This study evaluates early and sustained response to initial treatments and addresses both clinical remission and electrographic resolution of hypsarrhythmia. Secondarily, it assesses whether response to treatment differs by etiology or developmental status. The National Infantile Spasms Consortium established a multicenter, prospective database enrolling infants with new diagnosis of infantile spasms. Children were considered responders if there was clinical remission and resolution of hypsarrhythmia that was sustained at 3 months after first treatment initiation. Standard treatments of adrenocorticotropic hormone (ACTH), oral corticosteroids, and vigabatrin were considered individually, and all other nonstandard therapies were analyzed collectively. Developmental status and etiology were assessed. We compared response rates by treatment group using chi-square tests and multivariate logistic regression models. Two hundred thirty infants were enrolled from 22 centers. Overall, 46% of children receiving standard therapy responded, compared to only 9% who responded to nonstandard therapy (p infantile spasms, including those with impaired development or known structural or genetic/metabolic etiology. ACTH appeared to be more effective than other standard therapies. © 2016 American Neurological Association.

  2. Factors associated with treatment lag in infantile spasms.

    Science.gov (United States)

    Napuri, Silvia; LE Gall, Edouard; Dulac, Olivier; Chaperon, Jacques; Riou, Francoise

    2010-12-01

    the aim of this study was to evaluate the conditions in which infantile spasms are diagnosed and their possible impact on the course of the disease. we carried out a retrospective study of the reasons for delayed treatment of infantile spasms (treatment lag) in western France over the period 1990-2003. A total of 156 infants, 87 male (55%) and 69 female (45%), with infantile spasms were identified, in 45 (29%) of whom the spasms were symptomatic. They were aged 1 week to 24 months (median 20wks, mean 22.4, SD 13.3) at first symptoms. To be included in the study, participants had to exhibit a combination of clusters of spasms, altered psychomotor development, and paroxysmal electroencephalographic (EEG) activity, as defined by the International League Against Epilepsy. We did not restrict onset to the first year of life as infantile spasms may begin after the age of 1 year. the mean time from appearance of first symptom to first visit to a medical practitioner was 4 weeks. In 14% of cases, the reason for the visit was non-neurological, the parents having noticed no neurological symptoms before the visit. The diagnosis was missed at first visit in 38% of the cases examined, with the incorrect diagnosis mostly commonly being gastro-oesophageal reflux or no abnormality. This increased to 74% after a second visit, in all cases based on an abnormal EEG. However, in 5% the time between first presentation and diagnosis was over 2 months and up to 10 visits were required. The time lag between first presentation and diagnosis was significantly longer for individuals presenting to general practitioners than to paediatricians (p=0.03). Response to treatment was poorer in those in whom diagnosis was delayed. various steps could be taken to reduce treatment lag such as training general practitioners, informing the parents of individuals at risk about the possibility of infantile spasms, and recommending that EEG is performed before brain imaging in children with unexplained

  3. YouTube videos as a teaching tool and patient resource for infantile spasms.

    Science.gov (United States)

    Fat, Mary Jane Lim; Doja, Asif; Barrowman, Nick; Sell, Erick

    2011-07-01

    The purpose of this study was to assess YouTube videos for their efficacy as a patient resource for infantile spasms. Videos were searched using the terms infantile spasm, spasm, epileptic spasm, and West syndrome. The top 25 videos under each term were selected according to set criteria. Technical quality, diagnosis of infantile spasms, and suitability as a teaching resource were assessed by 2 neurologists using the Medical Video Rating Scale. There were 5858 videos found. Of the 100 top videos, 46% did not meet selection criteria. Mean rating for technical quality was 4.0 of 5 for rater 1 and 3.9 of 5 for rater 2. Raters found 60% and 64% of videos to accurately portray infantile spasms, respectively, with significant agreement (Cohen κ coefficient = 0.75, P infantile spasms if guided search practices are followed.

  4. Impaired slow wave sleep downscaling in patients with infantile spasms.

    Science.gov (United States)

    Fattinger, Sara; Schmitt, Bernhard; Bölsterli Heinzle, Bigna K; Critelli, Hanne; Jenni, Oskar G; Huber, Reto

    2015-03-01

    West syndrome is a severe epileptic encephalopathy of infancy, characterized by infantile spasms, global retardation, and a severely abnormal electroencephalogram (EEG) pattern known as hypsarrhythmia, which is most prominent during slow waves sleep. The restorative function of slow wave sleep has been linked to downscaling, a neuronal process ensuring a balance of global synaptic strength, which is important for normal cortical functioning and development. A key electrophysiological marker for this downscaling is the reduction of the slope of slow waves across the night. We retrospectively compared the slope of slow waves between 14 untreated patients with infantile spasms and healthy age and gender matched controls. Patients were examined in one all-night sleep EEG before treatment, and in two follow-up nap recordings, under and after treatment with corticosteroids. In patients with infantile spasms the overnight reduction in the slope of slow waves was significantly diminished compared to controls (p = 0.009). Moreover, untreated patients revealed overall steeper slopes. During corticosteroid treatment the slope was reduced compared to controls (p = 0.001). After successful treatment the slope was similar between patients and controls. Our results provide evidence for reduced downscaling in patients with infantile spasms. Moreover, the marked reduction of the slope during corticosteroid treatment may reflect a loss of synaptic connections due to the effect of glucocorticoids. This altered sleep dependent regulation of synaptic strength in infantile spasms may contribute the underlying pathomechanism of the developmental regression. Furthermore the normalization of synaptic strength due to corticosteroids might provide a potential mechanistic explanation for this treatment strategy. Copyright © 2014 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  5. The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium.

    Science.gov (United States)

    Demarest, Scott T; Shellhaas, Renée A; Gaillard, William D; Keator, Cynthia; Nickels, Katherine C; Hussain, Shaun A; Loddenkemper, Tobias; Patel, Anup D; Saneto, Russell P; Wirrell, Elaine; Sánchez Fernández, Iván; Chu, Catherine J; Grinspan, Zachary; Wusthoff, Courtney J; Joshi, Sucheta; Mohamed, Ismail S; Stafstrom, Carl E; Stack, Cynthia V; Yozawitz, Elissa; Bluvstein, Judith S; Singh, Rani K; Knupp, Kelly G

    2017-12-01

    The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without hypsarrhythmia. Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of hypsarrhythmia were determined clinically at each site. Response to therapy was defined as resolution of clinical spasms (and hypsarrhythmia if present) without relapse 3 months after initiation. Eighty-two percent of patients had hypsarrhythmia, but this was not associated with gender, mean age, preexisting developmental delay or epilepsy, etiology, or response to first-line therapy. Infants with hypsarrhythmia were more likely to receive standard treatment (adrenocorticotropic hormone, prednisolone, or vigabatrin [odds ratio (OR) 2.6, 95% confidence interval (CI) 1.4-4.7] and preexisting epilepsy reduced the likelihood of standard treatment (OR 3.2, 95% CI 1.9-5.4). Hypsarrhythmia was not a determinant of response to treatment. A logistic regression model demonstrated that later age of onset (OR 1.09 per month, 95% CI 1.03-1.15) and absence of preexisting epilepsy (OR 1.7, 95% CI 1.06-2.81) had a small impact on the likelihood of responding to the first-line treatment. However, receiving standard first-line treatment increased the likelihood of responding dramatically: vigabatrin (OR 5.2 ,95% CI 2-13.7), prednisolone (OR 8, 95% CI 3.1-20.6), and adrenocorticotropic hormone (ACTH; OR 10.2, 95% CI 4.1-25.8) . First-line treatment with standard therapy was by far the most important variable in determining likelihood of response to treatment of infantile spasms with or without hypsarrhythmia. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

  6. Coronary spasm induced by dipyridamole; Spasme coronaire plurifocal declenche par l'injection de dipyridamole

    Energy Technology Data Exchange (ETDEWEB)

    Wartski, M.; Caussin, C.; Lancelin, B. [Centre Chirurgical Marie Lannelongue, 92 - Le Plessis Robinson (France)

    2001-03-01

    A 59 years old man was admitted at hospital for recurrent instable angina 1 month after coronary artery bypass surgery. Coronary artery disease started with a transmural antero-septo-apical myocardial infarction without thrombolysis and a percutaneous angioplasty with endo-prothesis on proximal left anterior descendant artery (LAD) is performed Because of recurrent rest angina and subacute stent thrombosis, a coronary artery bypass surgery (CABG) is performed with anastomosis of the left internal thoracic artery on LAD. The patient is admitted for recurrent rest angina one month after CABG. On ECG performed during chest pain, a ST-T segment elevation occurred on inferior leads. Coronary angiography showed no significant stenosis on endo-prothesis and no bypass graft dysfunction. Dipyridamole scintigraphy was realized. 2 minutes after the beginning of Dipyridamole infusion, a ST-T elevation occurred on inferior leads and two marked antero-septal and inferior defects were noticed on myocardial scintigraphy. Images at rest showed a clear improvement in the anterior wall and the inferior wall became normally perfused Patient was treated with anti-spastic drugs and a new coronarography with methyl-ergotamine test was performed inducing chest pain, ST-T elevation on inferior leads and tri-truncular coronary spasm. Patient's treatment was then modified with introduction of Nifedipine. The patient did not experienced new recurrent chest pain and remained totally asymptomatic few months later. (authors)

  7. Ketogenic diet efficacy in the treatment of intractable epileptic spasms.

    Science.gov (United States)

    Kayyali, Husam R; Gustafson, Megan; Myers, Tara; Thompson, Lindsey; Williams, Michelle; Abdelmoity, Ahmad

    2014-03-01

    To determine the efficacy of the ketogenic diet in controlling epileptic spasms after failing traditional antiepileptic medication therapy. This is a prospective, case-based study of all infants with epileptic spasms who were referred for treatment with the ketogenic diet at our hospital between 2009 and 2012. All subjects continued to have epileptic spasms with evidence of hypsarrhythmia or severe epileptic encephalopathy on electroencephalography despite appropriate medication treatments. The diet efficacy was assessed through clinic visits, phone communications, and electroencephalography. Quality of life improvement was charted based on the caregiver's perspective. Twenty infants (15 males) were included in the study. The mean age at seizure onset was 4.5 months. Age at ketogenic diet initiation was 0.3 to 2.9 years (mean 1.20, standard deviation 0.78). Fifteen patients had epileptic spasms of unknown etiology; three had perinatal hypoxic ischemic encephalopathy, one had lissencephaly, and one had STXBP1 mutation. Fifteen infants failed to respond to adrenocorticotropin hormone and/or vigabatrin before going on the ketogenic diet. Three months after starting the diet, >50% seizure reduction was achieved in 70% of patients (95% CI 48-86). These results were maintained at 6- and 12-month intervals. All eight of the patients followed for 24 months had >50% seizure reduction (95% CI 63-100). At least 90% seizure reduction was reported in 20% of patients at 3 months (95% CI 7-42), 22% (95% CI 8-46) at 6 months, and 35% (95% CI 17-59) at 12 months. The majority of patients (63%) achieved improvement of their spasms within 1 month after starting the diet. Sixty percent of patients had electroencephalographic improvement. All caregivers reported improvement of the quality of life at the 3-month visit (95% confidence interval 81-100). This ratio was 94% at 6 months (95% CI 72-99) and 82% at 12 months (95% CI 58-95). The ketogenic diet is a safe and potentially

  8. Correlation of twisting motion phase and infantile spasms in high risk infants.

    Science.gov (United States)

    Wang, Y Q; Yang, Z X; Zhu, P; Gu, G X

    2016-01-01

    The aim of this study was to investigate the correlation of twisting motion phase and infantile spasms in high risk infants. One hundred seventy-eight high-risk newborns experiencing follow-up in the rehabilitation phase were selected and full-body motion quality assessment was performed in the twisting motion phase. The occurrence of infants with infantile spasms after 12 months (corrected age) was statistically analyzed. No clear correlation was found between monotonous movement twisting motion phase and infantile spasms, and spasm synchronized movement had no definite prediction for infantile spasms. The incidence of infant spasm with movement form having spastic synchronized characteristics had significant difference compared with monotonous systemic movement (p spasm-synchronous movement of infantile spasms was 90.9%, the specificity was 96.8%, the positive predictive value was 80%, and the negative predictive value was 98.7%. Spasm synchronized movement had some predictive value for infantile spasms in twisting motion stage. The newborns with this kind of movement form should be checked by regularly ambulatory EEG.

  9. Infantile spasms with localized cerebral lesion on SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Miyazaki, Masahito; Hashimoto, Toshiaki; Yoshimoto, Tsutomu; Fujii, Emiko; Tayama, Masanobu; Kuroda, Yasuhiro (Tokushima Univ. (Japan). School of Medicine)

    1994-08-01

    Ten infants with infantile spasms underwent single photon emission computed tomography (SPECT) with [sup 99m]Tc HMPAO to assess local cerebral blood flow as well as EEG and magnetic resonance imaging (MRI) studies. SPECT revealed localized cerebral hypoperfusion in 7 infants (unifocal in 4 and multifocal in 3). This hypoperfusion always involved the temporal regions either unilaterally or bilaterally. EEGs obtained around the time of the SPECT study showed focal abnormalities in all 7 infants with hypoperfusion on SPECT, and there was complete correspondence of the abnormalities in 5 of the infants. On the other hand, MRI only revealed localized cerebral lesions in 3 of the 10 infants, all of whom had corresponding areas of hypoperfusion on SPECT. The present study thus indicated that localized cerebral abnormalities (especially of the temporal lobes) may often be associated with infantile spasms and that these patients frequently have localized cerebral hypoperfusion on SPECT even when MRI is normal. (author).

  10. Hemifacial spasm due to vertebrobasilar dolichoectasia: a case report

    Directory of Open Access Journals (Sweden)

    Mustafa AbdelHamid, MD

    2015-12-01

    Full Text Available Hemifacial spasm (HFS happens because of vascular compression of the facial nerve at the root exit zone. Vertebrobasilar dolichoectasia (VBD is a very rare cause of HFS. VBD is diagnosed by computed tomography angiography and magnetic resonance imaging. Here, we report a case of 65-year-old female patient with HFS due to VBD. We discuss the complications and the treatment options for the case.

  11. Hemimasticatory Spasm: Report of a Case and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Corina Christie

    2014-04-01

    Full Text Available Background: Hemimasticatory Spasm is a very rare disorder. Case report: A 62-year-old woman consulted with 30 years of unusual involuntary twitches in preauricular region and spasms that hamper jaw opening.  During the spasms she cannot open her mouth for several seconds. At physical examination we observed hypertrophy of the masseter and temporalis muscles, shared features with Hemimasticatory Spasm. She was treated with botulinum toxin type A with excellent response. Discussion: Hemimasticatory Spasm is a rare movement disorder, but given the excellent response to botulinum toxin type A treatment, it should be considered in the spectrum of facial spasms.

  12. Topiramate and Adrenal Cortico-tropic Hormone as Initial Treatment of Infantile Spasms

    Science.gov (United States)

    Peltzer, Bradley; Alonso, William D.; Porter, Brenda E.

    2009-01-01

    Historically, adrenal cortico-tropic hormone (ACTH) was used as first line treatment for infantile spasms, however there has been increasing use of topiramate as initial therapy. Here we report a retrospective study of ACTH and topiramate as initial treatment of infantile spasms. The neurology patient database at the Children's Hospital of Philadelphia was searched using the ICD-9 code for infantile spasms, and 50 patients were randomly chosen for chart review. We identified thirty-one patients receiving either ACTH or topiramate monotherapy (ACTH, n = 12, topiramate n = 19) as a first line treatment for infantile spasms. Twenty-six patients were symptomatic and five cryptogenic. Six patients treated with ACTH had resolution of clinical spasms and hypsarrhithmia within a month, but three relapsed. Four of the nineteen patients treated with topiramate eventually, though over a period of 0,1,8 or 69 months, had resolution of spasms and hypsarrhythmia. PMID:19225138

  13. Cardiac Autonomic Dysfunction in Patients With Infantile Spasm and the Effect of Adrenocorticotropic Hormone Treatment.

    Science.gov (United States)

    Gencpinar, Pinar; Kocabas, Abdullah; Duman, Özgür; Dündar, Nihal Olgaç; Haspolat, Senay; Kardelen, Fırat

    2016-02-01

    Infantile spasm is an age-dependent epileptic-encephalopathy syndrome. Cardiac autonomic function is frequently altered in epilepsy. In this study, we examined heart rate variability in patients with infantile spasm before and after treatment. Nineteen patients with infantile spasm and 13 healthy comparisons were enrolled in the study. Cardiac rhythm was recorded with a Holter device for 24 hours before adrenocorticotropic hormone (ACTH) (Synacthen depot) and B6 vitamin administration and 1 month after treatment. Heart rate variability analysis found lower heart rate variability parameters in patients with infantile spasm at the onset of the syndrome, prior to treatment with ACTH. The time domain parameters of heart rate variability values showed a statistically significant increase following ACTH treatment. Our data suggest that patients with infantile spasm exhibit lower heart rate variability parameters, and the treatment of spasms with ACTH and B6 together diminished the autonomic dysfunction in our cohort. © The Author(s) 2015.

  14. Adrenocorticotropic hormone versus prednisolone in the treatment of infantile spasms post vigabatrin failure.

    Science.gov (United States)

    Jones, Kevin; Snead, O Carter; Boyd, Jennifer; Go, Cristina

    2015-04-01

    The Child Neurology Society/American Academy of Neurology practice parameter has recommended adrenocorticotropic hormone or vigabatrin in the short-term treatment of infantile spasms. When vigabatrin is unavailable or ineffective and adrenocorticotropic hormone is not a treatment option because of the prohibitive cost, other forms of corticosteroids have been considered in the treatment of infantile spasms. This retrospective study reviewed the Hospital for Sick Children's experience with the short-term effectiveness of prednisolone versus adrenocorticotropic hormone in patients with infantile spasms who have failed vigabatrin. The results showed that while adrenocorticotropic hormone was more likely to lead to short-term spasm freedom, there was no difference in the likelihood of longer-term spasm resolution without relapse. These findings can guide clinicians in the treatment of infantile spasms post vigabatrin failure. © The Author(s) 2014.

  15. Topiramate and adrenocorticotropic hormone (ACTH) as initial treatment for infantile spasms.

    Science.gov (United States)

    Peltzer, Bradley; Alonso, William D; Porter, Brenda E

    2009-04-01

    Historically, adrenocorticotropic hormone was used as a first-line treatment for infantile spasms; however, there has been increasing use of topiramate as initial therapy. Here, we report a retrospective study of adrenocorticotropic hormone (ACTH) and topiramate as initial treatment for infantile spasms. The neurology patient database at the Children's Hospital of Philadelphia was searched using the International Classification of Diseases, Ninth Revision code for infantile spasms, and 50 patients were randomly chosen for chart review. We identified 31 patients receiving either adrenocorticotropic hormone or topiramate monotherapy (adrenocorticotropic hormone n = 12, topiramate n = 19) as a first-line treatment for infantile spasms. A total of 26 patients were symptomatic and 5 cryptogenic. Six patients treated with adrenocorticotropic hormone had resolution of clinical spasms and hypsarrhythmia within a month, but 3 relapsed. Of the 19 patients treated with topiramate, 4 patients eventually, though over a period of 0, 1, 8, or 69 months, had resolution of spasms and hypsarrhythmia.

  16. Treatment of infantile spasms: emerging insights from clinical and basic science perspectives.

    Science.gov (United States)

    Stafstrom, Carl E; Arnason, Barry G W; Baram, Tallie Z; Catania, Anna; Cortez, Miguel A; Glauser, Tracy A; Pranzatelli, Michael R; Riikonen, Raili; Rogawski, Michael A; Shinnar, Shlomo; Swann, John W

    2011-11-01

    Infantile spasms is an epileptic encephalopathy of early infancy with specific clinical and electroencephalographic (EEG) features, limited treatment options, and a poor prognosis. Efforts to develop improved treatment options have been hindered by the lack of experimental models in which to test prospective therapies. The neuropeptide adrenocorticotropic hormone (ACTH) is effective in many cases of infantile spasms, although its mechanism(s) of action is unknown. This review describes the emerging candidate mechanisms that can underlie the therapeutic effects of ACTH in infantile spasms. These mechanisms can ultimately help to improve understanding and treatment of the disease. An overview of current treatments of infantile spasms, novel conceptual and experimental approaches to infantile spasms treatment, and a perspective on remaining clinical challenges and current research questions are presented here. This summary derives from a meeting of specialists in infantile spasms clinical care and research held in New York City on June 14, 2010.

  17. Reverse 201Tl myocardial redistribution induced by coronary artery spasm

    International Nuclear Information System (INIS)

    Xiang Dingcheng; Yin Jilin; Gong Zhihua; Xie Zhenhong; Zhang Jinhe; Wen Yanfei; Yi Shaodong

    2010-01-01

    Objective: To investigate the mechanism of reverse redistribution (RR) on dipyridamole 201 Tl myocardial perfusion studies in the patients with coronary artery spasm. Methods: Twenty-six patients with coronary artery spasm and presented as RR on dipyridamole 201 Tl myocardial perfusion studies were enlisted as RR group, while other 16 patients with no coronary artery stenosis nor RR were enlisted as control group. Dipyridamole test was repeated during coronary angiography. Corrected thrombolysis in myocardial infarction (TIMI) frame count (CTFC) and TIMI myocardial perfusion grade (TMPG) were measured at RR related and non-RR related coronary arteries before and after dipyridamole infusion respectively. All of the data were analyzed by Student's t-test or χ 2 -test and correlation analysis. Results: Coronary artery angiography showed slower blood flow and lower myocardial perfusion in RR related vessels when compared with non-RR related vessels in RR group, but there was no significant difference among the main coronary arteries in control group. The perfusion defects of RR area at rest were positively related to slower blood velocity at corresponding coronary arteries (r = 0.79, t =10.18, P 0.05). Conclusion: RR is related to the decreased blood flow and myocardial perfusion induced by coronary artery spasm at rest, which may be improved by stress test such as intravenous dipyridamole infusion. (authors)

  18. Three-dimensional MRI of hemifacial spasm with surgical correlation

    Energy Technology Data Exchange (ETDEWEB)

    Girard, N. [Dept. of Radiology, Hopital Nord, Marseille (France); Poncet, M. [Dept. of Radiology, Hopital Nord, Marseille (France); Caces, F. [Dept. of Otorhinolaryngology, Hopital Nord, Marseille (France); Tallon, Y. [Dept. of Radiology, Hopital Nord, Marseille (France); Chays, A. [Dept. of Otorhinolaryngology, Hopital Nord, Marseille (France); Martin-Bouyer, P. [Dept. of Radiology, Hopital Nord, Marseille (France); Magnan, J. [Dept. of Otorhinolaryngology, Hopital Nord, Marseille (France); Raybaud, C. [Dept. of Radiology, Hopital Nord, Marseille (France)

    1997-01-01

    MRI was used to investigate 100 patients with hemifacial spasm, using 3D-FT T2-weighted (CISS) and contrast-enhanced 3D-FT T1-weighted (turbo-FLASH) sequences in all cases. MR angiography was performed in 54 patients, using 3D-MT FISP images. Decompression of the facial nerve through a retromastoid craniotomy was performed in all patients. Hemifacial spasm caused by tumours in the cerebellopontine angle was not included. Vascular contact with the facial nerve root-exit zone or at the internal auditory canal was present in 96 of 100 patients with hemifacial spasm. The vessel responsible was the vertebral artery (VA) in 18 cases, the posterior inferior cerebellar artery (PICA) in 23, the anterior inferior cerebellar artery (AICA) in 22, the VA and PICA in 24, VA and AICA in 3, PICA and AICA in 1, VA, PICA and AICA in 4, and a vein in 1 case. CISS images showed compressive vascular loops better than contrast-enhanced turbo-FLASH images alone. The sensitivity of MRI was high, since only one false-negative case was found among the 100 patients who underwent surgery. (orig.). With 6 figs., 1 tab.

  19. Efficacy of Treatments for Infantile Spasms: A Systematic Review.

    Science.gov (United States)

    Song, Ji Min; Hahn, Jongsung; Kim, Se Hee; Chang, Min Jung

    West syndrome (also known as infantile spasm because of its main seizure type) is a rare form of epilepsy that begins during early infancy. Recent guidelines and reviews on West syndrome recommend the use of adrenocorticotropic hormone steroids, or vigabatrin, as the first-line treatment. However, West syndrome remains to be one of the most challenging epilepsies to treat. Here, we systematically reviewed the current literature obtained during the previous decade. This article provides an overview of the current treatment of infantile spasms. PubMed and EMBASE were searched to retrieve studies on human published during 2005-2015 and to identify patients with clinical diagnosis of infantile spasms. Drug or diet treatments were used as interventions and comparators. We included 55 studies, of which 1 study was a meta-analysis, 9 were randomized controlled trials, 21 were prospective studies, and 24 were retrospective studies. Topiramate, levetiracetam, zonisamide, and sodium valproate with benzodiazepine (clonazepam or nitrazepam) were found to be potential drugs for treating West syndrome besides adrenocorticotropic hormone, steroids, and vigabatrin. Ketogenic diet and modified Atkins diet were also found to be effective. To date, data regarding the efficacy of treatments of West syndrome still remain limited. Some treatments, including topiramate and ketogenic diet, seem promising besides adrenocorticotropic hormone, steroids, and vigabatrin. Well-designed trials are warranted to validate the findings.

  20. Infantile spasms are associated with abnormal copy number variations.

    Science.gov (United States)

    Tiwari, Vijay N; Sundaram, Senthil K; Chugani, Harry T; Huq, A H M M

    2013-10-01

    The authors tested the hypothesis that de novo copy number variations (CNVs) implicated in known genomic disorders ("pathogenic CNVs") are significant predisposing factors of infantile spasms. The authors performed a genome-wide analysis of single-nucleotide polymorphism genotyping microarray data to identify the role of de novo/known pathogenic large CNVs in 13 trios of children affected by infantile spasms. A rare, large (4.8 Mb) de novo duplication was detected in the 15q11-13 region of 1 patient. In addition, 3 known pathogenic CNVs (present in the patient as well as 1 of the parents) were detected in total. In 1 patient, a known pathogenic deletion was detected in the region of 2q32.3. Similarly, in 1 other patient, 2 known pathogenic deletions in the regions of 16p11.2 and Xp22.13 (containing CDKL5) were detected. These findings suggest that some specific pathogenic CNVs predispose to infantile spasms and may be associated with different phenotypes.

  1. Infantile spasms: a proposal for a staged evaluation.

    Science.gov (United States)

    Trasmonte, J V; Barron, T F

    1998-11-01

    The purpose of this study was to determine whether a staged, algorithmic evaluation of infantile spasms could be developed to minimize patient discomfort, treatment delay, and overall costs. A retrospective chart review of patients diagnosed with infantile spasms at the authors' institution during a 10-year period was performed, with 29 patients identified; 28 charts were reviewed. By history and physical examination, 21 were classified as symptomatic and seven as cryptogenic. Of the 21 symptomatic patients, 13 had a known etiology at presentation; with further testing the specific etiology was determined in two more. Two in the cryptogenic group were reclassified on the basis of neuroimaging findings. Evaluations included neuroimaging (27, 15 abnormal), cerebrospinal fluid studies (nine, all normal), comprehensive metabolic studies (17, all normal), chromosomal analysis (11, two abnormal), and ophthalmologic evaluation (27, six abnormal). The average cost of the studies per patient was $5,076 at the authors' institution. Etiologic yield was increased by 20% with neuroimaging. The other investigations either confirmed a known etiology or were noncontributory. On the basis of these findings, the authors propose an algorithm for a more focused evaluation of infantile spasms. Using the algorithm, the authors suggest directly proceeding to therapy in patients with specific etiologies determined by history and examination. Further evaluation should start with neuroimaging. Subsequent evaluations should be on the basis of those results. The authors estimate a potential 60-90% reduction in total costs if this algorithm is applied.

  2. Safety and Efficacy of Vigabatrin for the Treatment of Infantile Spasms

    OpenAIRE

    Michele A. Faulkner; Justin A. Tolman

    2011-01-01

    In 2009, vigabatrin became the first FDA approved medication for the treatment of infantile spasms in the United States. There are few well-designed prospective studies comparing the drug to placebo or other modalities used in the treatment of infantile spasms. The available data have demonstrated that vigabatrin is efficacious in the treatment of infantile spasms regardless of underlying etiology, but that it is particularly beneficial in patients with a diagnosis of tuberous sclerosis. Adre...

  3. The early electroclinical manifestations of infantile spasms: A video EEG study.

    Science.gov (United States)

    Iype, Mary; Kunju, Puthuvathra Abdul Mohammed; Saradakutty, Geetha; Mohan, Devi; Khan, Shahanaz Ahamed Mohammed

    2016-01-01

    Infantile spasms are described as flexor extensor and mixed; but more features of their semiology and ictal electroencephalography (EEG) changes are sparse in the literature. The purpose of the study was to describe the clinical and ictal video-EEG characteristics of consecutive cases with infantile spasms and to try to find an association with the etiology. The clinical phenomenology and EEG characteristics on video-EEG were analyzed in 16 babies with infantile spasms. A total of 869 spasms were reviewed. Nine (56.3%) showed focal seizures at least once during the recording and 1 (6.3%) had multifocal myoclonus in addition to the spasms. The duration of the cluster and interval between spasms was totally variable in all patients. Lateralizing phenomena were present in at least some of the spasms in all patients. Unilateral manual automatism in the form of holding the pinna was noted in three patients following the spasm. The ictal EEG activity in the majority (75%) was the slow wave. Four (25%) showed fast generalized spindle-like ictal discharges. Spikes, spike and wave activity, or electrodecremental pattern alone during the ictus was seen in none. On bivariate analysis, no factor noted on the video EEG had association with the etiology. Infantile spasms could be associated with focal and other seizures, has unique, non-uniform and variable semiology from patient to patient. The ictal EEG manifestation in the majority (75%) of our patients was the slow wave transient with 25% showing generalized fast spindle-like activity.

  4. Radial gas turbine design

    Energy Technology Data Exchange (ETDEWEB)

    Krausche, S.; Ohlsson, Johan

    1998-04-01

    The objective of this work was to develop a program dealing with design point calculations of radial turbine machinery, including both compressor and turbine, with as few input data as possible. Some simple stress calculations and turbine metal blade temperatures were also included. This program was then implanted in a German thermodynamics program, Gasturb, a program calculating design and off-design performance of gas turbines. The calculations proceed with a lot of assumptions, necessary to finish the task, concerning pressure losses, velocity distribution, blockage, etc., and have been correlated with empirical data from VAT. Most of these values could have been input data, but to prevent the user of the program from drowning in input values, they are set as default values in the program code. The output data consist of geometry, Mach numbers, predicted component efficiency etc., and a number of graphical plots of geometry and velocity triangles. For the cases examined, the error in predicted efficiency level was within {+-} 1-2% points, and quite satisfactory errors in geometrical and thermodynamic conditions were obtained Examination paper. 18 refs, 36 figs

  5. Insufficient efficacy of vagus nerve stimulation for epileptic spasms and tonic spasms in children with refractory epilepsy.

    Science.gov (United States)

    Okanishi, Tohru; Fujimoto, Ayataka; Nishimura, Mitsuyo; Kanai, Sotaro; Motoi, Hirotaka; Homma, Yoichiro; Enoki, Hideo

    2018-02-01

    Vagus nerve stimulation (VNS) leads to palliation of refractory seizures. Epileptic spasms (ES) and tonic spasms (TS) appear in children with West syndrome and symptomatic generalized epilepsy. Both types of spasms are often characterized by truncal muscular contractions and ictal electroencephalography (EEG) findings comprising the contiguous phases: phase 1) 15-20 Hz, spindle-like fast activity (occur in 70%), 2) diffuse polyphasic δ/θ waves (100%), and 3) electrodecremental activity (70%). Here, we examined the effect of VNS on these spasms that are uniformly associated with the EEG and electromyogram changes. A consecutive series of 32 patients satisfied the inclusion criteria consisting of 1) medically refractory epilepsy, 2) VNS implantation between 2010 and 2015, 3) implantation of VNS before the age of 20 years, and 4) follow-up >2 years. From this cohort, 16 patients had spasms (ES/TS group), whereas the remaining 16 had partial seizures with or without secondary generalization (PS/SG group). We compared seizure outcomes between the two groups, and also determined the factors predicting these outcomes within the ES/TS group. The outcomes after 2 years of implantation, defined using the McHugh classification, were as follows: II (for 2 patients), III (5), and V (9) in the ES/TS group; and I (3 patients), II (6), III (2), IV (1), and V (4) in the PS/SG group. The ES/TS group had significantly worse outcomes than the PS/SG group (p = 0.024, Mann-Whitney U test). Multivariate ordinal logistic regression analysis revealed that shorter mean durations of ictal events were associated with better seizure outcomes following VNS implantation (p = 0.007). Only 13% of the patients in the ES/TS group had seizure reductions of greater than 50%. VNS was less effective for the treatment of patients with ES/TS than for those with PS/SG and those described in previous studies. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. A Pulse Rapamycin Therapy for Infantile Spasms and Associated Cognitive Decline

    Science.gov (United States)

    Raffo, Emmanuel; Coppola, Antonietta; Ono, Tomonori; Briggs, Stephen W.; Galanopoulou, Aristea S.

    2011-01-01

    Infantile spasms are seizures manifesting within a spectrum of epileptic encephalopathies of infancy that often lead to cognitive impairment. Their current therapies, including adrenocorticotropic hormone (ACTH), high dose steroids, or vigabatrin, are not always effective and may be associated with serious side effects. Overactivation of the TORC1 complex of the mTOR pathway is implicated in the pathogenesis of certain genetic and acquired disorders that are linked with infantile spasms, like tuberous sclerosis. Here, we tested the therapeutic potential of rapamycin, a TORC1 inhibitor, as a potential treatment for infantile spasms in the multiple-hit rat model of ACTH-refractory symptomatic infantile spasms, which is not linked to tuberous sclerosis. Rapamycin or vehicle were given after spasms appeared. Their effects on spasms, other seizures, performance in Barnes maze, and expression of the phosphorylated S6 ribosomal protein (pS6: a TORC1 target) in the cortex, using immunofluorescence, were compared. Rapamycin suppressed spasms dose-dependently and improved visuospatial learning, although it did not reduce the frequency of other emerging seizures. High-dose pulse rapamycin effected acute and sustained suppression of spasms and improved cognitive outcome, without significant side effects. Therapeutically effective rapamycin doses normalized the pS6 expression, which was increased in perilesional cortical regions of pups with spasms. These findings support that pathological overactivation of TORC1 may be implicated in the pathogenesis of infantile spasms, including those that are not linked to tuberous sclerosis. Furthermore, a high-dose, pulse rapamycin treatment is a promising, well tolerated and disease-modifying new therapy for infantile spasms, including those refractory to ACTH. PMID:21504792

  7. Short-Term Outcome of Intravenous Methylprednisolone Pulse Therapy in Patients With Infantile Spasms.

    Science.gov (United States)

    Yeh, Hye-Ryun; Kim, Min-Jee; Ko, Tae-Sung; Yum, Mi-Sun; You, Su-Jeong

    2017-06-01

    Many studies advocate hormonal treatments including high-dose oral prednisolone as an effective treatment for epileptic spasms. However, little is known about the effects of intravenous methylprednisolone pulse therapy on infantile spasms. We investigated the short-term response to intravenous methylprednisolone pulse therapy for the treatment of infantile spasms. Patients with newly diagnosed infantile spasms and hypsarrhythmia on electroencephalography (EEG) at two tertiary centers in Korea were included. Patients received intravenous infusions of 30 mg/kg/day methylprednisolone for three days with tapering doses of oral prednisolone for two to four weeks for the treatment of infantile spasms. Response to methylprednisolone pulse therapy was evaluated by seizure frequency and follow-up EEG within three weeks. Fourteen patients were sudied. The mean age at the onset of spasms was 7.0 months (range, 2.0 to 11.0 months). Etiological factors included structural abnormalities (N = 11), chromosomal anomaly (N = 1), and unknown (N = 2). Nine of 14 participants (64.3%) demonstrated complete freedom from spasm and resolution of hypsarrhythmia on EEG within 3 weeks; however, only five of nine responders (55.5%) remained free of spasms after the discontinuation of oral steroids. Adverse effects, including irritability or infection, were observed in four patients but were tolerable in all. Short-term methylprednisolone pulse therapy for the treatment of infantile spasms or hypsarrhythmia demonstrated rapid improvement in EEG and cessation of spasms without serious adverse effects. Further studies are needed to determine the long-term effects of spasm control. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. A pulse rapamycin therapy for infantile spasms and associated cognitive decline.

    Science.gov (United States)

    Raffo, Emmanuel; Coppola, Antonietta; Ono, Tomonori; Briggs, Stephen W; Galanopoulou, Aristea S

    2011-08-01

    Infantile spasms are seizures manifesting within a spectrum of epileptic encephalopathies of infancy that often lead to cognitive impairment. Their current therapies, including adrenocorticotropic hormone (ACTH), high dose steroids, or vigabatrin, are not always effective and may be associated with serious side effects. Overactivation of the TORC1 complex of the mTOR pathway is implicated in the pathogenesis of certain genetic and acquired disorders that are linked with infantile spasms, like tuberous sclerosis. Here, we tested the therapeutic potential of rapamycin, a TORC1 inhibitor, as a potential treatment for infantile spasms in the multiple-hit rat model of ACTH-refractory symptomatic infantile spasms, which is not linked to tuberous sclerosis. Rapamycin or vehicle was given after spasms appeared. Their effects on spasms, other seizures, performance in Barnes maze, and expression of the phosphorylated S6 ribosomal protein (pS6: a TORC1 target) in the cortex, using immunofluorescence, were compared. Rapamycin suppressed spasms dose-dependently and improved visuospatial learning, although it did not reduce the frequency of other emerging seizures. High-dose pulse rapamycin effected acute and sustained suppression of spasms and improved cognitive outcome, without significant side effects. Therapeutically effective rapamycin doses normalized the pS6 expression, which was increased in perilesional cortical regions of pups with spasms. These findings support that pathological overactivation of TORC1 may be implicated in the pathogenesis of infantile spasms, including those that are not linked to tuberous sclerosis. Furthermore, a high-dose, pulse rapamycin treatment is a promising, well tolerated and disease-modifying new therapy for infantile spasms, including those refractory to ACTH. Copyright © 2011 Elsevier Inc. All rights reserved.

  9. Efficacy of ketogenic diet for infantile spasms: A systematic review.

    Science.gov (United States)

    Prezioso, G; Carlone, G; Zaccara, G; Verrotti, A

    2018-01-01

    The aim of this systematic review was to collect and analyze all the RCTs and observational studies investigating the efficacy of ketogenic diet (KD) in infantile spasms (IS) patients after a 1- to 6-month follow-up period, in terms of decrease in seizure frequency of >50% or a seizure-free interval. Moreover, the potential effect of gender, IS etiology, age at onset of IS, and age at start of KD have been investigated. Finally, we evaluated the seizure-free rate at 12 and 24 months of follow-up. In June 2016, a computer search was performed on MedLine (PubMed), EMBASE, and the Cochrane Library. Only, English language studies conducted after 1980 and those reporting in detail the variation in seizure frequency have been selected. Thirteen observational studies (341 patients) were included in the final analysis. A median rate of 64.7% of patients experienced a spasm reduction >50% (IQR: 38.94%). The median spasm-free rate was 34.61% (IQR: 37.94%). IS of unknown etiology seemed to have an increased probability of achieving freedom from seizures (RR: 1.72, 95%CI: 1.18-2.53). Long-time follow-up data revealed a median seizure-free rate of 9.54% (IQR: 18.23%). Although the literature is still lacking in high-quality studies, which could provide a stronger level evidence, our findings suggest a potential benefit of KD for drug-resistant IS patients. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  10. Blink reflexes and lateral spreading in patients with synkinesia after Bell's palsy and in hemifacial spasm

    NARCIS (Netherlands)

    Eekhof, J. L.; Aramideh, M.; Speelman, J. D.; Devriese, P. P.; Ongerboer de Visser, B. W.

    2000-01-01

    We compared various electrodiagnostical tests in patients with hemifacial spasm and in patients who developed synkinesia after Bell's palsy. We examined the evoked blink reflexes in the orbicularis oculi (o. oculi) and orbicularis oris (o. oris) muscles in 23 patients with hemifacial spasm (HFS), in

  11. Association analysis of polymorphisms of the CRHR1 gene with infantile spasms.

    Science.gov (United States)

    Yang, Guang; Zou, Li-Ping; Wang, Jing; Shi, Xiu-Yu; Yang, Xiao-Fan; Wang, Bin; Liu, Yu-Jie; Sun, Yan-Hong; Jia, Fei-Yong

    2015-08-01

    While >200 types of etiologies have been shown to be involved in the pathogenesis of infantile spasms, the pathophysiology of infantile spasms remains largely elusive. Pre-natal stress and hypothalamic-pituitary-adrenal axis dysfunction were shown to be involved in the development of infantile spasms. To test the genetic association between the CRHR1 gene, which encodes the corticotrophin-releasing hormone (CRH) receptor, and infantile spasms, five single nucleotide polymorphisms (SNPs) in the CRHR1 gene were genotyped in a sample set of 128 cases with infantile spasms and 131 healthy controls. Correlation analysis was performed on the genotyped data. Under the assumption of the dominant model, the selected five SNPs, rs4458044, rs171440, rs17689966, rs28364026 and rs242948, showed no association with the risk of infantile spasms and the effectiveness of adrenocorticotropic hormone treatment. In addition, subsequent haplotype analysis suggested none of them was associated with infantile spasms. In conclusion, the experimental results of the present study suggested no association between the CRHR1 gene and infantile spasms in a Chinese population.

  12. Vigabatrin for the treatment of infantile spasms: final report of a randomized trial.

    Science.gov (United States)

    Elterman, Roy D; Shields, W Donald; Bittman, Richard M; Torri, Sarah A; Sagar, Stephen M; Collins, Stephen D

    2010-11-01

    A large randomized study was conducted in patients with newly diagnosed infantile spasms to compare 2 doses of vigabatrin in achieving spasm cessation. High (100-148 mg/kg/d) and low (18-36 mg/kg/d) oral doses of vigabatrin were evaluated in a randomized, single-blind study of 14 to 21 days with subsequent open-label treatment up to 3 years. Spasm cessation was defined as 7 consecutive days of spasm freedom beginning within the first 14 days, confirmed by video-electroencephalogram. A total of 221 subjects comprised the modified intent-to-treat cohort. More subjects in the high-dose group achieved spasm cessation compared with the low-dose vigabatrin group (15.9% [17/107] vs 7.0% [8/114]; P = .0375). During follow-up, 39 of 171 (23%) subjects relapsed; 28 of 39 (72%) regained spasm freedom. Adverse events were primarily mild to moderate in severity. Vigabatrin had a dose-dependent effect in spasm reduction. Spasm cessation occurred rapidly and was maintained in the majority of infants.

  13. Cognitive Outcome of Acth-Treated Infantile Spasms

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2004-03-01

    Full Text Available The long-term cognitive and seizure outcomes of 37 patients with cryptogenic infantile spasms (onset, age 3 to 9 months treated with high-dose synthetic adrenocorticotropic hormone (ACTH (1 mg IM every 48 hrs for 2 weeks, 8-10-week slow taper, followed by oral prednisone, 10 mg/d for 1 month, and slow taper for 5 months or until age 1 year were evaluated at Schneider Children’s Medical Center of Israel, Petah Tiqva, Israel.

  14. Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013.

    Science.gov (United States)

    Pavone, Piero; Striano, Pasquale; Falsaperla, Raffaele; Pavone, Lorenzo; Ruggieri, Martino

    2014-10-01

    The current spectrum of disorders associated to clinical spasms with onset in infancy is wider than previously thought; accordingly, its terminology has changed. Nowadays, the term Infantile spasms syndrome (ISs) defines an epileptic syndrome occurring in children younger than 1 year (rarely older than 2 years), with clinical (epileptic: i.e., associated to an epileptiform EEG) spasms usually occurring in clusters whose most characteristic EEG finding is hypsarrhythmia [the spasms are often associated with developmental arrest or regression]. The term West syndrome (WS) refers to a form (a subset) of ISs, characterised by the combination of clustered spasms and hypsarrhythmia on an EEG and delayed brain development or regression [currently, it is no longer required that delayed development occur before the onset of spasms]. Less usually, spasms may occur singly rather than in clusters [infantile spasms single-spasm variant (ISSV)], hypsarrhythmia can be (incidentally) recorded without any evidence of clinical spasms [hypsarrhythmia without infantile spasms (HWIS)] or typical clinical spasms may manifest in absence of hypsarrhythmia [infantile spasms without hypsarrhythmia (ISW)]. There is a growing evidence that ISs and related phenotypes may result, besides from acquired events, from disturbances in key genetic pathways of brain development: specifically, in the gene regulatory network of GABAergic forebrain dorsal-ventral development, and abnormalities in molecules expressed at the synapse. Children with these genetic associations also have phenotypes beyond epilepsy, including dysmorphic features, autism, movement disorders and systemic malformations. The prognosis depends on: (a) the cause, which gives origin to the attacks (the complex malformation forms being more severe); (b) the EEG pattern(s); (c) the appearance of seizures prior to the spasms; and (d) the rapid response to treatment. Currently, the first-line treatment includes the adrenocorticotropic

  15. Vigabatrin and mental retardation in tuberous sclerosis: infantile spasms vs focal seizures

    Science.gov (United States)

    Yum, Mi-Sun; Lee, Eun Hye; Ko, Tae-Sung

    2013-01-01

    Tuberous sclerosis complex is a genetic disorder resulting in epilepsy and mental retardation. Vigabatrin has shown efficacy in the treatment of infantile spasms caused by tuberous sclerosis complex, but its effects on focal seizures caused by tuberous sclerosis complex have not been determined. We compared the efficacy of vigabatrin in patients with tuberous sclerosis complex-induced focal seizures and infantile spasms and assessed the mental outcomes in both groups. We retrospectively evaluated 31 children with tuberous sclerosis complex and epilepsy, who were treated with vigabatrin in single tertiary center in Seoul, Korea. Vigabatrin treatment resulted in spasms cessation in 16 of 18 (88.9%) patients with infantile spasms, whereas 6 of 13 (46.2%) patients with focal seizures became seizure-free. Initial response to vigabatrin had no effect on intellectual disability. Vigabatrin was highly effective in eliminating infantile spasms caused by tuberous sclerosis complex, but was less effective in patients with focal seizures. PMID:22752486

  16. The current evaluation and treatment of infantile spasms among members of the Child Neurology Society.

    Science.gov (United States)

    Mytinger, John R; Joshi, Sucheta

    2012-10-01

    The optimal evaluation and treatment of children with infantile spasms is unknown. To aid in the development of a standardized approach to infantile spasms, members of the Child Neurology Society were surveyed to determine common practice. The survey had 222 responders with a responder rate of 18.5%. We found that the diagnostic evaluation and the use of first-line treatments varied among responders. For example, although adrenocorticotropic hormone continues to be the most commonly used first-line treatment for infantile spasms not due to tuberous sclerosis, some clinicians use corticosteroids, vigabatrin, and topiramate as first-line treatments for this group. Seventy percent of our responders reported seeing fewer than 10 new-onset cases of infantile spasms per year. Thus, future clinical trials will require multicenter collaboration. An important first step in such collaboration is to standardize the evaluation and treatment of infantile spasms within and between participating centers.

  17. Vigabatrin and mental retardation in tuberous sclerosis: infantile spasms versus focal seizures.

    Science.gov (United States)

    Yum, Mi-Sun; Lee, Eun Hye; Ko, Tae-Sung

    2013-03-01

    Tuberous sclerosis complex is a genetic disorder resulting in epilepsy and mental retardation. Vigabatrin has shown efficacy in the treatment of infantile spasms caused by tuberous sclerosis complex, but its effects on focal seizures caused by tuberous sclerosis complex have not been determined. We compared the efficacy of vigabatrin in patients with tuberous sclerosis complex-induced focal seizures and infantile spasms and assessed the mental outcomes in both groups. We retrospectively evaluated 31 children with tuberous sclerosis complex and epilepsy, who were treated with vigabatrin in a single tertiary center in Seoul, Korea. Vigabatrin treatment resulted in spasms cessation in 16 of 18 (88.9%) patients with infantile spasms, whereas 6 of 13 (46.2%) patients with focal seizures became seizure free. Initial response to vigabatrin had no effect on intellectual disability. Vigabatrin was highly effective in eliminating infantile spasms caused by tuberous sclerosis complex but was less effective in patients with focal seizures.

  18. Vigabatrin therapy implicates neocortical high frequency oscillations in an animal model of infantile spasms.

    Science.gov (United States)

    Frost, James D; Le, John T; Lee, Chong L; Ballester-Rosado, Carlos; Hrachovy, Richard A; Swann, John W

    2015-10-01

    Abnormal high frequency oscillations (HFOs) in EEG recordings are thought to be reflections of mechanisms responsible for focal seizure generation in the temporal lobe and neocortex. HFOs have also been recorded in patients and animal models of infantile spasms. If HFOs are important contributors to infantile spasms then anticonvulsant drugs that suppress these seizures should decrease the occurrence of HFOs. In experiments reported here, we used long-term video/EEG recordings with digital sampling rates capable of capturing HFOs. We tested the effectiveness of vigabatrin (VGB) in the TTX animal model of infantile spasms. VGB was found to be quite effective in suppressing spasms. In 3 of 5 animals, spasms ceased after a daily two week treatment. In the other 2 rats, spasm frequency dramatically decreased but gradually increased following treatment cessation. In all animals, hypsarrhythmia was abolished by the last treatment day. As VGB suppressed the frequency of spasms, there was a decrease in the intensity of the behavioral spasms and the duration of the ictal EEG event. Analysis showed that there was a burst of high frequency activity at ictal onset, followed by a later burst of HFOs. VGB was found to selectively suppress the late HFOs of ictal complexes. VGB also suppressed abnormal HFOs recorded during the interictal periods. Thus VGB was found to be effective in suppressing both the generation of spasms and hypsarrhythmia in the TTX model. Vigabatrin also appears to preferentially suppress the generation of abnormal HFOs, thus implicating neocortical HFOs in the infantile spasms disease state. Copyright © 2015 Elsevier Inc. All rights reserved.

  19. RARS2 mutations in a sibship with infantile spasms.

    Science.gov (United States)

    Ngoh, Adeline; Bras, Jose; Guerreiro, Rita; Meyer, Esther; McTague, Amy; Dawson, Eleanor; Mankad, Kshitij; Gunny, Roxana; Clayton, Peter; Mills, Philippa B; Thornton, Rachel; Lai, Ming; Forsyth, Robert; Kurian, Manju A

    2016-05-01

    Pontocerebellar hypoplasia is a group of heterogeneous neurodevelopmental disorders characterized by reduced volume of the brainstem and cerebellum. We report two male siblings who presented with early infantile clonic seizures, and then developed infantile spasms associated with prominent isolated cerebellar hypoplasia/atrophy on magnetic resonance imaging (MRI). Using whole exome sequencing techniques, both were found to be compound heterozygotes for one previously reported and one novel mutation in the gene encoding mitochondrial arginyl-tRNA synthetase 2 (RARS2). Mutations in this gene have been classically described in pontocerebellar hypoplasia type six (PCH6), a phenotype characterized by early (often intractable) seizures, profound developmental delay, and progressive pontocerebellar atrophy. The electroclinical spectrum of PCH6 is broad and includes a number of seizure types: myoclonic, generalized tonic-clonic, and focal clonic seizures. Our report expands the characterization of the PCH6 disease spectrum and presents infantile spasms as an associated electroclinical phenotype. © 2016 The Authors. Epilepsia published by Wiley Periodicals, Inc. on behalf of International League Against Epilepsy.

  20. Variations in the usage and composition of a radial cocktail during radial access coronary angiography procedures.

    LENUS (Irish Health Repository)

    Pate, G

    2011-10-01

    A survey was conducted of medication administered during radial artery cannulation for coronary angiography in 2009 in Ireland; responses were obtained for 15 of 20 centres, in 5 of which no radial access procedures were undertaken. All 10 (100%) centres which provided data used heparin and one or more anti-spasmodics; verapamil in 9 (90%), nitrate in 1 (10%), both in 2 (20%). There were significant variations in the doses used. Further work needs to be done to determine the optimum cocktail to prevent radial artery injury following coronary angiography.

  1. Knowledge, Attitude and Practice (KAP) Study of Pediatricians on Infantile Spasms.

    Science.gov (United States)

    Vaddi, Vamsi Krishna; Sahu, Jitendra Kumar; Dhawan, Sumeet R; Suthar, Renu; Sankhyan, Naveen

    2018-02-14

    To investigate the knowledge, attitude, and practice of Infantile Spasms among pediatricians. A survey was carried out among pediatricians serving in Punjab, Haryana, Chandigarh, Himachal Pradesh and Delhi. The survey was done by Survey Monkey Software through emails by using a structured questionnaire between July 2016 and December 2017. A total of 236 pediatricians responded to the survey. Most of the respondents (95.5%) correctly considered Infantile Spasms as a seizure type. The most preferred investigation was Electroencephalogram by 91.8% pediatricians; however, only 57.7% considered it to decide the treatment. Perinatal asphyxia was the most recognized etiology (60.7% pediatricians). For treatment of Infantile Spasms, 66.8% follow Nelson textbook of Pediatrics. Adrenocorticotropic hormone was the most preferred first choice drug by 40% pediatricians. Alternate anti-epileptic drug was considered by 60.9% pediatricians when there is no clinical response. Only 24% pediatricians considered treatment response as a complete cessation of spasms. Majority (90%) of pediatricians felt that there is a necessity for increased awareness and 62% pediatricians felt that available information was insufficient. A substantial number of pediatricians lack precise knowledge on evidence-based practice of Infantile Spasms. In developing countries, where pediatricians provide the initial management of Infantile Spasms, there is need to empower them and develop simplified national guidelines/consensus statement for management of Infantile Spasms.

  2. Addition of pyridoxine to prednisolone in the treatment of infantile spasms: A pilot, randomized controlled trial.

    Science.gov (United States)

    Kunnanayaka, Vedavathi; Jain, Puneet; Sharma, Suvasini; Seth, Anju; Aneja, Satinder

    2018-01-01

    West syndrome is a catastrophic epilepsy syndrome characterized by infantile spasms, hypsarrhythmia, and developmental arrest or regression. The aim of this study was to explore the role of pyridoxine in the management of infantile spasms. This was a pilot, randomized, open-label trial conducted at a tertiary level hospital from November 2012 to March 2014. Children aged 3 months to 3 years presenting with infantile spasms in clusters (at least 1 cluster/day) with hypsarrhythmia or its variants on electroencephalogram (EEG) were enrolled. The study participants were randomized to receive either oral prednisolone (4 mg/kg/day) alone or 30 mg/kg/day of pyridoxine with oral prednisolone. The primary outcome measure was the proportion of children who achieved spasm freedom for 48 h on day-14 after treatment initiation, as per parental reports, in both the groups. The adverse effects were also monitored. The study was registered with clinicaltrials.gov (ClinicalTrials.gov Identifier: NCT01828437). Sixty-two children were randomized into the two groups with comparable baseline characteristics. The proportion of children with spasm cessation on day-14 was similar in the two groups (39 vs. 37%, P = 0.98). The adverse effects were comparable in both the groups. The combination of pyridoxine with oral prednisolone was not found to be a beneficial therapy as compared to prednisolone alone in the treatment of infantile spasms in this pilot study. However, high dose pyridoxine may be safe in children with infantile spasms.

  3. A New Rat Model of Epileptic Spasms Based on Methylazoxymethanol-Induced Malformations of Cortical Development

    Directory of Open Access Journals (Sweden)

    Eun-Hee Kim

    2017-06-01

    Full Text Available Malformations of cortical development (MCDs can cause medically intractable epilepsies and cognitive disabilities in children. We developed a new model of MCD-associated epileptic spasms by treating rats prenatally with methylazoxymethanol acetate (MAM to induce cortical malformations and postnatally with N-methyl-d-aspartate (NMDA to induce spasms. To produce cortical malformations to infant rats, two dosages of MAM (15 mg/kg, intraperitoneally were injected to pregnant rats at gestational day 15. In prenatally MAM-exposed rats and the controls, spasms were triggered by single (6 mg/kg on postnatal day 12 (P12 or 10 mg/kg on P13 or 15 mg/kg on P15 or multiple doses (P12, P13, and P15 of NMDA. In prenatally MAM-exposed rats with single NMDA-provoked spasms at P15, we obtain the intracranial electroencephalography and examine the pretreatment response to adrenocorticotropic hormone (ACTH or vigabatrin. Rat pups prenatally exposed to MAM exhibited a significantly greater number of spasms in response to single and multiple postnatal NMDA doses than vehicle-exposed controls. Vigabatrin treatment prior to a single NMDA dose on P15 significantly suppressed spasms in MAM group rats (p < 0.05, while ACTH did not. The MAM group also showed significantly higher fast oscillation (25–100 Hz power during NMDA-induced spasms than controls (p = 0.047. This new model of MCD-based epileptic spasms with corresponding features of human spasms will be valuable for future research of the developmental epilepsy.

  4. The early electroclinical manifestations of infantile spasms: A video EEG study

    Science.gov (United States)

    Iype, Mary; Kunju, Puthuvathra Abdul Mohammed; Saradakutty, Geetha; Mohan, Devi; Khan, Shahanaz Ahamed Mohammed

    2016-01-01

    Purpose: Infantile spasms are described as flexor extensor and mixed; but more features of their semiology and ictal electroencephalography (EEG) changes are sparse in the literature. The purpose of the study was to describe the clinical and ictal video-EEG characteristics of consecutive cases with infantile spasms and to try to find an association with the etiology. Materials and Methods: The clinical phenomenology and EEG characteristics on video-EEG were analyzed in 16 babies with infantile spasms. Results: A total of 869 spasms were reviewed. Nine (56.3%) showed focal seizures at least once during the recording and 1 (6.3%) had multifocal myoclonus in addition to the spasms. The duration of the cluster and interval between spasms was totally variable in all patients. Lateralizing phenomena were present in at least some of the spasms in all patients. Unilateral manual automatism in the form of holding the pinna was noted in three patients following the spasm. The ictal EEG activity in the majority (75%) was the slow wave. Four (25%) showed fast generalized spindle-like ictal discharges. Spikes, spike and wave activity, or electrodecremental pattern alone during the ictus was seen in none. On bivariate analysis, no factor noted on the video EEG had association with the etiology. Conclusion: Infantile spasms could be associated with focal and other seizures, has unique, non-uniform and variable semiology from patient to patient. The ictal EEG manifestation in the majority (75%) of our patients was the slow wave transient with 25% showing generalized fast spindle-like activity. PMID:27011629

  5. The early electroclinical manifestations of infantile spasms: A video EEG study

    Directory of Open Access Journals (Sweden)

    Mary Iype

    2016-01-01

    Full Text Available Purpose: Infantile spasms are described as flexor extensor and mixed; but more features of their semiology and ictal electroencephalography (EEG changes are sparse in the literature. The purpose of the study was to describe the clinical and ictal video-EEG characteristics of consecutive cases with infantile spasms and to try to find an association with the etiology. Materials and Methods: The clinical phenomenology and EEG characteristics on video-EEG were analyzed in 16 babies with infantile spasms. Results: A total of 869 spasms were reviewed. Nine (56.3% showed focal seizures at least once during the recording and 1 (6.3% had multifocal myoclonus in addition to the spasms. The duration of the cluster and interval between spasms was totally variable in all patients. Lateralizing phenomena were present in at least some of the spasms in all patients. Unilateral manual automatism in the form of holding the pinna was noted in three patients following the spasm. The ictal EEG activity in the majority (75% was the slow wave. Four (25% showed fast generalized spindle-like ictal discharges. Spikes, spike and wave activity, or electrodecremental pattern alone during the ictus was seen in none. On bivariate analysis, no factor noted on the video EEG had association with the etiology. Conclusion: Infantile spasms could be associated with focal and other seizures, has unique, non-uniform and variable semiology from patient to patient. The ictal EEG manifestation in the majority (75% of our patients was the slow wave transient with 25% showing generalized fast spindle-like activity.

  6. Radial wedge flange clamp

    Science.gov (United States)

    Smith, Karl H.

    2002-01-01

    A radial wedge flange clamp comprising a pair of flanges each comprising a plurality of peripheral flat wedge facets having flat wedge surfaces and opposed and mating flat surfaces attached to or otherwise engaged with two elements to be joined and including a series of generally U-shaped wedge clamps each having flat wedge interior surfaces and engaging one pair of said peripheral flat wedge facets. Each of said generally U-shaped wedge clamps has in its opposing extremities apertures for the tangential insertion of bolts to apply uniform radial force to said wedge clamps when assembled about said wedge segments.

  7. CT study in infantile spasms. Side effects of ACTH therapy

    Energy Technology Data Exchange (ETDEWEB)

    Kumagai, K.; Ota, H.; Tamai, I.; Hotta, H.; Kubo, M. (Jikei Univ., Tokyo (Japan). School of Medicine)

    1981-05-01

    For reevaluation of ACTH therapy for infantile spasms, side effects of the therapy were investigated. 1) Susceptibility to diseases: Eight of 16 patients developed serious infectious diseases within one to two months after initiation of the therapy. 2) Electrolyte metabolism: Evaluation of 13 cases revealed the low serum-P concentration and the high serum-Na concentration. 3) Cerebral contraction: Cerebral contraction was noted in all of the ten cases which were examined by CT. Serial CT scanning in one case revealed a remarkable contraction of the brain 6 weeks after initiation of the therapy, nevertheless seizures disappeared and EEG recovered. CT findings recovered to normal 4 months after withdrawal of ACTH therapy.

  8. Shaken baby syndrome manifesting as infantile spasms seizure type

    Directory of Open Access Journals (Sweden)

    Der-Shiun Wang

    2014-01-01

    Full Text Available The diagnosis of child maltreatment leading to head injury is challenging. Here, we present the case of a 3-month-old female infant who presented with focal seizures that lasted for several minutes. After admission, she began to show intermittent clusters of head nods, irritable crying, arching, writhing, stiffening, and jerking of both arms. These results and electroencephalography findings were attributed as the diagnosis of infantile spasms (IS. Brain computed tomography and magnetic resonance imaging (MRI revealed the presence of chronic subdural hematoma mixed with acute ischemic injuries. Examination of the eye fundus confirmed the presence of retinal hemorrhage. Therefore, all evidence pointed to a diagnosis of shaken baby syndrome (SBS. Based on this case, we suggest that physicians should consider a diagnosis of SBS for children with new-onset IS and that should be evaluated, diagnosed, and treated as promptly as possible.

  9. Prognostic factors of infantile spasms from the etiological viewpoint.

    Science.gov (United States)

    Matsumoto, A; Watanabe, K; Negoro, T; Sugiura, M; Iwase, K; Hara, K; Miyazaki, S

    1981-01-01

    We investigated the prognostic factors for mental and physical development and seizure control by dividing the subjects into various etiologic groups in 200 patients with infantile spasms, all of whom (except 48 who died) were aged six years or older. The results were as follows: 1) Intermediate (4-12 mos) onset was found to be a favorable prognostic factor for seizure control in cryptogenic cases, although there was no relation between the age of onset and prognosis in other etiologic groups. 2) There was a significant correlation between the treatment lag and long-term prognosis for mental and physical development only in cryptogenic cases. A short treatment lag (0-2 mos) was associated with a good prognosis. All cryptogenic patients who had no relapse after ACTH therapy developed normally, although in symptomatic cases, there was no correlation between the relapse and the outcome.

  10. Neuromyelitis optica: association with paroxysmal painful tonic spasms.

    Science.gov (United States)

    Carnero Contentti, E; Leguizamón, F; Hryb, J P; Celso, J; Pace, J L Di; Ferrari, J; Knorre, E; Perassolo, M B

    2016-10-01

    Paroxysmal painful tonic spasms (PPTS) were initially described in multiple sclerosis (MS) but they are more frequent in neuromyelitis optica (NMO). The objective is to report their presence in a series of cases of NMO and NMO spectrum disorders (NMOSD), as well as to determine their frequency and clinical features. We conducted a retrospective assessment of medical histories of NMO/NMOSD patients treated in 2 hospitals in Buenos Aires (Hospital Durand and Hospital Álvarez) between 2009 and 2013. Out of 15 patients with NMOSD (7 with definite NMO and 8 with limited NMO), 4 presented PPTS (26.66%). PPTS frequency in the definite NMO group was 57.14% (4/7). Of the 9 patients with longitudinally extensive transverse myelitis (LETM), 44.44% (9/15) presented PPTS. Mean age was 35 years (range, 22-38 years) and all patients were women. Mean time between NMO diagnosis and PPTS onset was 7 months (range, 1-29 months) and mean time from last relapse of LETM was 30 days (range 23-40 days). LETM (75% cervicothoracic and 25% thoracic) was observed by magnetic resonance imaging (MRI) in all patients. Control over spasms and pain was achieved in all patients with carbamazepine (associated with gabapentin in one case). No favourable responses to pregabalin, gabapentin, or phenytoin were reported. PPTS are frequent in NMO. Mean time of PPTS onset is approximately one month after an LETM relapse, with extensive cervicothoracic lesions appearing on the MRI scan. They show an excellent response to carbamazepine but little or no response to pregabalin and gabapentin. Prospective studies with larger numbers of patients are necessary in order to confirm these results. Copyright © 2014 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  11. Epileptic spasms: Nosological characteristics and approaches to therapy

    Directory of Open Access Journals (Sweden)

    M. B. Mironov

    2013-01-01

    Full Text Available Objective: to study the incidence, anamnestic, clinical, electroencephalographic, and neuroimaging features of epileptic syndromes associated with epileptic spasms (ES and the efficiency of antiepileptic therapy in patients with these conditions. Patients and methods. The study covered 1261 patients with epileptic seizures in the history with their onset from the first day of life to 18 years.Results. A history of ES was recorded in 112 patients, which accounted for 8.9% of cases among all the forms of epilepsy with its onset less than 18 years of age. ES was detected in 47.1% of the patients with seizure onset in the first year of life. There was a slight male preponderance: 59 (52.7% versus 53 (47.3%. Epilepsy with ES onset occurred in the first 6 months of life in 57.9% of cases. The West syndrome was found in 58.9% of the patients with ES-associated epilepsy, which amounted to 5.2% of all forms of epilepsy developing at the age of less than 18 years; Ohtahara syndrome in 32.1% (2.9% of all forms of epilepsy, early myoclonic encephalopathy in 0.9% (0.08% and symptomatic focal epilepsy with periodic spasms in 8.1% (0.7% of all forms of epilepsy. Antiepileptic therapy led to complete remission in 45.5% of ES-associated epilepsy cases. There was a 50% or more reduction in the rate of seizures in 35.7% of the patients treated with antiepileptic drugs. No effect was seen in 18.8% of cases.

  12. Hypsarrhythmia paroxysm index: A tool for early prediction of infantile spasms.

    Science.gov (United States)

    Altunel, Attila; Sever, Ali; Altunel, Emine Özlem

    2015-03-01

    Recurrence of infantile spasms (ISs) is common subsequent to treatment with adrenocorticotropic hormone (ACTH) for West syndrome, and prolonged hypsarrhythmia results in psychomotor deterioration. The evolution to hypsarrhythmia involves conversion of prehypsarrhythmic EEG findings to sporadic hypsarrhythmia paroxysms (HPs), and when paroxysms reach a certain frequency, ISs begin to occur. This retrospective chart study aimed to determine the HP threshold frequency after which ISs begin. Recorded either prior (Group A) or subsequent (Group B) to IS relapse, 248 EEGs were examined in 42 patients. The number of HPs in non-rapid eye movement (NREM) sleep divided by NREM duration constituted the countable hypsarrhythmia paroxysms index (cHPI). After reaching a rate of approximately 10/min, the cHPI lost its feasibility due to the merging of HPs. The durational HPI (dHPI) was also calculated (total duration of HPs during NREM/NREM sleep time×100). ACTH treatment was administered if cHPI was ≥2/min, with the aim of preventing relapse. The mean cHPI value without a concomitant spasm relapse (in Group A) was 1.20/min. Following relapse, this value rose to 4.10/min. EEGs performed subsequent to relapse (in Group B) were classified into three subgroups (B1, B2, and B3) according to the duration of the time interval between IS relapse and the succeeding EEG recording. One-way analysis of variance (ANOVA) indicated that cHPI values differed significantly between the Group B subgroups. In subgroups B2 and B3, a higher number of EEGs were evaluated via dHPI. Linear regression analysis established that the interval between recurrence and the succeeding EEG recording significantly predicted cHPI values and accounted for 54.2% of the explained variability in cHPI values. Therefore, use of the cHPI for early recognition and intervention may aid in preventing the onset and recurrence of ISs and further deterioration of psychomotor development. Copyright © 2015 Elsevier B.V. All

  13. Radially truncated galactic discs

    NARCIS (Netherlands)

    Grijs, R. de; Kregel, M.; Wesson, K H

    2000-01-01

    Abstract: We present the first results of a systematic analysis of radially truncatedexponential discs for four galaxies of a sample of disc-dominated edge-onspiral galaxies. Edge-on galaxies are very useful for the study of truncatedgalactic discs, since we can follow their light distributions out

  14. Parry-Romberg syndrome with hemimasticatory spasm in pregnancy; A dystonia mimic

    Directory of Open Access Journals (Sweden)

    Akhila Kumar Panda

    2014-01-01

    Full Text Available Parry-Romberg syndrome (PRS with hemimasticatory spasm (HMS is quite an uncommon overlapping phenomenon which very often mimics jaw closing dystonia. A previously healthy 35-year-old female, during her 5 th month of pregnancy started developing intermittent unilateral painful spasms of jaw while conversation, clinching of teeth, or eating, which led to frequent tongue bites. The spasms were worsened during pregnancy. She used to do certain manoeuvre like sensory tricks in form of touching involved side of the face to relieve the symptoms. Apart from this, she developed progressive hemifacial and hemitongue atrophy. Other medical and neurological examinations were normal. Laboratory investigations as well as neuroimaging were noncontributory. The spasm responded to carbamazepine but hemifacial atrophy persists. To our best knowledge, onset and worsening of this syndrome in pregnancy has not been described earlier which might be correlated either with some hormonal imbalance or some unknown mechanisms.

  15. Optimizing Care With a Standardized Management Protocol for Patients With Infantile Spasms.

    Science.gov (United States)

    Fedak, Erin M; Patel, Anup D; Heyer, Geoffrey L; Wood, Eric G; Mytinger, John R

    2015-09-01

    The primary aim of this quality improvement initiative was to increase the number of patients receiving first-line therapy (adrenocorticotropic hormone, corticosteroids, vigabatrin) as the initial treatment for infantile spasms. We implemented a standardized management protocol for infantile spasms based on the best available data and expert consensus. To assess the impact of this intervention, we compared the 3-month remission rates between prestandardization (January 2009 to August 2012) and poststandardization (September 2012 to May 2014) cohorts. We found that the percentage of patients receiving first-line therapy as the initial treatment was 57% (31/54) in the prestandardization cohort and 100% (35/35) in the poststandardization cohort (P infantile spasms remission was higher poststandardization compared to prestandardization (78.8% vs 30.6%, P infantile spasms remission 3 months after diagnosis. © The Author(s) 2014.

  16. A potential effect of ganaxolone in an animal model of infantile spasms.

    Science.gov (United States)

    Yum, Mi-Sun; Lee, Minyoung; Ko, Tae-Sung; Velíšek, Libor

    2014-11-01

    Infantile spasms (IS), a devastating epileptic encephalopathy of infancy, involve various etiologies associated with an unknown underlying common pathophysiology. The efficacy of adrenocorticotropic hormone (ACTH) as an IS therapy suggests a role for steroid hormones in treating IS. This study used an animal model of IS to test the efficacy of ganaxolone, a synthetic neurosteroid, promoting tonic GABAA inhibition. The model of cryptogenic IS used in this study involved prenatal priming of rats with betamethasone (0.4 mg/kg i.p. at 08:30 and 18:30) on gestational day 15. To test the acute effects of ganaxolone, rats were pretreated with ganaxolone (10, 25, or 50mg/kg i.p.) or vehicle (β-cyclodextrin, i.p.) 30 min prior to N-methyl-d-aspartate (NMDA)-induced spasms at postnatal day 15 (P15). To mimic human conditions, another group of rats was randomly divided and repeatedly treated with ganaxolone (20mg/kg at 9:00 and 18:00 from P13-15) or vehicle after experiencing NMDA-triggered spasms at P12. Additional spasms were triggered on P13 and P15. We determined latency to the onset of spasms and the total number of spasms per 90-min observation period after the trigger at P15. On P19 and P21, behavioral tests were performed in rats with randomized repeated treatments. The 25mg/kg and 50mg/kg doses of ganaxolone significantly delayed the onset of spasms compared with the controls, and significantly decreased the number of spasms or suppressed their incidence. Ganaxolone had significant side effects in terms of sedation: all animals with the 50mg/kg dose were sleeping during the test. Randomized ganaxolone treatment for 3 days also significantly delayed the onset and decreased the number of spasms triggered by NMDA on P15, and decreased exploratory behavior after multiple NMDA triggered spasms. Ganaxolone significantly suppresses the development of spasms in the rat model of cryptogenic IS. This synthetic neurosteroid active in an animal model of IS might contribute to

  17. Radial Halbach Magnetic Bearings

    Science.gov (United States)

    Eichenberg, Dennis J.; Gallo, Christopher A.; Thompson, William K.

    2009-01-01

    Radial Halbach magnetic bearings have been investigated as part of an effort to develop increasingly reliable noncontact bearings for future high-speed rotary machines that may be used in such applications as aircraft, industrial, and land-vehicle power systems and in some medical and scientific instrumentation systems. Radial Halbach magnetic bearings are based on the same principle as that of axial Halbach magnetic bearings, differing in geometry as the names of these two types of bearings suggest. Both radial and axial Halbach magnetic bearings are passive in the sense that unlike most other magnetic bearings that have been developed in recent years, they effect stable magnetic levitation without need for complex active control. Axial Halbach magnetic bearings were described in Axial Halbach Magnetic Bearings (LEW-18066-1), NASA Tech Briefs, Vol. 32, No. 7 (July 2008), page 85. In the remainder of this article, the description of the principle of operation from the cited prior article is recapitulated and updated to incorporate the present radial geometry. In simplest terms, the basic principle of levitation in an axial or radial Halbach magnetic bearing is that of the repulsive electromagnetic force between (1) a moving permanent magnet and (2) an electric current induced in a stationary electrical conductor by the motion of the magnetic field. An axial or radial Halbach bearing includes multiple permanent magnets arranged in a Halbach array ("Halbach array" is defined below) in a rotor and multiple conductors in the form of wire coils in a stator, all arranged so the rotary motion produces an axial or radial repulsion that is sufficient to levitate the rotor. A basic Halbach array (see Figure 1) consists of a row of permanent magnets, each oriented so that its magnetic field is at a right angle to that of the adjacent magnet, and the right-angle turns are sequenced so as to maximize the magnitude of the magnetic flux density on one side of the row while

  18. Frontal infraslow activity marks the motor spasms of anti-LGI1 encephalitis.

    Science.gov (United States)

    Wennberg, Richard; Steriade, Claude; Chen, Robert; Andrade, Danielle

    2018-01-01

    The clinical and electrographic features of seizures in anti-LGI1 encephalitis are distinct from those seen in other autoimmune encephalitides or non-encephalitic epilepsies. One electroclinical phenomenon specific to the condition consists of lateralized motor spasms, known as faciobrachial dystonic seizures (FBDS). An electrodecremental pattern overriding a "DC shift" has been described as the EEG correlate of these spasms. We sought to further characterize this pre-spasm infraslow activity (ISA). Continuous video-EEG recordings were acquired in four patients with anti-LGI1 encephalitis: each had frequent motor spasms/FBDS as well as frequent subclinical temporal lobe seizures (an independent indicator of anti-LGI1 encephalitis). In artifact-free recordings obtained using clinical amplifiers equipped with a low frequency analog filter of 0.07 Hz, ISA reliably preceded clinical onset of the motor spasms by ∼1.2 s and preceded the electrodecremental pattern by ∼700 ms. Pre-spasm ISA was invariably recorded contralateral to FBDS, with a voltage topographic maximum over the mid frontal region. The pre-movement ISA differed from the Bereitschaftspotential in timing and topography and was an order of magnitude higher in amplitude. Sporadic FBDS that occurred in association with temporal lobe seizures were preceded by identical ISA. The motor spasms of anti-LGI1 encephalitis are preceded by frontal ISA. A paucity of data at the microscale level precludes mechanistic explanations at the macroscale level, or even determination of the relative contributions of neurons and glia in the generation of the ISA. Although fundamental cellular mechanisms await elucidation, the pre-spasm ISA represents a singular and readily identifiable EEG response to this autoimmune brain disorder. Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

  19. Safety and Efficacy of Vigabatrin for the Treatment of Infantile Spasms

    Science.gov (United States)

    Faulkner, Michele A.; Tolman, Justin A.

    2011-01-01

    In 2009, vigabatrin became the first FDA approved medication for the treatment of infantile spasms in the United States. There are few well-designed prospective studies comparing the drug to placebo or other modalities used in the treatment of infantile spasms. The available data have demonstrated that vigabatrin is efficacious in the treatment of infantile spasms regardless of underlying etiology, but that it is particularly beneficial in patients with a diagnosis of tuberous sclerosis. Adrenocorticotropic hormone (ACTH), the only other medication with robust efficacy data, has been used as first line therapy for infantile spasms associated with other etiologies, and in general controls spasms sooner than vigabatrin, though relapse is common with both therapies. Vigabatrin is generally well tolerated. However, use has been associated with permanent loss of peripheral vision in some patients. In children with tuberous sclerosis, vigabatrin should be considered as initial therapy for infantile spasms. It is a viable alternative for patients with suboptimal response, contraindications or intolerance to ACTH. PMID:23861649

  20. Detection of global DNA hypomethylation of peripheral blood lymphocytes in patients with infantile spasms.

    Science.gov (United States)

    Yang, Guang; Wang, Jing; Shi, Xiu-Yu; Yang, Xiao-Fan; Ju, Jun; Liu, Yu-Jie; Li, Zhi-Fang; Li, Yu-Fen; Zou, Li-Ping

    2015-01-01

    The pathogenesis of infantile spasms remains unclear. DNA methylation may play a pivotal role in the development of some types of neurological diseases, such as epilepsy. In this study, we aimed to investigate the relationship between global DNA methylation of peripheral blood leukocytes and cryptogenic infantile spasms. DNA from peripheral blood leukocytes was extracted from 20 patients with cryptogenic infantile spasms and 20 gender and age matched healthy controls. Global DNA methylation percentage of peripheral blood leukocytes was measured using a global DNA methylation quantification kit. Global DNA methylation levels of peripheral blood lymphocytes in patients with cryptogenic infantile spasms (23.4 ± 20.0%) were significantly lower than those in healthy controls (46.8 ± 8.4%). Furthermore, we did not find any association between the levels of DNA methylation and effectiveness of Adrenocorticotropic hormone treatment. Our study demonstrates that global DNA hypomethylation of peripheral blood lymphocytes is correlated with infantile spasms. This finding provides information for better understanding of the pathogenesis of infantile spasms. Copyright © 2014 Elsevier B.V. All rights reserved.

  1. Common ABCB1 polymorphisms associated with susceptibility to infantile spasms in the Chinese Han population.

    Science.gov (United States)

    Dong, L; Mao, M; Luo, R; Tong, Y; Yu, D

    2011-10-19

    Infantile spasms are a severe epileptic encephalopathy with a variety of etiologies that occur in infancy and early childhood. Subjects with infantile spasms are at a higher risk for evolving into intractable epileptic spasms, tending to be refractory to conventional antiepileptic drugs. Genetic polymorphisms of the P-glycoprotein-encoding gene ABCB1 are suspected to be associated with pharmacoresistance phenotypes in epilepsy patients. Conflicting findings have been reported in different populations; few studies have explored whether this apparent association is affected by other host factors, such as specific epilepsy syndrome. We performed a case-control study to determine whether the risk of infantile spasms is influenced by common ABCB1 polymorphisms in a Han Chinese children's population consisting of 91 patients and 368 healthy individuals. DNA was isolated from whole blood, and three genetic polymorphisms (C1236T, G2677T/A, and C3435T) were assayed by PCR-RFLP. There were significant differences in the distributions of 3435TT [P = 0.001; odds ratio = 2.47; 95% confidence interval (CI) = 1.44-4.27] and 3435CT [P infantile spasm cases and controls. No significant differences were observed in allelic and haplotypic frequencies of ABCB1 polymorphisms between the two groups. This study demonstrated that variations in the C3435T gene play an important role in the pathogenesis of infantile spasms in the Han Chinese population; 3435TT is associated with increased risk of having this epilepsy syndrome.

  2. Safety and efficacy of vigabatrin for the treatment of infantile spasms.

    Science.gov (United States)

    Faulkner, Michele A; Tolman, Justin A

    2011-01-01

    In 2009, vigabatrin became the first FDA approved medication for the treatment of infantile spasms in the United States. There are few well-designed prospective studies comparing the drug to placebo or other modalities used in the treatment of infantile spasms. The available data have demonstrated that vigabatrin is efficacious in the treatment of infantile spasms regardless of underlying etiology, but that it is particularly beneficial in patients with a diagnosis of tuberous sclerosis. Adrenocorticotropic hormone (ACTH), the only other medication with robust efficacy data, has been used as first line therapy for infantile spasms associated with other etiologies, and in general controls spasms sooner than vigabatrin, though relapse is common with both therapies. Vigabatrin is generally well tolerated. However, use has been associated with permanent loss of peripheral vision in some patients. In children with tuberous sclerosis, vigabatrin should be considered as initial therapy for infantile spasms. It is a viable alternative for patients with suboptimal response, contraindications or intolerance to ACTH.

  3. Safety and Efficacy of Vigabatrin for the Treatment of Infantile Spasms

    Directory of Open Access Journals (Sweden)

    Michele A. Faulkner

    2011-01-01

    Full Text Available In 2009, vigabatrin became the first FDA approved medication for the treatment of infantile spasms in the United States. There are few well-designed prospective studies comparing the drug to placebo or other modalities used in the treatment of infantile spasms. The available data have demonstrated that vigabatrin is efficacious in the treatment of infantile spasms regardless of underlying etiology, but that it is particularly beneficial in patients with a diagnosis of tuberous sclerosis. Adrenocorticotropic hormone (ACTH, the only other medication with robust efficacy data, has been used as first line therapy for infantile spasms associated with other etiologies, and in general controls spasms sooner than vigabatrin, though relapse is common with both therapies. Vigabatrin is generally well tolerated. However, use has been associated with permanent loss of peripheral vision in some patients. In children with tuberous sclerosis, vigabatrin should be considered as initial therapy for infantile spasms. It is a viable alternative for patients with suboptimal response, contraindications or intolerance to ACTH.

  4. Radial Fuzzy Systems

    Czech Academy of Sciences Publication Activity Database

    Coufal, David

    2017-01-01

    Roč. 319, 15 July (2017), s. 1-27 ISSN 0165-0114 R&D Projects: GA MŠk(CZ) LD13002 Institutional support: RVO:67985807 Keywords : fuzzy systems * radial functions * coherence Subject RIV: BA - General Mathematics OBOR OECD: Computer sciences, information science, bioinformathics (hardware development to be 2.2, social aspect to be 5.8) Impact factor: 2.718, year: 2016

  5. Perceived radial translation during centrifugation.

    NARCIS (Netherlands)

    Bos, J.E.; Correia Gracio, B.J.

    2015-01-01

    BACKGROUND: Linear acceleration generally gives rise to translation perception. Centripetal acceleration during centrifugation, however, has never been reported giving rise to a radial, inward translation perception. OBJECTIVE: To study whether centrifugation can induce a radial translation

  6. Perceived radial translation during centrifugation

    NARCIS (Netherlands)

    Bos, J.E.; Correia Grácio, B.J.

    2015-01-01

    BACKGROUND: Linear acceleration generally gives rise to translation perception. Centripetal acceleration during centrifugation, however, has never been reported giving rise to a radial, inward translation perception. OBJECTIVE: To study whether centrifugation can induce a radial translation

  7. Radially inhomogeneous bounded plasmas

    Science.gov (United States)

    Zakeri-Khatir, H.; Aghamir, F. M.

    2016-07-01

    On the basis of kinetic theory along with self-consistent field equations, the expressions for dielectric tensor of radially inhomogeneous magnetized plasma columns are obtained. The study of dielectric tensor characteristics allows the accurate analysis of the inhomogeneous properties, beyond limitations that exist in the conventional method. Through the Bessel-Fourier transformation, the localized form of material equations in a radially inhomogeneous medium are obtained. In order to verify the integrity of the model and reveal the effect of inhomogeneity, a special case of a cylindrical plasma waveguide completely filled with inhomogeneous magnetized cold plasma was considered. The dispersion relation curves for four families of electromagnetic (EH and HE) and electrostatic (SC and C) modes are obtained and compared with the findings of the conventional model. The numerical analysis indicates that the inhomogeneity effect leads to coupling of electromagnetic and electrostatic modes each having different radial eigen numbers. The study also reveals that the electrostatic modes are more sensitive to inhomogeneous effects than the electromagnetic modes.

  8. Cerebrotendinous Xanthomatosis Presenting with Infantile Spasms and Intellectual Disability.

    Science.gov (United States)

    Larson, Austin; Weisfeld-Adams, James D; Benke, Tim A; Bonnen, Penelope E

    2017-01-01

    Cerebrotendinous xanthomatosis (CTX) is an inborn error of metabolism leading to progressive multisystem disease. Symptoms often begin in the first decade of life with chronic diarrhea, cataracts, developmental delay, intellectual disability, and cerebellar or pyramidal dysfunction. Later manifestations include tendon xanthomas, polyneuropathy, and abnormal neuroimaging. Pathogenic biallelic variants in CYP27A1 leading to compromised function of sterol 27-hydroxylase result in accumulation of detectable toxic intermediates of bile acid synthesis rendering both genetic and biochemical testing effective diagnostic tools. Effective treatment with chenodeoxycholic acid is available, making early diagnosis critical for patient care. Here we report a new patient with CTX and describe the early signs of disease in this patient. Initial symptoms included infantile spasms, which have not previously been reported in CTX. Developmental delay, mild intellectual disability with measured cognitive decline in childhood, was also observed. These clinical signs do not traditionally compel testing for CTX, and we highlight the need to consider this rare but treatable disorder among the differential diagnosis of children with similar clinical presentation. Increased awareness of early signs of CTX is important for improving time to diagnosis for this patient population.

  9. Revisiting the link between hypertension and hemifacial spasm.

    Science.gov (United States)

    Leong, Jia-Li; Li, Hui-Hua; Chan, Ling-Ling; Tan, Eng-King

    2016-02-19

    The relationship between hypertension and hemifacial spasm (HFS) has been debated. Microvascular decompression surgery is effective in some HFS patients with uncontrolled hypertension. To address current gaps in knowledge, we conducted a meta-analysis of case-control studies that have examined the prevalence of hypertension in HFS patients compared to non-HFS controls. We also evaluated the implications and limitations of the pooled studies. We identified 62 studies from PubMed, The Cochrane Library, Web of Science and Scholar.google.com and six studies that fit our inclusion criteria were included. A random-effects model was used to derive the pooled estimate of the Odds Ratio. The data was plotted on a Forest plot. A pooled analysis involving 51585 subjects, 549 cases, 720 neurological controls and 50316 controls from the general population, showed that HFS patients had a higher chance of developing hypertension (OR = 1.72, 95% CI = (1.12, 2.31), p-value  <0.001). The prevalence of hypertension was higher in HFS patients as compared to non-HFS patients. This meta-analysis highlights a positive correlation between hypertension and HFS. Blood pressure should be closely monitored during the follow-up of HFS patients. Preliminary links between ventrolateral medullary (VLM) compression and HFS should be further evaluated in future studies.

  10. [Abdominal spasms, meteorism, diarrhea: fructose intolerance, lactose intolerance or IBS?].

    Science.gov (United States)

    Litschauer-Poursadrollah, Margaritha; El-Sayad, Sabine; Wantke, Felix; Fellinger, Christina; Jarisch, Reinhart

    2012-12-01

    Meteorism, abdominal spasms, diarrhea, casually obstipation, flatulence and nausea are symptoms of fructose malabsorption (FIT) and/or lactose intolerance (LIT), but are also symptoms of irritable bowel syndrome (IBS). Therefore these diseases should be considered primarily in patients with digestive complaints. For diagnosis an H(2)-breath test is used.In 1,935 patients (526 m, 1,409 f) a fructose intolerance test and in 1,739 patients (518 m,1,221 f) a lactose intolerance test was done.FIT is found more frequently than LIT (57 versus 52 % in adults (p intolerance (HIT). Headache (ca. 10 %), fatigue (ca. 5 %) and dizziness (ca. 3 %) may occur after the test, irrespective whether the test was positive or negative.In more than 2/3 of patients a diet reduced in fructose or lactose may lead to improvement or remission of these metabolic disorders. IBS, which is often correlated with FIT (183/221 patients = 83 %), can be improved by relevant but also not relevant diets indicating that irritable bowel disease seems to be caused primarily by psychological disorders.

  11. Hereditary neurometabolic causes of infantile spasms in 80 children presenting to a tertiary care center.

    Science.gov (United States)

    Alrifai, Muhammad Talal; AlShaya, Mohammed Abdullah; Abulaban, Ahmad; Alfadhel, Majid

    2014-09-01

    Infantile spasms are a devastating infantile epileptic syndrome with multiple etiologies. Hereditary neurometabolic disorders are rarely recognized causes of infantile spasms. The aim of this study was to identify hereditary neurometabolic disorders when they were the cause of infantile spasms in patients presenting to a tertiary care center in Saudi Arabia. We conducted a retrospective review of children presenting to the Pediatric Department of King Abdulaziz Medical City in Riyadh, Saudi Arabia over a 15-year interval. Eighty patients with infantile spasms were identified. A hereditary neurometabolic disorder was diagnosed in 10 patients (12.5%). Of these patients, two had a Leigh-like disorder and one patient had each of the following diagnoses: ethylmalonic aciduria, nonketotic hyperglycinemia, hyperinsulinemic hypoglycemia, leukodystrophy, short-chain acyl-coenzyme A dehydrogenase deficiency, molybdenum cofactor deficiency, primary carnitine deficiency, and neonatal hypoglycemia due to panhypopituitarism. This article is the first to report the association of the last three conditions with infantile spasms. Compared with the other etiologies, the hereditary neurometabolic disorder group had a strong history of similar disease in the same family (P = 0.002), and most of the patients were born of consanguineous parents (P = 0.021). In addition, a typical hypsarrhythmia pattern was more common in the hereditary neurometabolic disorder group (P = 0.003). Furthermore, this group had a poor response to therapy (P = 0.04). Otherwise, there were no significant differences regarding the type of spasms, neuroimaging or outcome; however, there was a trend toward poorer outcomes and death in the hereditary neurometabolic disorder group. Hereditary neurometabolic disorders are relatively common causes of infantile spasms in this subpopulation of Saudi patients. An early diagnosis via proper metabolic and genetic testing has significant implications for applying specific

  12. Infantile spasms in down syndrome: Rescue by knockdown of the GIRK2 channel.

    Science.gov (United States)

    Joshi, Krutika; Shen, Lily; Michaeli, Avner; Salter, Michael; Thibault-Messier, Gabrielle; Hashmi, Sumaiya; Eubanks, James H; Cortez, Miguel A; Snead, O Carter

    2016-10-01

    The Ts65Dn (Ts) mouse model of Down syndrome (DS) is exquisitely sensitive to an infantile spasms phenotype induced by γ-aminobutyric acidB receptor (GABAB R) agonists. The Ts mouse contains the core genomic triplication of the DS critical region, which includes 3 copies of the Kcnj6 gene that encodes the GABAB R-coupled G protein-coupled inward rectifying potassium channel subunit 2 (GIRK2) channel. We test the hypothesis that GIRK2 is necessary for the GABAB R agonist-induced infantile spasms phenotype in Ts. We assessed the result of either genetic or pharmacological knockdown of the GIRK2 channel in Ts brain upon the GABAB R agonist-induced infantile spasms phenotype in the Ts mouse model of DS. As well, we examined GABAB R currents in hippocampal neurons prepared from GIRK2-trisomic Ts control mice and GIRK2-disomic Ts mice in which Kcnj6 had been genetically knocked down from 3 to 2 copies. The reduction of the copy number of Kcnj6 in Ts mice rescued the GABAB R agonist-induced infantile spasms phenotype. There was an increase in GABAB R-mediated GIRK2 currents in GIRK2-trisomic Ts mouse hippocampal neurons, which were normalized in the GIRK2-disomic Ts mice. Similarly, pharmacological knockdown of the GIRK2 channel in Ts brain using the GIRK antagonist tertiapin-Q also rescued the GABAB R agonist-induced infantile spasms phenotype in Ts mutants. The GABAB R-coupled GIRK2 channel is necessary for the GABAB R agonist-induced infantile spasms phenotype in the Ts mouse and may represent a novel therapeutic target for the treatment of infantile spasms in DS. Ann Neurol 2016;80:511-521. © 2016 American Neurological Association.

  13. MRI findings in infants with infantile spasms after neonatal hypoxic-ischemic encephalopathy.

    Science.gov (United States)

    Gano, Dawn; Sargent, Michael A; Miller, Steven P; Connolly, Mary B; Wong, Peter; Glass, Hannah C; Poskitt, Kenneth J; Chau, Vann

    2013-12-01

    To evaluate the predominant pattern of brain injury and the anatomic areas of injury in children with infantile spasms following neonatal hypoxic-ischemic encephalopathy. A nested case-control study of infantile spasms in children with term neonatal hypoxic-ischemic encephalopathy was performed. All patients had T1/T2-weighted magnetic resonance imaging with diffusion-weighted imaging performed on the third day of life. Using a validated scoring system, the magnetic resonance imaging was classified as: normal, watershed, basal ganglia/thalamus, total, or focal-multifocal. Two study investigators scored additional anatomic areas of injury (cortical extent, levels of the brainstem, hypothalamus) on T1/T2-weighted magnetic resonance imaging and diffusion-weighted imaging blinded to the outcome. The predominant pattern of brain injury and anatomic areas of injury were compared between patients who developed infantile spasms and randomly selected controls. Eight patients who developed infantile spasms were identified among a cohort of 176 term newborns with hypoxic-ischemic encephalopathy (4.5%). There were no significant differences in the perinatal and neonatal course between newborns who developed infantile spasms and controls who did not. The development of infantile spasms after neonatal hypoxic-ischemic encephalopathy was significantly associated with basal ganglia/thalamus and total brain injury (P = 0.001), extent of cortical injury greater than 50% (odds ratio = 11.7, 95% confidence interval = 1.1-158.5, P = 0.01), injury to the midbrain (odds ratio = 13, 95% confidence interval = 1.3-172, P = 0.007) and hypothalamic abnormalities (P = 0.01). The development of infantile spasms after hypoxic-ischemic encephalopathy is associated with injury to the basal ganglia and thalami on neonatal magnetic resonance imaging, particularly when extensive cortical injury and/or injury to the midbrain is present. Copyright © 2013 Elsevier Inc. All rights reserved.

  14. Vigabatrin as First-Line Treatment for Infantile Spasms Not Related to Tuberous Sclerosis Complex.

    Science.gov (United States)

    Jones, Kevin; Go, Cristina; Boyd, Jennifer; Ochi, Ayako; McCoy, Blathnaid; Puka, Klajdi; Snead, O Carter

    2015-08-01

    Infantile spasms are a rare, catastrophic, age-specific seizure disorder of infancy. Adrenocorticotropic hormone or vigabatrin have been recommended for the short-term treatment of infantile spasms by the Child Neurology Society/American Academy of Neurology practice parameter. This retrospective study reviewed the Hospital for Sick Children's experience with the short-term efficacy of vigabatrin as first-line treatment for infantile spasms not related to tuberous sclerosis complex. We performed a single-center, retrospective analysis of all cases of newly diagnosed infantile spasms between January 2010 and September 2013. Duration of follow-up was at least 6 months from treatment initiation. Eighteen of the 61 infants (30%) had a clinical and electrographic response to vigabatrin therapy within 4 weeks of treatment. Of the vigabatrin responders, 2/18 (11%) relapsed. At final follow-up after initiation of vigabatrin therapy, 17/61 (27%) of the vigabatrin responders were free of all clinical seizure types. Normal development at the time of infantile spasms diagnosis was statistically associated with vigabatrin response. These findings do not support our hypothesis that vigabatrin is effective as first-line, short-term treatment of infantile spasms in non-tuberous sclerosis complex patients. However, when used in this setting, vigabatrin is most effective in children with normal development at the time of diagnosis. These findings may assist clinicians in the optimal treatment choice for children at the first presentation of infantile spasms not related to tuberous sclerosis complex. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Long-term prognosis after infantile spasms: a statistical study of prognostic factors in 200 cases.

    Science.gov (United States)

    Matsumoto, A; Watanabe, K; Negoro, T; Sugiura, M; Iwase, K; Hara, K; Miyazaki, S

    1981-02-01

    A follow-up study was made on 200 children (115 boys, 85 girls) who had had infantile spasms, in order to compare their present condition over the age of six years with various prognostic factors. 48 of the children (30 males and 18 females) had died, and all the rest were aged six years or older at the time of final follow-up. 139 of the children had received ACTH therapy: at final follow-up, spasms had ceased in 43.5 per cent, and about the same proportion showed normal physical development; 23 per cent had normal mental development and 15.4 per cent were attending ordinary schools. Complete recovery (normal mental and physical development and attending ordinary schools) was achieved in only 19 cases (9.5 per cent). Of the cryptogenic cases, 44.4 per cent had made a full recovery. The poor prognostic factors for continuing seizures were evolution into other types of fits, relapse of seizures after ACTH therapy, seizures concomitant with spasms, and convulsions before the onset of spasms. Poor prognostic factors for physical development were delayed development before the onset of spasms, neurological abnormalities, PEG abnormality, symptomatic aetiology, neonatal convulsions, low birthweight, perinatal asphyxia and being female. Poor prognostic factors for mental development were delayed development before the onset of spasms, neurological abnormalities, PEG abnormality, prenatal and perinatal aetiology, relapse after initial ACTH therapy, laughing attacks, and evolution into other types of fits. Only in the cryptogenic cases was there significant correlation between the delay in treatment and the long-term prognosis for mental development. Poor prognostic factors for educability were very similar to those for mental development. In spite of conflicting views as to the long-term effects of ACTH, prompt treatment seems to be mandatory, at least in cryptogenic cases of infantile spasms.

  16. The ARCS radial collimator

    Directory of Open Access Journals (Sweden)

    Stone M.B.

    2015-01-01

    Full Text Available We have designed, installed, and commissioned a scattered beam radial collimator for use at the ARCS Wide Angular Range Chopper Spectrometer at the Spallation Neutron Source. The collimator has been designed to work effectively for thermal and epithermal neutrons and with a range of sample environments. Other design considerations include the accommodation of working within a high vacuum environment and having the ability to quickly install and remove the collimator from the scattered beam. We present here characterization of the collimator's performance and methodologies for its effective use.

  17. Radial cutting torch

    Energy Technology Data Exchange (ETDEWEB)

    Robertson, M.C.

    1997-01-08

    The project`s aim is to complete development of the Radial Cutting Torch, a pyrotechnic cutter, for use in all downhole tubular cutting operations in the petroleum industry. Project objectives are to redesign and pressure test nozzle seals to increase product quality, reliability, and manufacturability; improve the mechanical anchor to increase its temperature tolerance and its ability to function in a wider variety of wellbore fluids; and redesign and pressure test the RCT nozzle for operation at pressures from 10 to 20 ksi. The proposal work statement is included in the statement of work for the grant via this reference.

  18. Harvesting the radial artery.

    Science.gov (United States)

    Blitz, Arie; Osterday, Robert M; Brodman, Richard F

    2013-07-01

    The radial artery (RA) has emerged as an important arterial graft for coronary bypass surgery. With improving five-year patency rates and increasing uptake, great attention has been focused on the optimal conduit harvesting technique. We herein present our approach to RA harvesting. Prerequisites of a successful harvest include adherence to important anatomical landmarks, protection of the sensory innervation to the volar forearm, and meticulous handling of the RA branches. Regardless of the harvesting methodology chosen, adherence to a "no-touch" technique will optimize the patency and durability of the RA conduit.

  19. Radial Bragg Resonators

    Science.gov (United States)

    Scheuer, Jacob; Sun, Xiankai

    Circular resonators are promising candidates for a wide range of applications, ranging from optical communication systems through basic research involving highly confined fields and strong photon-atom interactions to biochemical and rotation sensing. The main characteristics of circular resonators are the Q factor, the free spectral range (FSR), and the modal volume, where the last two are primarily determined by the resonator radius. The total internal reflection (TIR) mechanism used for guidance in "conventional" resonators couples these attributes and limits the ability to realize compact devices exhibiting large FSR, small modal volume, and high Q. Recently, a new class of annular resonator, based on a single defect surrounded by radial Bragg reflectors, has been proposed and analyzed. The radial Bragg confinement decouples the modal volume from the Q and paves the way for the realization of compact, low-loss resonators. These properties as well as the unique mode profile of these circular Bragg nanoresonators (CBNRs) and nanolasers (CBNLs) make the devices within this class an excellent tool to realize nanometer scale semiconductor lasers and ultrasensitive detectors, as well as to study nonlinear optics.

  20. Prevention

    Science.gov (United States)

    ... Error processing SSI file About Heart Disease & Stroke Prevention Heart disease and stroke are an epidemic in ... secondhand smoke. Barriers to Effective Heart Disease & Stroke Prevention Many people with key risk factors for heart ...

  1. The minimum coronary artery diameter in which coronary spasm can be identified by synchrotron radiation coronary angiography

    International Nuclear Information System (INIS)

    Matsushita, Shonosuke; Hyodo, Kazuyuki; Imazuru, Tomohiro; Tokunaga, Chiho; Sato, Fujio; Enomoto, Yoshiharu; Hiramatsu, Yuji; Sakakibara, Yuzuru

    2008-01-01

    Background: Coronary vasospasm is defined as a temporary, intense narrowing of the coronary conduit artery. It brings about ischemic chest pain and becomes one of the causes of myocardial infarction. Coronary spasms are divided into two categories. One is the coronary spasm of the conduit artery and the other is the coronary microvascular spasm. Although coronary spasms are diagnosed with the images of coronary angiography, microvascular spasms cannot be diagnosed because of the limitations of conventional angiographic systems. However, synchrotron radiation coronary angiography (SRCA) can identify coronary arteries down to 100 μm in diameter in the beating heart and 50 μm in arrested heart. Aim: The purpose of this study was to confirm whether microvascular spasms could be identified or not using SRCA, and then down that size identification was possible. Methods: The Langendorff perfusion system with isolated rat hearts was employed. Krebs-Henseleit solution (KH solution) was used as a perfusate. 10 mM of 4-aminopyridine (4-AP: a voltage-gated potassium channel blocker; spasm inducer) was added to the KH solution and maintained for 5 min. SRCA was performed at pre-, during and 10 min after cessation of the KH solution with 4-AP. Coronary spasms were defined as a temporal 75% reduction of coronary arterial diameter. Results and conclusion: Multiple sizes of coronary arteries showed coronary spasms. The minimum stenosed coronary artery size was 100 μm. Since coronary microvascular spasms are seen in the arterioles (50-400 μm), coronary microvascular spasms may be diagnosed with the use of synchrotron radiation coronary angiography

  2. The response to ACTH is determined early in the treatment of infantile spasms.

    Science.gov (United States)

    Mytinger, John R; Weber, Amanda; Heyer, Geoffrey L

    2015-03-01

    Although adrenocorticotropic hormone is the most commonly used treatment for infantile spasms in the United States, the optimal regimen for this indication is not known. The purpose of this study was to elucidate the optimal adrenocorticotropic hormone treatment duration. We conducted a retrospective chart review of response to adrenocorticotropic hormone among all patients with infantile spasms managed at our institution from January 2009 to September 2013. Treatment response was defined as clinical remission for greater than or equal to 28 days starting at any point within the adrenocorticotropic hormone course and remission of hypsarrhythmia (or definite EEG improvement if hypsarrhythmia was absent at baseline). For responders, the diagnostic and post-treatment EEG tracings were reviewed. Electroclinical remission was achieved in 21 of 39 patients (54%) receiving adrenocorticotropic hormone, including 11/25 (44%) receiving a long course (typically 12 weeks) and 10/14 (71%) receiving a short course (typically four weeks). The mean time to clinical remission was 5.8 days (median: 5 days; range: 1-20 days). Only one patient responded beyond two weeks of treatment. This study provides Class IV evidence that among patients with infantile spasms, the response to adrenocorticotropic hormone is most often determined early in the treatment course. Given the importance of rapid remission, clinicians should consider adding or changing treatment if infantile spasms do not resolve within two weeks of adrenocorticotropic hormone initiation. Further study is needed to determine the optimal adrenocorticotropic hormone regimen for infantile spasms.

  3. Genetic testing in infantile spasms identifies a chromosome 13q deletion and retinoblastoma.

    Science.gov (United States)

    Jones, Kevin; Minassian, Berge A

    2014-05-01

    Infantile spasms is an epileptic encephalopathy and the common final manifestation of numerous disparate insults to the developing brain during infancy. The varied etiologies may be structural, metabolic, genetic, or unknown. Etiological diagnosis is important as it may lead to specific therapy, which may affect developmental outcome. We report a case of infantile spasms of unknown etiology with dysmorphic features, in which genetic copy number variation microarray testing was included in the investigation of the cause of the disease. A large deletion of chromosome 13 was identified in the region 13q13 to 13q21.3 encompassing the retinoblastoma gene (13q14.2). Urgent ophthalmological evaluation revealed an asymptomatic retinoblastoma of the left eye, leading to early treatment. This is the first case report of infantile spasms specifically associated with a chromosome 13q deletion. Chromosomal region 13q13 to 13q21.3 may contain one or more genes whose hemizygous loss leads to infantile spasms. Copy number variation testing for cryptogenic infantile spasms led to the discovery of a mutation responsible for retinoblastoma, enabling early diagnosis and treatment of a potentially life-threatening cancer. High-sensitivity molecular diagnosis improves health care and substantially reduces expenses. This shift in diagnostic evaluation is broadly relevant to health care. Crown Copyright © 2014. Published by Elsevier Inc. All rights reserved.

  4. Use of the modified Atkins diet in infantile spasms refractory to first-line treatment.

    Science.gov (United States)

    Sharma, Suvasini; Sankhyan, Naveen; Gulati, Sheffali; Agarwala, Anuja

    2012-01-01

    This prospective, open label, uncontrolled study was performed to evaluate the efficacy and tolerability of the modified Atkins diet in children with refractory infantile spasms. Fifteen consecutive children aged six months to three years having daily infantile spasms in clusters with electroencephalographic evidence of hypsarrhythmia despite treatment with hormonal treatment (oral corticosteroids/adrenocorticotrophic hormone) and/or vigabatrin, and at least one additional anti-epileptic drug were enrolled. Children with known or suspected inborn errors of metabolism or systemic illnesses were excluded. Carbohydrate intake was restricted to ten grams/day. Among these 12 boys and three girls (median age-24 months), 13 had symptomatic etiology. After three months of diet, six children were spasm free. The time to spasm freedom after diet initiation ranged from two days to two months. The most frequent adverse effect observed was constipation. The modified Atkins diet was found to be effective and well tolerated in children with refractory infantile spasms (ClinicalTrials.gov identifier: NCT01006811). Copyright © 2011 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  5. Screening of Inherited Metabolic Disorders in Infants with Infantile Spasms.

    Science.gov (United States)

    Liu, Xiao-Ming; Li, Rui; Chen, Sheng-Zhi; Sang, Yan; Chen, Jiao; Fan, Cong-Hai

    2015-05-01

    The objective of this study is to explore the incidence of inherited metabolic disorders (IMD) in infants with infantile spasms (IS), with an attempt to improve the early diagnosis and etiological and symptomatic treatment. Urine and blood samples were collected from 60 IS patients and analyzed for the quantification of amino acids, organic acids, and fatty acids by gas chromatography-mass spectrometry and tandem mass spectrum. Routine urine tests, hepatic function tests, blood biochemistry, brain imaging, as well as examinations of the brain stem auditory/visual evoked potentials were also examined. In addition to antiepileptic therapy, etiological and symptomatic treatments were also conducted in infants with confirmed IMD and the follow-up lasted for 6 months in these pediatric patients. Metabolic disorders were found in 28 (46.67 %) of 60 IS infants, among them 13 (21.67 %) were confirmed to be with IMD. Twelve of these 13 IS patients with definite IMD diagnoses (92.31 %) experienced varying degrees of delayed development of intelligence and motor function, 8 patients (61.54 %) had abnormal cranial CT or MRI findings, 11 patients (84.61 %) had abnormal brain stem evoked potentials, 4 patients (30.77 %) had abnormal hepatic functions, 3 patients (23.07 %) had abnormal blood biochemistry, 2 patients (15.38 %) had positive (+ to ++) results for routine urine ketones, and 2 patients (15.38 %) had skin lesions. After treatment in children who were diagnosed IMD, the well controlled epileptic seizures and the satisfactory developments in mental and motor were found in 4 cases of methylmalonic acidemia, 2 cases of classical phenylketonuria, and one case of biotin deficiency disease, glutaric acidemia type I, and 4-hydroxybutyric aciduria in each. IMD is a key biological cause in IS. Early screening for IMD is warranted in IS infants to facilitate the improvement for the prognosis and an early etiological treatment.

  6. [Immunological mechanism of prednisone in the treatment of infantile spasm].

    Science.gov (United States)

    Chen, Hui; Zhong, Jian-Min; Yi, Zhao-Shi; Zha, Jian; Chen, Yong; Cai, Lan-Yun

    2017-10-01

    To investigate the immunological mechanism of prednisone in the treatment of infantile spasm (IS) by evaluating the immune function of IS children before and after treatment. Thirty children with IS were enrolled as IS group. Thirty healthy infants who underwent physical examination were enrolled as healthy control group. Fasting venous blood was collected for both groups before and after prednisone treatment. Chemiluminescence was used to measure serum levels of interleukin-1B (IL-1B), interleukin-2R (IL-2R), interleukin-6 (IL-6), interleukin-8 (IL-8), and tumor necrosis factor-α (TNF-α). Immunoturbidimetric assay was used to measure serum levels of immunoglobulin A (IgA), immunoglobulin M (IgM), and immunoglobulin G (IgG). Flow cytometry was used to measure the percentages of T lymphocyte subsets (CD3 + , CD4 + , and CD8 + ). The clinical outcome and electroencephalographic findings were evaluated for all IS children after prednisone treatment. The IS group had significantly higher serum levels of IL-2R, IL-8, and TNF-α than the healthy control group before treatment (Ptreatment, the IS group had significant reductions in the numbers of daily ictal clusters and total daily seizures, significant improvement in developmental quotient (P<0.05), and significant reductions in serum levels of IL-2R, L-8, and TNF-α, the percentage of CD4 + T lymphocytes, and CD4 + /CD8 + ratio (P<0.05), as well as a significant increase in the percentage of CD8 + T lymphocytes (P<0.05). IS children have immune dysfunction. Prednisone can control seizures in IS children, possibly by regulating and improving immune dysfunction.

  7. ISR Radial Field Magnet

    CERN Multimedia

    1983-01-01

    There were 37 (normal) + 3 (special) Radial Field magnets in the ISR to adjust vertically the closed orbit. Gap heights and strengths were 200 mm and .12 Tm in the normal magnets, 220 mm and .18 Tm in the special ones. The core length was 430 mm in both types. Due to their small length as compared to the gap heights the end fringe field errors were very important and had to be compensated by suitably shaping the poles. In order to save on cables, as these magnets were located very far from their power supplies, the coils of the normal type magnets were formed by many turns of solid cpper conductor with some interleaved layers of hollow conductor directly cooled by circulating water

  8. Clinical Review of the Effects of Hominis Placental Pharmacopuncture in the Treatment of Facial Spasm Patients

    Directory of Open Access Journals (Sweden)

    Jo Na-Young

    2013-09-01

    Full Text Available Objectives: The main purpose of this research is to investigate the effect of treatment with Hominis Placental pharmacopuncture (HPP for 32 patients with hemifacial spasm. Methods: We treated facial spasm patients with acupuncture and HPP at Sabaek (ST2, Seung-eup (ST1, Gwallyeo (SI18, Chanjuk (BL2, Sajukgong (TE23, Hagwan (ST7, Hyeopgeo (ST6, Jichang (ST4, Wan-gol (SI4 and Yepung (TE17, and we investigated the effect by using Scott’s scale. The data were analyzed by using the SPSS/10.0 for windows program with descriptive statistics, the paired t-test, and the Shapiro-Wilk normality test. Results: After treatment, the grade of the spasm’s intensity based on Scott’s description were decreased significantly. About 72% of the patients felt that the combination treatment had produced excellent results. Conclusion: These data suggested that HPP can be useful for treating facial spasm patients.

  9. Noninvasive ergonovine maleate provocative testing for coronary artery spasm: the need for routine thallium-201 imaging

    International Nuclear Information System (INIS)

    Shanes, J.G.; Krone, R.J.; Fisher, K.; Shah, B.; Eisenkramer, G.; Humphrey, J.R.

    1983-01-01

    We administered ergonovine and used both electrocardiographic monitoring and thallium- 201 [ 201 Tl] imaging to detect reversible ischemia in 100 patients. Patients already established as having coronary artery spasm and those with nonbypassed, proximal, high-grade coronary artery stenosis were excluded. No complication occurred in any patient. The use of thallium imaging in addition to electrocardiographic monitoring resulted in a higher degree of sensitivity than did ECG monitoring alone. Fourteen patients demonstrated evidence of coronary artery spasm as documented by 201 Tl imaging but of the 14, significant ECG changes occurred in only 50%, and classic ST segment elevation in 21%. Thus, in carefully selected patients the noninvasive provocation of coronary spasm can be accomplished safely, but ECG monitoring must be combined with thallium- 201 imaging to achieve an acceptable degree of sensitivity

  10. Clinical profile and treatment of infantile spasms using vigabatrin and ACTH - a developing country perspective

    Science.gov (United States)

    2010-01-01

    Background Infantile spasms represent a serious epileptic syndrome that occurs in the early infantile age. ACTH and Vigabatrin are actively investigated drugs in its treatment. This study describes the comparison of their efficacy in a large series of patients with infantile spasms from Pakistan. Methods All patients with infantile spasms who presented to Aga Khan University Hospital, Karachi, Pakistan from January, 2006 to April, 2008 were included in this study. Inclusion criteria were clinical symptoms of infantile spasms, hypsarrythmia or modified hyparrythmia on electroencephalography, at least six months of follow-up period and receipt of any of the two drugs mentioned above. The type of drug distribution was random according to the availability, cost and ease of administration. Results Fifty six cases fulfilled the inclusion criteria. 62.5% were males. Mean age at onset of seizures was 5 ± 1.4 months. Fifty two (92.8%) patients demonstrated hypsarrythmia on electroencephalography. 64.3% cases were identified as symptomatic while 19.6% were cryptogenic and 16.1% were idiopathic. Eighteen patients received ACTH while 38 patients received Vigabatrin as first line therapy. Initial response to first line therapy was similar (50% for ACTH and 55.3% for Vigabatrin). Overall, the symptomatic and idiopathic groups responded better to Vigabatrin. The relapse rate was higher for ACTH as compared to Vigabatrin (55.5% vs. 33.3%) when considering the first line therapy. Four patients evolved to Lennox-Gastaut variant; all of these patients had initially received Vigabatrin and then ACTH. Conclusion Vigabatrin and ACTH showed no significant difference in the initial treatment of infantile spasms. However, patients receiving ACTH were 1.2 times more likely to relapse as compared to the patients receiving Vigabatrin when considering monotherapy. We suggest that Vigabatrin should be the initial drug of choice in patients presenting with infantile spasms. However, larger

  11. Clinical profile and treatment of infantile spasms using vigabatrin and ACTH - a developing country perspective

    Directory of Open Access Journals (Sweden)

    Ishaque Sidra

    2010-01-01

    Full Text Available Abstract Background Infantile spasms represent a serious epileptic syndrome that occurs in the early infantile age. ACTH and Vigabatrin are actively investigated drugs in its treatment. This study describes the comparison of their efficacy in a large series of patients with infantile spasms from Pakistan. Methods All patients with infantile spasms who presented to Aga Khan University Hospital, Karachi, Pakistan from January, 2006 to April, 2008 were included in this study. Inclusion criteria were clinical symptoms of infantile spasms, hypsarrythmia or modified hyparrythmia on electroencephalography, at least six months of follow-up period and receipt of any of the two drugs mentioned above. The type of drug distribution was random according to the availability, cost and ease of administration. Results Fifty six cases fulfilled the inclusion criteria. 62.5% were males. Mean age at onset of seizures was 5 ± 1.4 months. Fifty two (92.8% patients demonstrated hypsarrythmia on electroencephalography. 64.3% cases were identified as symptomatic while 19.6% were cryptogenic and 16.1% were idiopathic. Eighteen patients received ACTH while 38 patients received Vigabatrin as first line therapy. Initial response to first line therapy was similar (50% for ACTH and 55.3% for Vigabatrin. Overall, the symptomatic and idiopathic groups responded better to Vigabatrin. The relapse rate was higher for ACTH as compared to Vigabatrin (55.5% vs. 33.3% when considering the first line therapy. Four patients evolved to Lennox-Gastaut variant; all of these patients had initially received Vigabatrin and then ACTH. Conclusion Vigabatrin and ACTH showed no significant difference in the initial treatment of infantile spasms. However, patients receiving ACTH were 1.2 times more likely to relapse as compared to the patients receiving Vigabatrin when considering monotherapy. We suggest that Vigabatrin should be the initial drug of choice in patients presenting with infantile

  12. Clinical profile and treatment of infantile spasms using vigabatrin and ACTH--a developing country perspective.

    Science.gov (United States)

    Ibrahim, Shahnaz; Gulab, Shamshad; Ishaque, Sidra; Saleem, Taimur

    2010-01-15

    Infantile spasms represent a serious epileptic syndrome that occurs in the early infantile age. ACTH and Vigabatrin are actively investigated drugs in its treatment. This study describes the comparison of their efficacy in a large series of patients with infantile spasms from Pakistan. All patients with infantile spasms who presented to Aga Khan University Hospital, Karachi, Pakistan from January, 2006 to April, 2008 were included in this study. Inclusion criteria were clinical symptoms of infantile spasms, hypsarrythmia or modified hyparrythmia on electroencephalography, at least six months of follow-up period and receipt of any of the two drugs mentioned above. The type of drug distribution was random according to the availability, cost and ease of administration. Fifty six cases fulfilled the inclusion criteria. 62.5% were males. Mean age at onset of seizures was 5 +/- 1.4 months. Fifty two (92.8%) patients demonstrated hypsarrythmia on electroencephalography. 64.3% cases were identified as symptomatic while 19.6% were cryptogenic and 16.1% were idiopathic. Eighteen patients received ACTH while 38 patients received Vigabatrin as first line therapy. Initial response to first line therapy was similar (50% for ACTH and 55.3% for Vigabatrin). Overall, the symptomatic and idiopathic groups responded better to Vigabatrin. The relapse rate was higher for ACTH as compared to Vigabatrin (55.5% vs. 33.3%) when considering the first line therapy. Four patients evolved to Lennox-Gastaut variant; all of these patients had initially received Vigabatrin and then ACTH. Vigabatrin and ACTH showed no significant difference in the initial treatment of infantile spasms. However, patients receiving ACTH were 1.2 times more likely to relapse as compared to the patients receiving Vigabatrin when considering monotherapy. We suggest that Vigabatrin should be the initial drug of choice in patients presenting with infantile spasms. However, larger studies from developing countries are

  13. Infantile Spasms and Injuries of Prematurity: Short-Term Treatment-Based Response and Long-Term Outcomes.

    Science.gov (United States)

    Wallace, Adam; Allen, Victoria; Park, Kristen; Knupp, Kelly

    2017-09-01

    The association of infantile spasms and periventricular leukomalacia and/or intraventricular hemorrhage is well documented. Data regarding early treatment-based and long-term outcomes are limited. A retrospective chart review identified children with infantile spasms born prematurely (infantile spasms was 8 months. Nine children had intraventricular hemorrhage, 10 had periventricular leukomalacia, and 6 children had both. Twelve of 13 children had resolution of spasms. In responders, the successful medication was adrenocorticotropic hormone (ACTH) in 7, topiramate in 3, and vigabatrin in 2. Follow-up after a median of 7.1 years found that all patients had developmental delay but only 1 had refractory epilepsy. Standard therapies (ACTH and vigabatrin) appeared to be more effective than other treatments. Developmental delay is common in children with periventricular leukomalacia / intraventricular hemorrhage and infantile spasms, but refractory epilepsy might be less frequent.

  14. [Ischemic cardiopathy with normal epicardial coronary arteries. Probable spasm of the coronary arteries. A case report].

    Science.gov (United States)

    Jorge, P A; Bolsonaro, L R; Jorge, L R

    1989-02-01

    The authors discuss the case of a young woman, with chest pain at rest and normal coronary arteries. Sixty-three days after the onset of the symptoms the patient had a myocardial infarction. The coronariogram showed obstruction of the left coronary artery in its full length. The authors consider that a coronary spasm was the cause of the infarction. It is very likely that independent of personal characteristics, coronary spasm may be persistent, involve the whole extent of the vessel and occur even with use of vasodilator drugs.

  15. Convergence spasm due to aquaporin-positive neuromyelitis optica spectrum disorder

    Directory of Open Access Journals (Sweden)

    Pınar Özçelik

    2017-06-01

    Full Text Available A female 27 presented with nausea and diplopia for 1 week. On examination she had normal vertical gaze but would develop convergence with miosis whenever she made horizontal saccades. Pupils were 6 mm and unreactive to light. MRI showed extensive hyperintensity in the dorsal midbrain and thalamus. Spinal MRI and CSF were both normal. Serum aquaporin-4-antibody was positive. She was treated with steroids and plasmapheresis and after 3 months convergence spasm resolved but pupils remained unreactive. Neuromyelitis optica often presents with brainstem signs, rarely a dorsal midbrain syndrome. Convergence spasm is occasionally of organic neurologic origin.

  16. Radial flow heat exchanger

    Science.gov (United States)

    Valenzuela, Javier

    2001-01-01

    A radial flow heat exchanger (20) having a plurality of first passages (24) for transporting a first fluid (25) and a plurality of second passages (26) for transporting a second fluid (27). The first and second passages are arranged in stacked, alternating relationship, are separated from one another by relatively thin plates (30) and (32), and surround a central axis (22). The thickness of the first and second passages are selected so that the first and second fluids, respectively, are transported with laminar flow through the passages. To enhance thermal energy transfer between first and second passages, the latter are arranged so each first passage is in thermal communication with an associated second passage along substantially its entire length, and vice versa with respect to the second passages. The heat exchangers may be stacked to achieve a modular heat exchange assembly (300). Certain heat exchangers in the assembly may be designed slightly differently than other heat exchangers to address changes in fluid properties during transport through the heat exchanger, so as to enhance overall thermal effectiveness of the assembly.

  17. APC conditional knock-out mouse is a model of infantile spasms with elevated neuronal β-catenin levels, neonatal spasms, and chronic seizures.

    Science.gov (United States)

    Pirone, Antonella; Alexander, Jonathan; Lau, Lauren A; Hampton, David; Zayachkivsky, Andrew; Yee, Amy; Yee, Audrey; Jacob, Michele H; Dulla, Chris G

    2017-02-01

    Infantile spasms (IS) are a catastrophic childhood epilepsy syndrome characterized by flexion-extension spasms during infancy that progress to chronic seizures and cognitive deficits in later life. The molecular causes of IS are poorly defined. Genetic screens of individuals with IS have identified multiple risk genes, several of which are predicted to alter β-catenin pathways. However, evidence linking malfunction of β-catenin pathways and IS is lacking. Here, we show that conditional deletion in mice of the adenomatous polyposis coli gene (APC cKO), the major negative regulator of β-catenin, leads to excessive β-catenin levels and multiple salient features of human IS. Compared with wild-type littermates, neonatal APC cKO mice exhibit flexion-extension motor spasms and abnormal high-amplitude electroencephalographic discharges. Additionally, the frequency of excitatory postsynaptic currents is increased in layer V pyramidal cells, the major output neurons of the cerebral cortex. At adult ages, APC cKOs display spontaneous electroclinical seizures. These data provide the first evidence that malfunctions of APC/β-catenin pathways cause pathophysiological changes consistent with IS. Our findings demonstrate that the APC cKO is a new genetic model of IS, provide novel insights into molecular and functional alterations that can lead to IS, and suggest novel targets for therapeutic intervention. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Prevention

    Science.gov (United States)

    ... Contact Aging & Health A to Z Find a Geriatrics Healthcare Professional Medications & Older Adults Making Your Wishes ... Prevention Hearing Loss Heart Attack High Blood Pressure Nutrition Osteoporosis Shingles Skin Cancer Related News Quitting Smoking, ...

  19. Hemifacial spasm in a patient with basilar artery dolichoectasia caused by uncontrolled hypertension

    Directory of Open Access Journals (Sweden)

    Gordon S. Crabtree

    2016-10-01

    Full Text Available A 47-year-old male presented with a 2-year history of hemifacial spasm. Magnetic resonance imaging performed showed his tortuous basilar artery with nerve compression, and the patient was treated conservatively with botulinum toxin injections with complete resolution of symptoms. This rare disease was caused by his long history of hypertension, which led to his major basilar artery dolichoectasia.

  20. Modified Ashworth scale and spasm frequency score in spinal cord injury

    DEFF Research Database (Denmark)

    Baunsgaard, C. B.; Nissen, U. V.; Christensen, K. B.

    2016-01-01

    STUDY DESIGN: Intra- and inter-rater reliability study. OBJECTIVES: To assess intra- and inter-rater reliability of the Modified Ashworth Scale (MAS) and Spasm Frequency Score (SFS) in lower extremities in a population of spinal cord-injured persons, as well as correlations between the two scales...

  1. A case of convergence spasms – do not be caught off-guard

    African Journals Online (AJOL)

    the primary position with the eyes adducted (turned toward the nose bridge). Fluctuating esotropia was present. When the object for fixation was held closer than 30 cm to examine the eye ... lesions in multiple sclerosis,[5] myasthenia gravis and Wernicke's encephalopathy.[1] Patients with functional spasm of the near reflex.

  2. SCN2A mutation in a Chinese boy with infantile spasm - response to Modified Atkins Diet.

    Science.gov (United States)

    Wong, Virginia C N; Fung, C W; Kwong, Anna K Y

    2015-08-01

    Mutation of SCN2A, encoding for voltage-gated sodium channel type II alpha subunit, has been demonstrated in various epilepsy phenotypes, ranging from benign to severe epileptic disorders and recently this had been reported for cases with infantile spasm (IS). We study a 6 years-old Chinese boy with severe developmental delay who had infantile spasm since 15 months. He later had severe intellectual disability and autistic features. He failed to respond to most anticonvulsants. Modified Atkins Diet was introduced at 4 years of age and he showed a seizure remission for 12 months with only 1 anticonvulsants. To clarify the unknown etiology, mutations were screened for genes associated with brain development or synaptic function. A heterozygous mutation (c.3631G>A; p.E1211K) was identified in exon 21 of SCN2A gene. This mutation has been reported previously only in a Japanese patient with IS. This is the first case of SCN2A mutation identified in Chinese. Similarity of our case and one Japanese case of infantile spasm indicated that this E1211K mutation is important as possible etiology of IS. Trial of Modified Atkins Diet for other cases of infantile spasm with similar SCN2A mutations is worthwhile pursuing. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  3. Effectiveness of once-daily high-dose ACTH for infantile spasms.

    Science.gov (United States)

    Hodgeman, Ryan M; Kapur, Kush; Paris, Ann; Marti, Candice; Can, Afra; Kimia, Amir; Loddenkemper, Tobias; Bergin, Ann; Poduri, Annapurna; Libenson, Mark; Lamb, Nathan; Jafarpour, Saba; Harini, Chellamani

    2016-06-01

    There is insufficient evidence to recommend a specific protocol for treatment of infantile spasms (IS) and a lack of standardization among, and even within, institutions. Twice-daily dosing (for the first two weeks) of high-dose natural ACTH for IS is used by many centers and recommended by the National Infantile Spasms Consortium (NISC). Conversely, it is our practice to use once-daily dosing of high-dose natural ACTH for IS. In order to determine the effectiveness of our center's practice, we retrospectively reviewed 57 cases over the past four years at Boston Children's Hospital (BCH). We found that 70% of infants were spasm-free at 14days from ACTH initiation and 54% continued to be spasm-free at 3-month follow-up. Electroencephalogram showed resolution of hypsarrhythmia (when present on the pretreatment EEG) in all responders. Additionally, once-daily dosing of ACTH was well tolerated. We performed a meta-analysis to compare our results against the reports of published literature using twice-daily high-dose ACTH for treatment of IS. The meta-analysis revealed that our results were comparable to previously published outcomes using twice-daily ACTH administration for IS treatment. Our experience shows that once-daily dosing of ACTH is effective for treatment of IS. If larger prospective trials can confirm our findings, it would obviate the need for additional painful injections, simplify the schedule, and support a universal standardized protocol. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. An unknown combination of infantile spasms, retinal lesions, facial dysmorphism and limb abnormalities

    NARCIS (Netherlands)

    Plomp, A. S.; Reardon, W.; Benton, S.; Taylor, D.; Larcher, V. F.; Sundrum, R.; Winter, R. M.

    2000-01-01

    A female patient is presented with infantile spasms, punched-out retinal lesions, facial dysmorphism, short upper arms, short thumbs, left lower limb hypoplasia with foot deformity, a hemivertebra, atrial septal defect, growth retardation and severe developmental delay. There is some similarity to

  5. Practice experience in the treatment of infantile spasms at a tertiary care center.

    Science.gov (United States)

    Thodeson, Drew; Sogawa, Yoshimi

    2014-11-01

    The current treatment guidelines for treatment of infantile spasms is ambiguous regarding individuals with known etiology and is backed by limited evidence. Recently published survey data reveal diverse treatment variation for infantile spasms. We conducted a retrospective medical record review to better understand the clinical variables which affect treatment selection for new-onset infantile spasms. We systematically extracted demographic data and treatment response of children with new onset infantile spasms over a 3-year period at a single institution. Treatment was divided into three groups: vigabatrin, hormone treatment, and other therapies. Our final cohort had 65 patients; 74% had a known etiology. Sixty-two percent were initially treated with vigabatrin. Other therapies were used more often in known etiology than in unknown etiology as initial treatment (40% versus 6%; P = 0.002). Treatment response at 3 months was not statistically different between unknown etiology and known etiology groups (71% versus 46%; P = 0.08). Overall, initial treatment choice was effective in 35% (23 of 65). Eighty-six percent (37 of 42) who failed the initial medication had subsequent medication trials within 3 months. Etiology was strongly associated with initial treatment choice. The variation in treatment choice at our center reflects the limited evidence derived from well-designed clinical trials. Copyright © 2014 Elsevier Inc. All rights reserved.

  6. Long-Range Temporal Correlations Reflect Treatment Response in the Electroencephalogram of Patients with Infantile Spasms.

    Science.gov (United States)

    Smith, Rachel J; Sugijoto, Amanda; Rismanchi, Neggy; Hussain, Shaun A; Shrey, Daniel W; Lopour, Beth A

    2017-11-01

    Infantile spasms syndrome is an epileptic encephalopathy in which prompt diagnosis and treatment initiation are critical to therapeutic response. Diagnosis of the disease heavily depends on the identification of characteristic electroencephalographic (EEG) patterns, including hypsarrhythmia. However, visual assessment of the presence and characteristics of hypsarrhythmia is challenging because multiple variants of the pattern exist, leading to poor inter-rater reliability. We investigated whether a quantitative measurement of the control of neural synchrony in the EEGs of infantile spasms patients could be used to reliably distinguish the presence of hypsarrhythmia and indicate successful treatment outcomes. We used autocorrelation and Detrended Fluctuation Analysis (DFA) to measure the strength of long-range temporal correlations in 21 infantile spasms patients before and after treatment and 21 control subjects. The strength of long-range temporal correlations was significantly lower in patients with hypsarrhythmia than control patients, indicating decreased control of neural synchrony. There was no difference between patients without hypsarrhythmia and control patients. Further, the presence of hypsarrhythmia could be classified based on the DFA exponent and intercept with 92% accuracy using a support vector machine. Successful treatment was marked by a larger increase in the DFA exponent compared to those in which spasms persisted. These results suggest that the strength of long-range temporal correlations is a marker of pathological cortical activity that correlates with treatment response. Combined with current clinical measures, this quantitative tool has the potential to aid objective identification of hypsarrhythmia and assessment of treatment efficacy to inform clinical decision-making.

  7. Treatment of infantile spasms with very high dose prednisolone before high dose adrenocorticotropic hormone.

    Science.gov (United States)

    Hussain, Shaun A; Shinnar, Shlomo; Kwong, Grace; Lerner, Jason T; Matsumoto, Joyce H; Wu, Joyce Y; Shields, W Donald; Sankar, Raman

    2014-01-01

    This study investigated the short-term response to a standardized hormonal therapy protocol for treatment of infantile spasms. Twenty-seven children with video electroencephalography (EEG)-confirmed infantile spasms received very high dose (8 mg/kg/day, max 60 mg/day) oral prednisolone for 2 weeks. Response (absence of both hypsarrhythmia and spasms) to prednisolone was ascertained by repeat overnight video-EEG. Responders were tapered over 2 weeks and nonresponders were immediately transitioned to high dose (150 IU/m(2)/day) intramuscular adrenocorticotropic hormone (ACTH) for two additional weeks. Response was again determined by overnight video-EEG after ACTH therapy. Sixty-three percent (17/27) of patients responded completely to prednisolone. Subsequently, 40% (4/10) of prednisolone nonresponders exhibited a complete response after an additional 2-week course with ACTH. Among 27 subjects with median follow-up of 13.5 months (interquartile range [IQR] 4.8-25.9), 12% (2/17) of prednisolone responders and 50% (2/4) of ACTH responders experienced a relapse between 2 and 9 months after initial response. Very high dose prednisolone demonstrated significantly higher efficacy than previously reported for lower doses in prior studies. High dose ACTH may be superior to very high dose prednisolone, and in lieu of a definitive clinical trial, the choice between prednisolone and ACTH for initial treatment of infantile spasms remains controversial. Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.

  8. Epileptic spasms - 175 years on: Trying to teach an old dog new tricks.

    Science.gov (United States)

    Wilmshurst, Jo M; Ibekwe, Roland C; O'Callaghan, Finbar J K

    2017-01-01

    This text provides an overview of how the condition "infantile spasms" has evolved in the last 175 years. Key references are summarised to assimilate this review. Infantile spasms, first described by Dr West in 1841, has undergone extensive investigation to understand the pathogenesis, aetiologies, optimal intervention and most likely prognosis for the affected child. The terminology has recently evolved such that the preferred term for the condition is now "epileptic spasms" in recognition of the fact that cases can present outside infancy. The aetiologies are diverse and can be structural, genetic, metabolic or acquired. Increasing numbers of presumed causative genetic mutations are now being identified. The condition is an epileptic encephalopathy such that without adequate control of the clinical seizures and correction of the abnormal EEG, ongoing neurological damage occurs. In some cases neuroregression is inevitable despite intervention. First-line treatments are either hormonal therapies, adrenocortcotrophic hormone or prednisolone, or vigabatrin. In the sub-group of patients with tuberous sclerosis complex, vigabatrin is the preferred treatment. High dose prednisolone may be a more viable option in resource limited settings. Recent research has suggested that combining hormonal therapies with vigabatrin will result in more patients achieving spasm cessation. Despite extensive study, the pathogenic mechanisms remain an area of debate and in need of further exploration. The enigma, however, may be explained as the role of resting state and dysfunctional brain networks are elucidated further. Crown Copyright © 2016. Published by Elsevier Ltd. All rights reserved.

  9. Hemifacial spasm : Intraoperative electromyographic monitoring as a guide for microvascular decompression

    NARCIS (Netherlands)

    Mooij, JJA; Mustafa, MK; van Weerden, TW

    2001-01-01

    OBJECTIVE: Microvascular decompression is the logical and well-accepted treatment of choice for hemifacial spasm (HFS). In experienced hands, good to excellent results can be obtained. However, sometimes the exact site of the vascular compression is unclear. The aim of this study was to analyze

  10. Correlation study between genetic polymorphisms of melanocortin receptors and adrenocorticotropic hormone responsiveness in infantile spasms

    Directory of Open Access Journals (Sweden)

    SHI Xiu-yu

    2012-10-01

    Full Text Available Objective To explore the possible correlation between the genetic variations of the melanocortin receptors (MCRs, including MC2R, MC3R and MC4R and adrenocorticotropic hormone (ACTH responsiveness in patients with infantile spasms, and to investigate the function of single nucleotide polymorphism (SNP found in this study. Methods Direct sequencing method was used to test variations and polymorphisms in the promoter and coding regions of the MC2R, MC3R and MC4R gene. Haplotypes were structured by using SHEsis and Haploview3.32 programs to analyze the distribution frequencies of polymorphism genotypes, alleles and structured haplotypes in Chinese patients with infantile spasms and normal controls. The association between ACTH responsiveness and genetic variations was also assessed. Results Four SNPs were identified in the MC2R promoter region, one of which was new -found locus named-2T > C. Three SNPs (rs1893220, rs2186944 and -2T > C showed a significant difference between the cases and controls (P = 0.04, 0.02, 0.01. The common haplotype TCCT may give protection against the development of infantile spasms (P = 0.00. Besides, TCCT carriers were more sensitive to ACTH therapy than non-carriers (P = 0.00. The in vitro study proved that the translational efficiency of TCCT promoter in MC2R gene was four times higher than that of TCCC promoter (P = 0.00. MC2R expression assay showed a 5-fold increase in the TCCT promoter in presence of ACTH, compared with that in absence of ACTH (P = 0.00. However, responsiveness to ACTH in expression by TCCC promoter showed only 1.50-fold increase after ACTH stimulation (P > 0.05. The SNP rs11872992 in MC4R gene was related to the development of infantile spasms, as the efficiency of TC genotype in cases was lower than that of normal controls (P = 0.00. The ACTH therapy results of T-allele-carriers were better than that of non-T-allele-carriers (P = 0.01. The difference of SNP distribution frequencies in MC3R gene

  11. Clinical features of a female with WDR45 mutation complicated by infantile spasms: a case report and literature review.

    Science.gov (United States)

    Morikawa, Manami; Takano, Kyoko; Motobayashi, Mitsuo; Shiba, Naoko; Kosho, Tomoki; Nakazawa, Yozo; Inaba, Yuji

    2017-10-01

    We present a 3-year-old girl with beta-propeller protein-associated neurodegeneration (BPAN) who had a de novo heterozygous splice-site mutation of c.831-1G>C in WDR45 and developed infantile spasms; her onset age of infantile spasms was relatively late. Her infantile spasms and hypsarrhythmia disappeared promptly by adrenocorticotropic hormone therapy (CORTROSYN®Z, 0.0125mg/kg/day daily for 2weeks intramuscularly), though the administration of pyridoxal phosphate and valproic acid had poor efficacy. BPAN is known to be associated with various types of seizures, but there are few reports on infantile spasms, especially in females. To date, only 5 patients with BPAN have been reported to develop infantile spasms, and our patient is the second case in females. In this report, we showed that female patients with BPAN had milder phenotypic features than males: males developed intractable infantile spasms in early infancy, while females had treatable infantile spasms in late infancy. Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  12. Infantile spasms and 15q11.2q13.1 chromosome duplication in two successive generations.

    Science.gov (United States)

    Riikonen, Raili Sylvia; Wallden, Tiina; Kokkonen, Hannaleena

    2016-01-01

    Familial cases of West syndrome have been reported only in Japan. In that study no chromosomal analyses were made. It has been suggested that microarray analysis should be included in the diagnostic evaluation of patients with infantile spasms and developmental delay, when an evaluation for structural brain lesions and metabolic disorders reveal no abnormal findings. We report here the first case of infantile spasms and 15q11.2q13.1 chromosome duplication in two successive generations. The daughter and mother with infantile spasms, and the autistic son had the duplication. The clinical course of infantile spasms was very similar in the mother and daughter. The spasms were primarily considered to be of unknown aetiology. Chromosomal microarray analysis revealed a 6.2 Mb size 15q11.2q13.1 duplication. The duplication belongs to the 15q11q13 duplication syndrome (OMIM 608636) which when maternally derived is characterised by neuro-behavioural disorders like autism, hypotonia, cognitive deficit, language delay and epilepsy. The proportion of patients with unknown aetiology for infantile spasms will decrease when more careful chromosomal studies are made. Our report expands the phenotype of chromosome 15q duplication syndrome and is the first report of this abnormality in two successive generations of infantile spasms. Copyright © 2015 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  13. Prenatal corticosteroids modify glutamatergic and GABAergic synapse genomic fabric: insights from a novel animal model of infantile spasms.

    Science.gov (United States)

    Iacobas, D A; Iacobas, S; Chachua, T; Goletiani, C; Sidyelyeva, G; Velíšková, J; Velíšek, L

    2013-11-01

    Prenatal exposure to corticosteroids has long-term postnatal somatic and neurodevelopmental consequences. Animal studies indicate that corticosteroid exposure-associated alterations in the nervous system include hypothalamic function. Infants with infantile spasms, a devastating epileptic syndrome of infancy with characteristic spastic seizures, chaotic irregular waves on interictal electroencephalogram (hypsarhythmia) and mental deterioration, have decreased concentrations of adrenocorticotrophic hormone (ACTH) and cortisol in cerebrospinal fluid, strongly suggesting hypothalamic dysfunction. We have exploited this feature to develop a model of human infantile spasms by using repeated prenatal exposure to betamethasone and a postnatal trigger of developmentally relevant spasms with NMDA. The spasms triggered in prenatally primed rats are more severe compared to prenatally saline-injected ones and respond to ACTH, a treatment of choice for infantile spasms in humans. Using autoradiography and immunohistochemistry, we have identified a link between the spasms in our model and the hypothalamus, especially the arcuate nucleus. Transcriptomic analysis of the arcuate nucleus after prenatal priming with betamethasone but before trigger of spasms indicates that prenatal betamethasone exposure down-regulates genes encoding several important proteins participating in glutamatergic and GABAergic transmission. Interestingly, there were significant sex-specific alterations after prenatal betamethasone in synapse-related gene expression but no such sex differences were found in prenatally saline-injected controls. A pairwise relevance analysis revealed that, although the synapse gene expression in controls was independent of sex, these genes form topologically distinct gene fabrics in males and females and these fabrics are altered by betamethasone in a sex-specific manner. These findings may explain the sex differences with respect to both normal behaviour and the occurrence

  14. Limited efficacy of the ketogenic diet in the treatment of highly refractory epileptic spasms.

    Science.gov (United States)

    Hussain, Shaun A; Shin, Ji Hyun; Shih, Evan J; Murata, Kristina K; Sewak, Sarika; Kezele, Michele E; Sankar, Raman; Matsumoto, Joyce H

    2016-02-01

    Numerous studies have suggested that the ketogenic diet is effective in the treatment of epileptic spasms, even in refractory cases. However, there has been very limited demonstration of prompt and complete (video-EEG confirmed) response. We set out to describe our center's experience with the ketogenic diet in the treatment of children with highly refractory epileptic spasms, with rigorous seizure outcome assessment. Children treated with the ketogenic diet for epileptic spasms between April, 2010 and June, 2014 were retrospectively identified. Seizure burden was tabulated at baseline and after 1, 3, 6, and 12-months of ketogenic diet exposure. Adverse events were similarly ascertained. We identified a cohort of 22 consecutive patients who received ketogenic diet therapy, with median age of onset of epileptic spasms of 5.2 (IQR 2.0-9.0) months, with diet initiation beginning a median of 26.4 (12.5-38.7) months after onset, and following a median of 7 (IQR 5-7) treatment failures. Only 2 patients exhibited a complete response during ketogenic diet exposure, and response was more reasonably attributed to alternative therapies in both cases. A modest early reduction in seizure frequency was not sustained beyond 1 month of diet exposure. The diet was well tolerated, and continued in 6 patients with subjective and/or partial response. In contrast to prior studies reporting substantial efficacy of the ketogenic diet, our findings suggest limited efficacy, albeit in a highly refractory cohort. Prospective studies in both refractory and new-onset populations, with both video-EEG confirmation of response and rigorous cognitive outcome assessment, would be of great value to more clearly define the utility of the ketogenic diet in the treatment of epileptic spasms. Copyright © 2016 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  15. SCN2A mutation is associated with infantile spasms and bitemporal glucose hypometabolism.

    Science.gov (United States)

    Sundaram, Senthil K; Chugani, Harry T; Tiwari, Vijay N; Huq, A H M M

    2013-07-01

    Genetic mutations play a crucial role in the etiology of cryptogenic infantile spasms, but the cause is still unknown in a significant proportion of patients. Whole exome sequencing technology shows great promise in identifying genetic causes of infantile spasms. In this study whole exome sequencing was performed with 2-deoxy-2-((18)F)fluoro-d-glucose positron emission tomography scan of an infant boy with infantile spasms. Exome sequencing was also performed in the parents to identify any de novo mutations. The positron emission tomography scan showed a pattern of bilateral symmetric temporal lobe glucose hypometabolism. A total of 8171 nonsynonymous variants were identified in the child. Despite the large number of nonsynonymous variants, there was only a single de novo missense mutation in SCN2A in the child (NCBI hg19 assembly, position: Chr2:166234116, K1422E). Subsequent Sanger sequencing confirmed the de novo status of this variant. This mutation has never been reported in 6500 individuals of the exome variant server database. Similarly, this variant is not reported in the Online Mendelian Inheritance in Man Database or the Human Gene Mutation Database. It has previously been shown that SCN2A mutations are associated with hippocampal hyperexcitability. Therefore, this study indicates that infantile spasms and bitemporal hypometabolism in this patient might have been caused by hippocampal hyperexcitability due to SCN2A mutation. The simultaneous presence of an SCN2A mutation and bitemporal hypometabolism in this patient with infantile spasms suggests a plausible hippocampal origin. However, additional mechanistic and clinical studies are required to validate this link. Copyright © 2013 Elsevier Inc. All rights reserved.

  16. Scalp EEG Ictal gamma and beta activity during infantile spasms: Evidence of focality.

    Science.gov (United States)

    Nariai, Hiroki; Beal, Jules; Galanopoulou, Aristea S; Mowrey, Wenzhu B; Bickel, Stephan; Sogawa, Yoshimi; Jehle, Rana; Shinnar, Shlomo; Moshé, Solomon L

    2017-05-01

    We investigated temporal and spatial characteristics of ictal gamma and beta activity on scalp EEG during spasms in patients with West syndrome (WS) to evaluate potential focal cortical onset. A total of 1,033 spasms from 34 patients with WS of various etiologies were analyzed on video-electroencephalography (EEG) using time-frequency analysis. Ictal gamma (35-90 Hz) and beta (15-30 Hz) activities were correlated with visual symmetry of spasms, objective EMG (electromyography) analysis, and etiology of WS. Prior to the ictal motor manifestation, focal ictal gamma activity emerged from one hemisphere (71%, 24/34) or from midline (26%, 9/34), and was rarely simultaneously bilateral (3%, 1/34). Focal ictal beta activity emerged from either one hemisphere (68%, 23/34) or from midline (32%, 11/34). Onsets of focal ictal gamma and beta activity were most commonly observed around the parietal areas. Focal ictal gamma activity propagated faster than ictal beta activity to adjacent electrodes (median: 65 vs. 170 msec, p spasms showed asymmetry in peak amplitude and interhemispheric onset latency difference in both ictal gamma and beta activity. Spasms may be a seizure with focal electrographic onset regardless of visual symmetry. Asymmetric involvement of ictal gamma activity to the centroparietal areas may determine the motor manifestations in WS. Scalp EEG ictal gamma and beta activity may be useful to demonstrate localized seizure onset in infants with WS. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

  17. Prognostic factors of infantile spasms: role of treatment options including a ketogenic diet.

    Science.gov (United States)

    Lee, Jeehun; Lee, Jun Hwa; Yu, Hee Jun; Lee, Munhyang

    2013-09-01

    The aim of this study was to provide additional evidences on prognostic factors for infantile spasms and the possible role of a ketogenic diet. A retrospective analysis was performed for patients with infantile spasms who had been followed up for more than 6months between January 2000 and July 2012 at Samsung Medical Center (Seoul, Republic of Korea). We analyzed the association between possible prognostic factors and seizure/developmental outcomes. Sixty-nine patients were included in this study and their mean follow-up duration was 52.5 (9-147) months. In the patients who had been followed up for more than 2years, 53.6% (n=30/57) remained seizure-free at the last visit. Sixty patients (86.9%) showed developmental delay at last follow-up. Forty-two patients (60.9%) became spasm-free with one or two antiepileptic drugs, one patient with epilepsy surgery for a tumor, and seven patients with a ketogenic diet after the failure of two or more antiepileptic drugs. The etiology and age of seizure onset were the significant prognostic factors. In this study, about 60% of the patients became spasm-free with vigabatrin and topiramate. Ketogenic diet increased the rate by 10% in the remaining antiepileptic drug resistant patients. However, 86.9% of the patients showed developmental delay, mostly a severe degree. Early diagnosis and prompt application of treatment options such as antiepileptic drugs, a ketogenic diet or epilepsy surgery can improve outcomes in patients with infantile spasms. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  18. Prevention

    DEFF Research Database (Denmark)

    Halken, S; Høst, A

    2001-01-01

    , breastfeeding should be encouraged for 4-6 months. In high-risk infants a documented extensively hydrolysed formula is recommended if exclusive breastfeeding is not possible for the first 4 months of life. There is no evidence for preventive dietary intervention neither during pregnancy nor lactation...... populations. These theories remain to be documented in proper, controlled and prospective studies. Breastfeeding and the late introduction of solid foods (>4 months) is associated with a reduced risk of food allergy, atopic dermatitis, and recurrent wheezing and asthma in early childhood. In all infants....... Preventive dietary restrictions after the age of 4-6 months are not scientifically documented....

  19. Prevention: Exercise

    Medline Plus

    Full Text Available ... Spasms Pinched Nerve Discitis Degenerative Conditions Bulge vs Herniation Cervical Stenosis, Myelopathy, and Radiculopathy Herniated Lumbar Disc Herniated Cervical Disc Lumbar Degenerative Disc Disease Lumbar ...

  20. The Clinical Observation on 10 cases of patients with Hemifacial Spasm Treated by Soyeom Pharmacupuncture at G20(Pungji

    Directory of Open Access Journals (Sweden)

    Jin Heo

    2010-06-01

    Full Text Available Objectives : The main purpose of this research is to evaluate the effect of treatment with Soyeom Pharmacupuncture at G20(Punji for ten patients with hemifacial spasm. Methods : We have treated them with acupuncture treatment and Soyeom Pharmacupuncture at G20(Pungji, and evaluated the effect by Scott`s scale. Results : After treatment, the grades of spasm intensity classified by Scott`s description were improved in 9 cases. Conclusion : This data suggested that Soyeom Pharmacupuncture at G20(Pungji for hemifacial spasm was effective and will be attempted to the patients with it.

  1. Microvascular decompression to treat hemifacial spasm: long-term results for a consecutive series of 143 patients.

    Science.gov (United States)

    Samii, Madjid; Günther, Thomas; Iaconetta, Giorgio; Muehling, Michael; Vorkapic, Peter; Samii, Amir

    2002-04-01

    The concept of neurovascular decompression for the treatment of hemifacial spasm is now widely accepted. In this study, we report our long-term results for 145 cases treated with this procedure. The results of 145 microvascular decompressions to treat hemifacial spasm (performed between 1980 and 1998) among 143 patients (62.2% female patients and 37.8% male patients; mean age, 54.5 yr) are presented. The onset of symptoms was typical in 95.9% of cases and atypical in 4.1%. Platysma muscle involvement was observed for 24.5% of patients, with a higher incidence among female patients (74.3%). Patients were monitored with annual questionnaires. Twenty-six patients were lost to follow-up monitoring, and 117 are still undergoing follow-up monitoring, with an average period of 9.6 years (range, 1-17.6 yr). At discharge, 69 patients (59%) were spasm-free and 48 patients (41%) experienced further spasm. At 6 months, the number of spasm-free patients had increased to 108 (92.3%), whereas only 9 patients (7.7%) complained of hemifacial spasm; 44 patients were spasm-free at an average time of 15 weeks. In follow-up examinations (average period, 9.4 yr), 106 patients were spasm-free. Seven patients experienced only temporary relief, with recurrence after 4.5 years. Two patients were spasm-free after 4 or 6 weeks, and the recurrence of spasm was observed 1 year later. Two patients were never completely spasm-free. Among the patients who did not undergo previous surgery elsewhere, only two experienced recurrence. Deafness was the main postoperative complication (8.3%); most of those cases (66%) occurred before the routine use of intraoperative evoked potential monitoring. Analysis of our series demonstrates that this surgical procedure involves very low risk, is well tolerated by elderly patients, is associated with very low recurrence rates, and is a definitive treatment for more than 90% of cases.

  2. Predictive value of EEG findings at control of epileptic spasms for seizure relapse in patients with West syndrome.

    Science.gov (United States)

    Yamada, Keitaro; Toribe, Yasuhisa; Kimizu, Tomokazu; Kimura, Sadami; Ikeda, Tae; Mogami, Yukiko; Yanagihara, Keiko; Mano, Toshiyuki; Suzuki, Yasuhiro

    2014-10-01

    To evaluate the prognostic importance of electroencephalography (EEG) findings at cessation of epileptic spasms for seizure outcome. We reviewed 71 children with West syndrome (cryptogenic 14) who had obtained control of epileptic spasms with initial treatment (adrenocorticotropic hormone (ACTH) 37, high-dose vitamin B6 2, and antiepileptic drugs 32). According to the EEG findings at control of epileptic spasms, the subjects were divided into three groups: normal group (no epileptic activity, n=12), abnormal group (residual epileptic activity without hypsarrhythmia, n=53), and hypsarrhythmic group (persisting hypsarrhythmia, n=6). Overall, 47 (66%) of the 71 patients (cryptogenic 4) had experienced relapses of seizures (epileptic spasms 23 and focal seizure 24) after initial control of epileptic spasms. Within symptomatic cases, seizure relapse rate varied widely from 0% (Down syndrome) to 100% (tuberous sclerosis), depending on underlying causes. Seizure relapse depended on the EEG findings at control of epileptic spasms. The normal group had a significantly lower seizure relapse rate (17%) in comparison with the abnormal group (75%), the hypsarrhythmic group (83%), and the epileptiform (abnormal plus hypsarrhythmic, 76%) group. No significant difference in seizure relapse rate was observed between non-hypsarrhythmic (normal plus abnormal, 65%) and hypsarrhythmic groups. At the last follow-up, normal group children also showed a favorable seizure prognosis (seizure control 100%). A favorable seizure prognosis is associated with the disappearance of epileptic activity, but not the resolution of hypsarrhythmic pattern on EEG at control of epileptic spasms. We suggest that effective treatment for West syndrome should produce both cessation of epileptic spasms and disappearance of epileptic activity on EEG. Copyright © 2014 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  3. Vigabatrin therapy for infantile spasms: review of major trials in Europe, Canada, and the United States; and recommendations for dosing.

    Science.gov (United States)

    Carmant, L

    2011-01-01

    Infantile spasms (IS) are a unique and severe form of epilepsy associated with poor neurologic and developmental outcomes. The refractory spasms and abnormal electroencephalogram (EEG) patterns associated with the condition are believed to have a progressively detrimental impact. Therefore, rapid and complete control of spasms is the primary goal of treatment. Well-controlled clinical trials in Europe, Canada, and the United States have demonstrated that vigabatrin is efficacious and generally well-tolerated as monotherapy for IS. Several key studies, including pivotal trials that led to United States approval of vigabatrin in 2009, as well as comparative trials of vigabatrin and hormonal treatment, are the focus of this review. All studies assessed spasm cessation - usually as the primary endpoint - and adverse events. Vigabatrin dosages generally ranging from 100 to 150 mg/kg/day demonstrated efficacy to decrease or eradicate spasms and eliminate hypsarrhythmic EEG in patients with newly diagnosed IS. Several studies demonstrated long-term sustainability of spasm freedom with no negative impact on developmental outcomes. Vigabatrin was generally well-tolerated with few severe adverse events. Visual field defects cannot be adequately assessed in infants and young children, so this potential adverse effect was not evaluated in children with spasms. Notably, the time to response with vigabatrin was very rapid, generally occurring within 2 weeks of initial treatment. This allows for early treatment modification as needed. For infants who respond well to vigabatrin, treatment duration up to 6 months appears to be appropriate for realizing spasm freedom while limiting potential risks of adverse events and recurrences. © 2011 John Wiley & Sons A/S.

  4. Radial lean direct injection burner

    Science.gov (United States)

    Khan, Abdul Rafey; Kraemer, Gilbert Otto; Stevenson, Christian Xavier

    2012-09-04

    A burner for use in a gas turbine engine includes a burner tube having an inlet end and an outlet end; a plurality of air passages extending axially in the burner tube configured to convey air flows from the inlet end to the outlet end; a plurality of fuel passages extending axially along the burner tube and spaced around the plurality of air passage configured to convey fuel from the inlet end to the outlet end; and a radial air swirler provided at the outlet end configured to direct the air flows radially toward the outlet end and impart swirl to the air flows. The radial air swirler includes a plurality of vanes to direct and swirl the air flows and an end plate. The end plate includes a plurality of fuel injection holes to inject the fuel radially into the swirling air flows. A method of mixing air and fuel in a burner of a gas turbine is also provided. The burner includes a burner tube including an inlet end, an outlet end, a plurality of axial air passages, and a plurality of axial fuel passages. The method includes introducing an air flow into the air passages at the inlet end; introducing a fuel into fuel passages; swirling the air flow at the outlet end; and radially injecting the fuel into the swirling air flow.

  5. Comparison of the serum cytokine levels before and after adrenocorticotropic hormone (ACTH) therapy in patients with infantile spasm.

    Science.gov (United States)

    Türe, Esra; Kamaşak, Tülay; Cora, Merve; Şahin, Sevim; Arslan, Elif Acar; Kaklıkaya, Neşe; Cansu, Ali

    2016-10-01

    Infantile spasm is an age-dependent epileptic syndrome seen in infancy or early childhood. Although studies have investigated the epilepsy-cytokine relationship, there has been insufficient research into the relation between cytokines and infantile spasm. The purpose of this study was to examine the role of cytokines in the pathogenesis of infantile spasm by investigating cytokine levels before and 1month after adrenocorticotropic hormone (ACTH) therapy in patients diagnosed with the condition. Twenty patients aged between 1month and 2years and diagnosed with infantile spasm at the Karadeniz Technical University Medical Faculty Department of Child Health and Diseases Pediatric Neurology Clinic, Turkey, and 20 healthy children were included in the study. Patients received 11 doses of ACTH on 2days a week. Levels of TNF-alpha and IL-2, the main cytokines involved in inflammation and recently associated with infantile spasm, and of IL-1beta, IL-6 and IL-17A, associated with epileptic seizures, and serum levels of the IL-17A activator IL-23 were investigated in all patients at the start of treatment and 1month after completion of treatment. No statistically significant difference was observed between pre- and post-treatment patient group and control group IL-1beta, IL-2, IL-23 or TNF-alpha levels. Pre-treatment IL-6 and IL-17A levels were significantly higher in the untreated patient group compared to the healthy control group (pinfantile spasm. Copyright © 2016. Published by Elsevier Ltd.

  6. A Case of Painful Hemimasticatory Spasm with Masseter Muscle Hypertrophy Responsive to Botulinum Toxin

    Directory of Open Access Journals (Sweden)

    Jin-Hyuck Kim

    2009-10-01

    Full Text Available Hemimasticatory spasm (HMS is a rare disorder of the trigeminal nerve characterized by paroxysmal involuntary contractions of the unilateral jaw-closing muscles. HMS has been frequently described in association with facial hemiatrophy or localized scleroderma. A 42-year-old female presented with involuntary paroxysmal spasms of the left face, of 6 months duration. Her lower face on the left was markedly hypertrophied without skin lesions. An electrophysiological study indicated that the masseter reflexes and masseteric silent period were attenuated on the affected side. Surface electromyography demonstrated irregular bursts of motor unit potentials at high frequencies up to 200 Hz. Magnetic resonance imaging of the head showed marked hypertrophy of the left masseter muscle. Biopsy of the hypertrophied masseter muscle was normal. Repeated local injections of botulinum toxin noticeably reduced the size of the hypertrophied muscle as well as improved the patient’s symptoms.

  7. Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms.

    Science.gov (United States)

    Ferrari, Taíssa P F; Hamad, Ana P A; Caboclo, Luís Otávio S F; Centeno, Ricardo S; Zaninotto, Ana Luiza; Scattolin, Monica; Carrete Junior, Henrique; Lancellotti, Carmem L P; Yacubian, Elza Márcia T

    2011-09-01

    A five-and-a-half-year-old girl started experiencing progressive left hemiparesis at age two and a half years. At age five years and four months she started presenting clusters of asymmetric periodic epileptic spasms with no hypsarrhythmia. The ictal EEG showed periodic, constant and stereotyped complexes. Serial brain imaging revealed progressive atrophy of the right hemisphere with increased T2 signal on MRI. She underwent a right hemispherotomy, and histological examination showed signs of inflammation and features of focal cortical dysplasia (FCD). She has been seizure-free for 16 months. This case is unique in the following aspects: the presence of typical Rasmussen encephalitis features of progressive unilateral brain involvement without seizures, a delay of almost three years prior to seizure onset; an atypical seizure type presentation with periodic epileptic spasms and the presence of FCD associated with inflammatory changes. [Published with video sequences].

  8. Clinical profile of vigabatrin as monotherapy for treatment of infantile spasms

    Science.gov (United States)

    Lerner, Jason T; Salamon, Noriko; Sankar, Raman

    2010-01-01

    Vigabatrin, the first therapeutic agent to be approved by the Food and Drug Administration for the treatment of infantile spasms, as well as for adjunctive use in the treatment of refractory complex partial epilepsy, represents an important advance for patients with difficult-to-manage epilepsy. This review summarizes the complex history, chemistry, and pharmacology, as well as the clinical data leading to the approval of vigabatrin for infantile spasms in the US. The long path to its approval reflects the visual system and white matter toxicity concerns with this agent. This review provides a brief description of these concerns, and the regulatory safety monitoring and mitigation systems that have been put in place to enhance benefit over risk. PMID:21127692

  9. Self-consistent radial sheath

    International Nuclear Information System (INIS)

    Hazeltine, R.D.

    1988-12-01

    The boundary layer arising in the radial vicinity of a tokamak limiter is examined, with special reference to the TEXT tokamak. It is shown that sheath structure depends upon the self-consistent effects of ion guiding-center orbit modification, as well as the radial variation of E /times/ B-induced toroidal rotation. Reasonable agreement with experiment is obtained from an idealized model which, however simplified, preserves such self-consistent effects. It is argued that the radial sheath, which occurs whenever confining magnetic field-lines lie in the plasma boundary surface, is an object of some intrinsic interest. It differs from the more familiar axial sheath because magnetized charges respond very differently to parallel and perpendicular electric fields. 11 refs., 1 fig

  10. Brief atonia associated with electroencephalographic paroxysm in an infant with infantile spasms.

    Science.gov (United States)

    Hakamada, S; Watanabe, K; Hara, K; Miyazaki, S

    1981-06-01

    The loss of muscular tone or muscular inhibition associated with sharp waves observed during abnormal tonic posture in a patient with infantile spasms of early onset is described. The latency between the beginning of a sharp wave and the muscular inhibition varied from 30 to 700 msec, and the duration of the inhibition ranged from 100 to 400 msec. These brief muscular inhibitions may sometimes precede tonic seizures, and these phenomena might be noticeable only in the state of steady tonic muscular contraction.

  11. Epileptic spasms in paediatric post-traumatic epilepsy at a tertiary referral centre.

    Science.gov (United States)

    Park, Jun T; Chugani, Harry T

    2017-03-01

    To recognize epileptic spasms (ES) as a seizure type after traumatic brain injury (TBI), accidental or non-accidental, in infants and children. In the process, we aim to gain some insight into the mechanisms of epileptogenesis in ES. A retrospective electronic chart review was performed at the Children's Hospital of Michigan from 2002 to 2012. Electronic charts of 321 patients were reviewed for evidence of post-traumatic epilepsy. Various clinical variables were collected including age at TBI, mechanism of trauma, severity of brain injury, electroencephalography/neuroimaging data, and seizure semiology. Six (12.8%) of the 47 patients diagnosed with post-traumatic epilepsy (PTE) had ES. Epileptic spasms occurred between two months to two years after TBI. All patients with ES had multiple irritative zones, manifesting as multifocal epileptiform discharges, unilateral or bilateral. Cognitive delay and epileptic encephalopathy were seen in all six patients, five of whom were free of spasms after treatment with vigabatrin or adrenocorticotropic hormone. The risk of PTE is 47/321(14.6%) and the specific risk of ES after TBI is 6/321 (1.8%). The risk of ES appears to be high if the age at which severe TBI occurred was during infancy. Non-accidental head trauma is a risk factor of epileptic spasms. While posttraumatic epilepsy (not ES) may start 10 years after the head injury, ES starts within two years, according to our small cohort. The pathophysiology of ES is unknown, however, our data support a combination of previously proposed models in which the primary dysfunction is a focal or diffuse cortical abnormality, coupled with its abnormal interaction with the subcortical structures and brainstem at a critical maturation stage.

  12. Acanthamoeba infection after radial keratotomy.

    Science.gov (United States)

    Friedman, R F; Wolf, T C; Chodosh, J

    1997-03-01

    To describe a case of Acanthamoeba infection of the cornea after radial and astigmatic keratotomy. A 29-year-old man developed ulcerative keratitis in the right eye 6 weeks after uncomplicated radial and astigmatic keratotomy. Three sets of corneal cultures for bacteria and fungi were negative. Culture on non-nutrient agar grew Acanthamoeba organisms. Clinical improvement occurred after topical antiamebic therapy was instituted. Incisional keratotomy may predispose the cornea to delayed-onset infectious keratitis. Acanthamoeba should be considered as a possible cause of infection and should be cultured for in refractory cases.

  13. Detonation in supersonic radial outflow

    KAUST Repository

    Kasimov, Aslan R.

    2014-11-07

    We report on the structure and dynamics of gaseous detonation stabilized in a supersonic flow emanating radially from a central source. The steady-state solutions are computed and their range of existence is investigated. Two-dimensional simulations are carried out in order to explore the stability of the steady-state solutions. It is found that both collapsing and expanding two-dimensional cellular detonations exist. The latter can be stabilized by putting several rigid obstacles in the flow downstream of the steady-state sonic locus. The problem of initiation of standing detonation stabilized in the radial flow is also investigated numerically. © 2014 Cambridge University Press.

  14. Worsening of coronary spasm during the perioperative period: A case report

    Science.gov (United States)

    Teragawa, Hiroki; Nishioka, Kenji; Fujii, Yuichi; Idei, Naomi; Hata, Takaki; Kurushima, Shuji; Shokawa, Tomoki; Kihara, Yasuki

    2014-01-01

    We present the case of a 65-year-old male with vasospastic angina (VSA) whose condition worsened during the perioperative period. He had been diagnosed with VSA 10 years prior. He was treated with two types of vasodilators and had not experienced any chest symptoms for 5 years. At this juncture, he underwent surgery for relapsed maxillary sublingual carcinoma. He had taken two vasodilators one day prior to surgery. Intravenous infusion of nitroglycerin (NTG) was initiated immediately before the surgery and continued the following day. Instead of stopping NTG, a dermal isosorbide dinitrate tape was applied on post-operative day 1. Two days later, a complete atrioventricular block with pulseless electrical activity appeared. After cardiopulmonary resuscitation, emergent coronary angiography showed severe coronary spasm in both the left and right coronary arteries. Intracoronary infusion of nitroglycerin and epinephrine with percutaneous cardiopulmonary support relieved the coronary spasm. During the perioperative period, several factors can trigger coronary vasospasm, including the discontinuation of vasodilators. Thus, surgeons, anesthetists, and cardiologists should watch for coronary vasospasm during this period and for worsening coronary spasm when discontinuing vasodilators in patients at risk for VSA. PMID:25068030

  15. Clinical profile of vigabatrin as monotherapy for treatment of infantile spasms

    Directory of Open Access Journals (Sweden)

    Jason T Lerner

    2010-11-01

    Full Text Available Jason T Lerner1, Noriko Salamon2, Raman Sankar1,31Departments of Pediatrics, 2Radiological Sciences, 3Neurology, David Geffen School of Medicine, University of California Los Angeles and Mattel Children’s Hospital at UCLA, Los Angeles, CA, USAAbstract: Vigabatrin, the first therapeutic agent to be approved by the Food and Drug Administration for the treatment of infantile spasms, as well as for adjunctive use in the treatment of refractory complex partial epilepsy, represents an important advance for patients with difficult-to-manage epilepsy. This review summarizes the complex history, chemistry, and pharmacology, as well as the clinical data leading to the approval of vigabatrin for infantile spasms in the US. The long path to its approval reflects the visual system and white matter toxicity concerns with this agent. This review provides a brief description of these concerns, and the regulatory safety monitoring and mitigation systems that have been put in place to enhance benefit over risk.Keywords: vigabatrin, infantile spasms, monotherapy

  16. Development of a mouse model of infantile spasms induced by N-methyl-D-aspartate.

    Science.gov (United States)

    Shi, Xiu-Yu; Yang, Xiao-Fan; Tomonoh, Yuko; Hu, Lin-Yan; Ju, Jun; Hirose, Shinichi; Zou, Li-Ping

    2015-12-01

    Using N-methyl-D-aspartate (NMDA) injection, we attempt to develop a mouse model for infantile spasms (IS). Experiments were performed in postnatal 11- to 13-day-old C57 and Balbc mice. In the pilot experiment, mice were injected with different doses of NMDA (7, 15, and 30 mg/kg) to determine the optimal age and convulsant doses of NMDA. In further experiment optimal age mice were divided into five groups: group A, control group that received intraperitoneal injection of physiological saline; group B, convulsion group that was given intraperitoneal NMDA; group C, pretreatment group that received adrenocorticotropin (ACTH) injection (100 IU/kg) 30 min before NMDA administration; group D, electroencephalogram (EEG) group that received EEG recording, group E, performance group that received motor and learning test at different time point after NMDA administration. The behaviors of each group were observed continuously for 3h, the latency and the total numbers of spasms were recorded. Pilot experiments showed that a 15 mg/kg dose of NMDA could induce typical spasm-like seizures in P13 C57 mice, NMDA administration caused anxiety and deficits in motor and cognitive functions at early time and that large doses of ACTH reduced the number of seizures and rating scale (Pspasm-like seizures, cognitive impairment and response to ACTH, which fulfills the criteria of an IS model. Copyright © 2015 Elsevier B.V. All rights reserved.

  17. Three patients manifesting early infantile epileptic spasms associated with 2q24.3 microduplications.

    Science.gov (United States)

    Yoshitomi, Shinsaku; Takahashi, Yukitoshi; Ishizuka, Mamiko; Yamaguchi, Tokito; Watanabe, Akito; Nasu, Hirosato; Ueda, Yuki; Ohtani, Hideyuki; Ikeda, Hiroko; Imai, Katsumi; Shigematsu, Hideo; Inoue, Yushi; Tanahashi, Yoshihiro; Aiba, Kaori; Ohta, Hodaka; Shimada, Shino; Yamamoto, Toshiyuki

    2015-10-01

    Recent development of genetic analyses enabled us to reveal underlying genetic causes of the patients with epileptic encephalopathy in infancy. Mutations of voltage-gated sodium channel type I alpha subunit gene (SCN1A) are to be causally related with several phenotypes of epilepsy, generalized epilepsy with febrile seizure plus (GEFS+), Dravet syndrome, and other infantile epileptic encephalopathies. In addition to SCN1A, contiguous genes such as SCN2A and SCN3A in 2q24.3 are also reported to have contribution to epileptic seizures. Therefore, gene abnormality involving this region is reasonable to contribute to epilepsy manifestation. We encountered three patients with 2q24.3 microduplication diagnosed by Array comparative genomic hybridization array (aCGH). They developed partial seizures and epileptic spasms in their early infantile periods and showed remarkable developmental delay, although their seizures disappeared from 11 to 14 months of age. One of three patients had 2q24.3 microduplication which excludes SCN1A. Therefore, characteristics of epilepsy with 2q24.3 microduplication do not necessarily need duplication of SCN1A. This study suggested that 2q24.3 microduplication is one of the causes for early infantile epileptic spasms. Epileptic spasms associated with 2q24.3 microduplications may have better seizure outcome comparing with other etiologies. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  18. Two infants with infantile spasms associated with vitamin B12 deficiency.

    Science.gov (United States)

    Malbora, Baris; Yuksel, Deniz; Aksoy, Ayse; Ozkan, Mehpare

    2014-07-01

    In developing countries, nutritional vitamin B12 deficiency in infants because of maternal deficiency often causes hematological and neurological disorders. However, epilepsy is a rare manifestation of vitamin B12 deficiency. The biological basis for the observed neurological symptoms of infantile vitamin B12 deficiency remains uncertain. There are only a few reports in the English literature regarding the relationship between infantile spasms and vitamin B12 deficiency. We report two unrelated infants having infantile spasms associated with vitamin B12 deficiency related to maternal nutritional deficiency. During the first month of adrenocorticotropic hormone (ACTH), phenobarbital, and vitamin B12 treatments, both infants' abnormalities resolved. After 3 months, electroencephaography was completely normal. ACTH and phenobarbital treatments were ended. The children are disease-free 9 months after the treatment. We suggest that vitamin B12 deficiency should be considered in the differential diagnosis of infantile spasms as a treatable cause, especially with a history of maternal nutritional deficiency. Copyright © 2014 Elsevier Inc. All rights reserved.

  19. Upregulations of CRH and CRHR1 in the Epileptogenic Tissues of Patients with Intractable Infantile Spasms.

    Science.gov (United States)

    Yang, Xiao-Lin; Chen, Bing; Zhang, Xiao-Qing; Chen, Xin; Yang, Mei-Hua; Zhang, Wei; Chen, Huan-Ran; Zang, Zhen-Le; Li, Wei; Yang, Hui; Liu, Shi-Yong

    2017-01-01

    Infantile spasms (IS) are an age-specific epileptic syndrome with specific clinical symptom and electroencephalogram (EEG) features, lacking treatment options, and a poor prognosis. Excessive endogenous corticotropin-releasing hormone (CRH) in infant brain might result in IS. However, the data from human IS are limited. In our study, we investigated the expressions of CRH and its receptor type 1 (CRHR1) in surgical tissues from patients with IS and autopsy controls. Specimens surgically removed from 17 patients with IS, and six autopsy controls were included in the study. Real-time PCR, Western blotting, and immunostaining were used to detect the expressions of mRNA, protein expression, and distribution. The correlation between variates was analyzed by Spearman rank correlation. The expressions of CRH and CRHR1 were significantly upregulated in the epileptogenic tissues of IS patients compared with the control group. CRH was distributed mainly in neurons, while CRHR1 was distributed in neurons, astrocytes, and microglia. The expression levels of CRH and CRHR1 were positively correlated with the frequency of epileptic spasms. Moreover, the expression of protein kinase C (PKC), which was an important downstream factor of CRHR1, was significantly upregulated in the epileptogenic tissues of patients with IS and was positively correlated with the CRHR1 expression levels and the frequency of epileptic spasms. These results suggest that the CRH signal transduction pathway might participate in the epileptogenesis of IS, supporting the hypothesis that CRH is related to the pathogenesis of IS. © 2016 John Wiley & Sons Ltd.

  20. Carpal spasm in a girl as initial presentation of celiac disease: a case report.

    Science.gov (United States)

    Ramosaj-Morina, Atifete; Keka-Sylaj, A; Hasbahta, V; Baloku-Zejnullahu, A; Azemi, M; Zunec, R

    2017-09-04

    Celiac disease is an immune-mediated disorder elicited by ingestion of gluten in genetically susceptible persons. This disorder is characterized by specific histological changes of the small intestine mucosa resulting in malabsorption. This case was written up as it was an unusual and dramatic presentation of celiac disease. We report the case of a 3-year-old Albanian girl who presented at our clinic with carpal spasms and hand paresthesia. A physical examination at admission revealed a relatively good general condition and body weight of 10.5 kg (10 percentile). Carpal spasms and paresthesias of her extremities were present. Neuromuscular irritability was demonstrated by positive Chvostek and Trousseau signs. Blood tests showed severe hypocalcemia with a total serum calcium of 1.2 mmol/L (normal range 2.12 to 2.55 mmol/L), ionized calcium of 0.87 (normal range 1.11 to 1.30 mmol/L), and 24-hour urine calcium excretion of 9.16 mmol (normal range female celiac disease was performed: antigliadin immunoglobulin A, anti-tissue transglutaminase, and anti-endomysial immunoglobulin A antibodies were positive. A duodenal biopsy revealed lymphocyte infiltration, crypt hyperplasia, and villous atrophy compatible with celiac disease grade IIIb according to the Marsh classification. Following the diagnosis of celiac disease, human leukocyte antigen typing was performed, giving a definite diagnosis of celiac disease. She was started on a gluten-free diet. Due to failure to follow a gluten-free diet, episodes of carpal spasms appeared again. Unfortunately, at the age of 7 years she presents with delayed psychophysical development. Although hypocalcemia is a common finding in celiac disease, hypocalcemic carpal spasm is a rare initial manifestation of the disease. Therefore, the possibility of celiac disease should be considered in patients with repeated carpal spasms that seem unduly difficult to treat. This should be evaluated even in the absence of gastrointestinal

  1. Perceived radial translation during centrifugation.

    Science.gov (United States)

    Bos, Jelte E; Correia Grácio, Bruno J

    2015-01-01

    Linear acceleration generally gives rise to translation perception. Centripetal acceleration during centrifugation, however, has never been reported giving rise to a radial, inward translation perception. To study whether centrifugation can induce a radial translation perception in the absence of visual cues. To that end, we exposed 12 subjects to a centripetal acceleration with eyes closed. To avoid confounding with angular motion perception, subjects were fist rotated on-axis, and were shifted out fast and slow only after rotation sensation had vanished. They were asked for translation direction and velocity right after the shift-out, as well as after about 60 seconds of constant centrifugation. Independent of fast or slow shift-out, the vast statistically significant majority of trials yielded an inward radial translation perception, which velocity was constant after 60 seconds of constant centrifugation. We therefore conclude that during centrifugation, an inward radial translation perception does exist in humans, which perception reaches a constant, non-zero value during constant rotation, lasting for at least one minute. These results can be understood by high-pass filtering of otolith afferents to make a distinction between inertial and gravitational acceleration, followed by a mere integration over time to reach a constant velocity perception.

  2. Vortex Whistle in Radial Intake

    National Research Council Canada - National Science Library

    Tse, Man-Chun

    2004-01-01

    In a radial-to-axial intake with inlet guide vanes (IGV) at the entry, a strong flow circulation Gamma can be generated from the tangential flow components created by the IGVs when their setting exceed about halfclosing (approx. 45 deg...

  3. Efficacy and tolerability of the galanin analog NAX 5055 in the multiple-hit rat model of symptomatic infantile spasms.

    Science.gov (United States)

    Jequier Gygax, Marine; Klein, Brian D; White, H Steve; Kim, Mimi; Galanopoulou, Aristea S

    2014-01-01

    Infantile spasms are seizures manifesting in infantile epileptic encephalopathies that are associated with poor epilepsy and cognitive outcomes. The current therapies are not always effective or are associated with serious side effects. Early cessation of spasms has been proposed to improve long-term outcomes. To identify new therapies for infantile spasms with rapid suppression of spasms, we are using the multiple-hit rat model of infantile spasms, which is a model of refractory infantile spasms. Here, we are testing the efficacy and tolerability of a single dose of the galanin receptor 1 preferring analog, NAX 5055, in the multiple-hit model of spasms. To induce the model, postnatal day 3 (PN3) male Sprague-Dawley rats underwent right intracerebral infusions of doxorubicin and lipopolysaccharide; p-chlorophenylalanine was then injected intraperitoneally (i.p.) at PN5. After the onset of spasms at PN4, 11-14 rats/group were injected i.p. with either NAX 5055 (0.5, 1, 2, or 4mg/kg) or vehicle. Video monitoring for spasms included a 1h pre-injection period, followed by 5h of recording post-injection, and two 2h sessions on PN5. The study was conducted in a randomized, blinded manner. Neurodevelopmental reflexes were assessed daily as well as at 2h after injection. Respiratory function, heart rate, pulse distension, oximetry and blood glucose were measured 4h after injection. The relative expression of GalR1 and GalR2 mRNA over β-actin in the cerebral cortex and hippocampus was determined with real time reverse transcription polymerase chain reaction. There was no acute effect of NAX 5055 on spasm frequency after the single dose of NAX 5055 (n=11-13 rats/group, following exclusions). Neurodevelopmental reflexes, vital signs, blood glucose measured 4h post-injection, and survival were not affected. A reduction in pulse and breath distention of unclear clinical significance was observed with the 7mg/kg NAX 5055 dose. GalR1 mRNA was present in the cerebral cortex and

  4. Radial head prosthesis: results overview.

    Science.gov (United States)

    Carità, E; Donadelli, A; Cugola, L; Perazzini, P

    2017-12-01

    Radial head replacement is frequently used in treatment of radial head fractures or sequela. Impossibility to restore a correct anatomy, acute elbow traumatic instability and failure of osteosynthesis hardware are the most common indications. The authors describe their case studies and results on the implantation of various radial head prostheses. Between June 2005 and June 2016, 28 radial head prostheses were implanted in the same number of patients with an average follow-up of 49 months (6-104). Indications for implantation were: Mason type III and IV radial head fractures and post-traumatic arthritis due to failure of previous treatments. Monopolar prostheses were used and were press-fit implanted via Kaplan's lateral access and Kocher's anconeus approach to the humeroradial joint. At the follow-up, assessments were made of the pain, according to the visual analogic scale, range of motion (ROM), stability and functionality according to the Mayo Elbow Performance Score, presence of osteolysis and mobilization during radiography tests, personal satisfaction of the patients, Disabilities of the Arm, Shoulder and Hand and Patient-Rated Wrist Evaluation outcomes measurements. At the follow-up, we recorded an average level of pain of 1.8 in patients under acute treatments for radial head fractures and a marked reduction in the remaining cases from 6.7 to 2.1. ROM was found on average to be 107° of flexion-extension and 159° of pronosupination. Personal satisfaction was good-excellent in 23 cases. There was no case of infection; removal of the implant was necessary in three cases due to mobilization of the stem and oversized implants. In six cases, bone resorption was seen at the level of the prosthetic collar and it was in all cases asymptomatic. The results of this study suggest that the use of prostheses, if well positioned, is a valid solution in the treatment of secondary arthritis and fractures of the radial head with poor prognosis, with good results in the

  5. Radial head dislocation during proximal radial shaft osteotomy.

    Science.gov (United States)

    Hazel, Antony; Bindra, Randy R

    2014-03-01

    The following case report describes a 48-year-old female patient with a longstanding both-bone forearm malunion, who underwent osteotomies of both the radius and ulna to improve symptoms of pain and lack of rotation at the wrist. The osteotomies were templated preoperatively. During surgery, after performing the planned radial shaft osteotomy, the authors recognized that the radial head was subluxated. The osteotomy was then revised from an opening wedge to a closing wedge with improvement of alignment and rotation. The case report discusses the details of the operation, as well as ways in which to avoid similar shortcomings in the future. Copyright © 2014 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

  6. ST Elevation Infarction after Heart Transplantation Induced by Coronary Spasms and Mural Thrombus Detected by Optical Coherence Tomography

    Directory of Open Access Journals (Sweden)

    Tor Skibsted Clemmensen

    2016-01-01

    Full Text Available The case illustrates the possible link between coronary spasms, intraluminal thrombus formation, and widespread organized and layered thrombi in HTx patients. Furthermore, the case underlines the clinical value of OCT as a novel method for high-resolution vessel imaging in heart-transplanted (HTx patients with coronary spasms and suspected coronary artery disease. Coronary spasms and sudden death are frequent complications after HTx. The underlying mechanisms leading to these complications are unknown. The present case displays the clinical course of a 19-year-old HTx patient who was hospitalized due to acute myocardial infarction induced by severe coronary spasms. The patients remained unstable on conservative therapy. Therefore, an optical coherence tomography (OCT was performed and revealed massive, organized thrombi in the left main coronary artery, the circumflex coronary artery, and the left anterior descending coronary artery. The patient was stabilized after percutaneous coronary intervention. As a mural thrombus often goes undetected by coronary angiography, OCT may prove benefit in HTx patients with myocardial infarction or suspected coronary spasms.

  7. CRYOTHERAPY: A NON-SURGICAL MANAGEMENT OPTION FOR SEVERE, MEDICALLY REFRACTORY SPASMS AFTER SPINAL CORD INJURY: TWO CASE REPORTS

    Directory of Open Access Journals (Sweden)

    Luxwell Jokonya

    2017-12-01

    Full Text Available Introduction: Neuromodulation in its various forms is emerging as a promising method of dealing with chronic pain and movement disorders. The scale of ablative vs augmentative procedures seems to be tilting towards augmentative procedures. We observed 8 patients who had failed medical treatment for muscle spasm respond to the cold application. Case summary: We report 2 cases of complete traumatic spinal cord injury patients, who developed severe, medically intractable muscle spasms. We applied cryotherapy to their legs with significant improvement. Outcome measurements: The spasm frequency score dropped immediately from a 4 to 0 in one patient. The other dropped from a 2 to 1 on day one then disappeared by day 7. Spasm severity dropped significantly on the first day in both cases. Conclusion: Cryotherapy as a form of neuromodulation, Is an effective, simple but safe way to symptomatically manage severe medically refractory muscle spasms in spinal cord injured patients. It becomes an important adjunct in the management of these patients in resource-limited settings where surgical options are not readily available.

  8. Radial Coordinates for Conformal Blocks

    CERN Document Server

    Hogervorst, Matthijs

    2013-01-01

    We develop the theory of conformal blocks in CFT_d expressing them as power series with Gegenbauer polynomial coefficients. Such series have a clear physical meaning when the conformal block is analyzed in radial quantization: individual terms describe contributions of descendants of a given spin. Convergence of these series can be optimized by a judicious choice of the radial quantization origin. We argue that the best choice is to insert the operators symmetrically. We analyze in detail the resulting "rho-series" and show that it converges much more rapidly than for the commonly used variable z. We discuss how these conformal block representations can be used in the conformal bootstrap. In particular, we use them to derive analytically some bootstrap bounds whose existence was previously found numerically.

  9. Superficial radial neuropathy following venepuncture.

    Science.gov (United States)

    Sheu, J J; Yuan, R Y

    2001-01-01

    A 42-year-old female suffered excruciating pain and paraesthesia on venepuncture of the cephalic vein in her left wrist. The left superficial radial nerve was injured. A flexed wrist during venepuncture renders the superficial radial nerve immobile and vulnerable to being punctured by the needle. To reduce the risk of nerve injury during venepuncture, the phlebotomist should choose a large and visible vein and insert the needle at a 5-15 degrees angle with the skin. The wrist should be selected only if the veins in the antecubital area are deemed unsuitable. The feeling of an electric shock along the distribution of the nerve, or rupture of the vein during venepuncture, should alert the phlebotomist to the possibility of nerve injury and the procedure should be stopped immediately.

  10. [Primary humero-radial arthrodesis].

    Science.gov (United States)

    Sándor, T

    1975-01-01

    The surgical treatment of a severe injury in the cubital region of a bus-driver, aged 47, is reported. Because of the extended contamination and the splintered fracture radical wound excision - involving also the chondral surfaces - has been performed and hereupon humero-radial arthrodesis was carried out. The skin defect has been successfully treated secondarily by insert of a flap. After uneventful recovery the patient could resume his work 6 months after the injury again.

  11. Radial magnetic bearings: An overview

    Directory of Open Access Journals (Sweden)

    Weiyu Zhang

    Full Text Available Radial magnetic bearings (RMBs are one of the most commonly used magnetic bearings. They are used widely in the field of ultra-high speed and ultra-precise numerical control machine tools, bearingless motors, high speed flywheels, artificial heart pumps, and molecular pumps, and they are being strengthened and extended in various important areas. In this paper, a comprehensive overview is given of different bearing topologies of RMBs with different stator poles that differ in their construction, the driving mode of electromagnets, power consumption, cost, magnetic circuits, and symmetry. RMBs with different poles and couplings between the two bearing axes in the radial direction responsible for cross-coupling generation are compared. In addition, different shaped rotors are compared, as the performances of magnetic bearing-rotor systems are of great concern to rotor constructions. Furthermore, the parameter design methods, the mathematical models and control strategies of the RMBs are described in detail. From the comparison of topologies, models and control methods for RMBs, the advantages, disadvantages and utilizable perspectives are also analyzed. Moreover, several possible development trends of the RMBs are discussed. Keywords: Radial magnetic bearings (RMBs, Topologies, Mathematical mode, Control strategies, Development trends

  12. Velocidades radiales en Collinder 121

    Science.gov (United States)

    Arnal, M.; Morrell, N.

    Se han llevado a cabo observaciones espectroscópicas de unas treinta estrellas que son posibles miembros del cúmulo abierto Collinder 121. Las mismas fueron realizadas con el telescopio de 2.15m del Complejo Astronómico El Leoncito (CASLEO). El análisis de las velocidades radiales derivadas del material obtenido, confirma la realidad de Collinder 121, al menos desde el punto de vista cinemático. La velocidad radial baricentral (LSR) del cúmulo es de +17 ± 3 km.s-1. Esta velocidad coincide, dentro de los errores, con la velocidad radial (LSR) de la nebulosa anillo S308, la cual es de ~20 ± 10 km.s-1. Como S308 se encuentra físicamente asociada a la estrella Wolf-Rayet HD~50896, es muy probable que esta última sea un miembro de Collinder 121. Desde un punto de vista cinemático, la supergigante roja HD~50877 (K3Iab) también pertenecería a Collinder 121. Basándonos en la pertenencia de HD~50896 a Collinder 121, y en la interacción encontrada entre el viento de esta estrella y el medio interestelar circundante a la misma, se estima para este cúmulo una distancia del orden de 1 kpc.

  13. Epileptic spasms and early-onset photosensitive epilepsy in Patau syndrome: An EEG study.

    Science.gov (United States)

    Spagnoli, Carlotta; Kugathasan, Umaiyal; Brittain, Helen; Boyd, Stewart G

    2015-08-01

    Patau syndrome, trisomy 13, is the third commonest autosomal trisomy. It is associated with a 25-50% prevalence of epilepsy, but detailed electroclinical descriptions are rare. The occurrence of early-onset photosensitivity has recently been reported in single patients. We collected electroclinical data on 8 infants (age range from 2 months to 3 years and 9 months, median: 17 months) with Patau syndrome referred for an EEG in our Clinical Neurophysiology Department between 1991 and 2011. All EEGs, case-notes, cytogenetic diagnosis and neuroimaging when available were reviewed; data on the occurrence of seizures, epileptiform discharges, photoparoxysmal response and their characteristics in terms of positive frequencies, latencies, grade and duration were noted and analysed. Two patients had been previously diagnosed with epilepsy (one with tonic spasms and one with multiple seizure types). We found 3 patients with photosensitive myoclonic epilepsy (37.5%), and one with non-photosensitive myoclonic epilepsy. We also recorded non-epileptic myoclonic jerks in one patient known to suffer from epileptic spasms. Among photosensitive patients we found self-limited, Waltz's grade 2-4, spike-wave/polyspike-wave discharges in low, medium and high frequency ranges in two patients and in the high frequency range in the third patient, with latencies and duration from less than 1s to a maximum of 9s. In our cohort of Patau syndrome patients, we found a high prevalence of spasms and photic-induced myoclonic jerks. Photosensitivity shows an unusual early age of onset. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  14. Adrenal function testing following hormone therapy for infantile spasms: case series and review of literature

    Directory of Open Access Journals (Sweden)

    John R Mytinger

    2015-12-01

    Full Text Available Prednisolone and adrenocorticotropic hormone (ACTH are hormone therapies for infantile spasms. There is limited data on the occurrence of decreased adrenal reserve or signs of clinical adrenal insufficiency after hormone therapy. This is a retrospective medical record review of patients referred to our Infantile Spasms Program. Our standardized infantile spasms management guideline began in September 2012 and initially included a post-hormone laboratory assessment of adrenal function. Medical records were assessed for hormone treatments, adrenal function testing, and signs of adrenal insufficiency. Forty-two patients who received one or both hormone therapies met inclusion criteria. A post-hormone laboratory assessment of adrenal function was done in 14 patients. Of these 14 patients, two had an abnormal laboratory assessment of adrenal function, both by adrenal stimulation testing – one after ACTH and one after prednisolone. One patient received hydrocortisone replacement and the other received stress dose hydrocortisone as needed; neither patient developed signs of adrenal insufficiency. Another patient treated with both types of hormone therapy in tandem, who did not have a post-hormone laboratory assessment, developed signs of mild adrenal insufficiency and required replacement hydrocortisone. Our study suggests that adrenal suppression can occur after modern hormone therapy regimens. We found two patients with abnormal adrenal function testing after hormone therapy and another patient with signs adrenal insufficiency. Given the seriousness of adrenal crisis, caregiver education on the signs of adrenal insufficiency is critical. Greater vigilance may be indicated in patients receiving both types of hormone therapy in tandem. Although a routine post-hormone laboratory assessment of adrenal function may not be feasible in all patients, replacement or stress dose hydrocortisone is necessary for all patients with suspected adrenal

  15. Outcome of synthetic adrenocorticotropin hormone treatment in children with infantile spasm

    Directory of Open Access Journals (Sweden)

    I Gusti Ngurah Made Suwarba

    2011-04-01

    Full Text Available Background Infantile spasms (IS is an age-spedfic epilepsy syndrome characterized by flexor, extensor, and mixed flexor-extensor spasms which often occur in clusters during the first 2 years of life. IS is often difficult to manage 'With the usual anti-epilepsy drugs (AEDs. Therapy with adrenocorticotropin honnone (ACTH has been used since 1958. In Indonesia, ACTH usage is still rare. Objective This study aims to examine the effectiveness of ACTH as an anti-epileptic drug in managing IS. Methods This was descriptive retrospective cohort study. Subjects were IS patients who visited the neurology outpatient clinic in Sanglah Hospital, Bali, from January 2007 until June 2010. Each subject received AED(s plus either ACTH or methylprednisolone for 46 weeks. Results There were 19 IS patients over the four year duration of this study. They were mostly boys (11, aged 2 weeks to 17 months, with a mean age at treatment of 9 months. Eighteen patients received poly therapy, while one patient received only phenobarbital as monotherapy. Most patients who received ACTH (13/16 had a seizure-free period, while the 3 that did not receive ACTH continued having seizures. Patients who received ACTH showed a good response (seizure-free after 5-13 days therapy and their EEG pattern showed disappearance of burst suppression Mthin 1-2 weeks. ACTH side effects included weight gain and cushingoid appearance. One patient died from pneumonia. Conclusions Diagnosis of IS should be considered in patients pre-senting Mth spasms at less than 6 months old. IS treatment should begin as soon as possible. IS patients responded well to a short course of ACTH therapy.

  16. Adrenal Function Testing Following Hormone Therapy for Infantile Spasms: Case Series and Review of Literature.

    Science.gov (United States)

    Mytinger, John R; Bowden, Sasigarn A

    2015-01-01

    Prednisolone and adrenocorticotropic hormone (ACTH) are "hormone" therapies for infantile spasms. There is limited data on the occurrence of decreased adrenal reserve or signs of clinical adrenal insufficiency after hormone therapy. This is a retrospective medical record review of patients referred to our Infantile Spasms Program. Our standardized infantile spasms management guideline began in September 2012 and initially included a post-hormone laboratory assessment of adrenal function. Medical records were assessed for hormone treatments, adrenal function testing, and signs of adrenal insufficiency. Forty-two patients who received one or both hormone therapies met inclusion criteria. A post-hormone laboratory assessment of adrenal function was done in 14 patients. Of these 14 patients, 2 had an abnormal laboratory assessment of adrenal function, both by adrenal stimulation testing - one after ACTH and one after prednisolone. One patient received hydrocortisone replacement and the other received stress dose hydrocortisone as needed; neither patient developed signs of adrenal insufficiency. Another patient treated with both types of hormone therapy in tandem, who did not have a post-hormone laboratory assessment, developed signs of mild adrenal insufficiency and required replacement hydrocortisone. Our study suggests that adrenal suppression can occur after modern hormone therapy regimens. We found two patients with abnormal adrenal function testing after hormone therapy and another patient with signs adrenal insufficiency. Given the seriousness of adrenal crisis, caregiver education on the signs of adrenal insufficiency is critical. Greater vigilance may be indicated in patients receiving both types of hormone therapy in tandem. Although a routine post-hormone laboratory assessment of adrenal function may not be feasible in all patients, replacement or stress dose hydrocortisone is necessary for all patients with suspected adrenal insufficiency.

  17. 5-Fluorouracil-induced acute reversible heart failure not explained by coronary spasms, myocarditis or takotsubo

    DEFF Research Database (Denmark)

    Fakhri, Yama; Dalsgaard, Morten; Nielsen, Dorte

    2016-01-01

    A 69-year-old woman presented with arterial hypotension, pulmonary oedema and a severely depressed left ventricular ejection fraction (LVEF) of 25% only 3 days after having received her first treatment for colorectal cancer with 5-fluorouracil (5-FU)-based therapy. The ECG demonstrated widespread......, cardiac MRI scan 9 days later showed a normal LVEF with signs of neither myocardial oedema nor necrosis. Despite the high therapeutic efficacy of 5-FU in treatment of colorectal cancer, it is associated with undesired cardiac toxicities including coronary spasms, toxic inflammation and takotsubo...

  18. Post-valvular surgery multi-vessel coronary artery spasm - A literature review.

    Science.gov (United States)

    Formica, Francesco; Bamodu, Oluwaseun Adebayo; Mariani, Serena; Paolini, Giovanni

    2016-03-01

    Coronary artery spasm (CAS) refers to the spontaneous or stimuli-induced transient, often localized and intense subtotal or total constriction/occlusion of the epicardial coronary artery, usually concomitant with angina pectoris with associated elevation of the ST segment on electrocardiogram (ECG). In this article, we present a literature review on post-valvular surgery CAS and report the clinical case of a 77 year-old man who experienced severe early post-aortic surgery chest pain and hemodynamic instability. Emergent coronary angiography revealed severe occlusion of multiple branches of both coronary arteries. The CAS was alleviated with intracoronary infusion of nitroglycerin.

  19. Occurrence of bilaterally independent epileptic spasms after a corpus callosotomy in West syndrome.

    Science.gov (United States)

    Kobayashi, Katsuhiro; Endoh, Fumika; Toda, Yoshihiro; Oka, Makio; Baba, Hiroshi; Ohtsuka, Yoko; Yoshinaga, Harumi

    2016-01-01

    We report a patient with intractable West syndrome whose epileptic spasms (ESs) were initially bilaterally synchronous, as is typical; after a complete corpus callosotomy, however, bilaterally independent ESs originated in either hemisphere. Activity of probable cortical origin associated with ESs was detected by observing ictal gamma oscillations. Brain MRI revealed no structural abnormality before surgery. This case suggests that ESs with a hemispheric origin may appear generalized because of synchronizing effects in the corpus callosum in some patients. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  20. Efficacy and tolerability of high-dose prednisone in Chinese children with infantile spasms.

    Science.gov (United States)

    Yi, Zhao-Shi; Wu, Hua-Ping; Yu, Xiong-Ying; Chen, Yong; Zhong, Jian-Min

    2015-01-01

    The aim of this study is to preliminarily evaluate the clinical efficacy and safety of high-dose prednisone in the treatment of infantile spasms (IS) in China, and to provide additional choice of the therapy of IS. Twenty patients aged 3-53 months with IS were collected in the Department of Neurology of Jiangxi Children's Hospital from May in 2011 to December in 2012, who were placed on high-dose prednisone (took prednisone tablet of 10mg four times a day) for 2 weeks during admission to our hospital. The assessment of spasms seizure and video-EEG monitoring were preformed before treatment and after 2 weeks and the end of treatment of the regimen (7 weeks), respectively. All of the children were followed-up for 2-14 months. Among 20 cases, there were 16 cases (80.0%) with complete cessation of spasms after 2 weeks and 13 cases (65.0%) after 7 weeks. There were 19 cases with typical or modified hypsarrhythmia in 20 cases. No matter after 2 or 7 weeks, there were 12 cases showed complete resolution of hypsarrhythmia and 7 cases with only a partial remission of hypsarrhythmia. After a follow-up of 2-14 months, the longest spasm-free interval was 14 months and the shortest one was 11 days. Six cases relapsed in different periods, and the relapse rate was 35.3%. Amongst the main adverse events, there were Cushing's symptoms in 15 cases (75.0%), irritability in 8 cases (40.0%), drosiness in 3 cases (15.0%), high blood pressure in 3 cases (15.0%), and infections in 8 cases (40.0%), but no one stopped the treatment because of the adverse reactions. In total, high-dose prednisone was effective and well-tolerated in children with IS in China. Maybe the regimen will become a new choice in the treatment of IS. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  1. Efficacy and safety of vigabatrin in Japanese patients with infantile spasms: Primary short-term study and extension study.

    Science.gov (United States)

    Ohtsuka, Yoko

    2018-01-01

    Vigabatrin was approved for the treatment of infantile spasms by the US Food and Drug Administration, but not in Japan at the time of initiating this clinical study because of concerns about irreversible peripheral visual field defects (VFDs). This study evaluated the efficacy and safety of vigabatrin for Japanese patients with infantile spasms. Of 15 patients (aged ≥4weeks and spasms that were treatment refractory; these patients were concurrently treated with at least one other antiepileptic drug. One patient received vigabatrin monotherapy. Eight of the 13 patients (61.5% [95% CI: 31.6-86.1%]) had a ≥50% reduction during the dose-adjustment phase compared with baseline in the frequency of spasms, with efficacy maintained through a 2-week maintenance phase. Spasms disappeared in six out of nine patients (66.7% [95% CI: 29.9-92.5%]) who transitioned to the maintenance phase and hypsarrhythmia on electroencephalography also resolved in four patients. Hypsarrhythmia was improved in another two patients. Six out of seven patients who continued treatment through Week 32 of an extension study reported ongoing efficacy for vigabatrin. The most common adverse events (AEs) were psychiatric disorders and nervous system disorders (n=8; 61.5%) that were generally mild in severity. No treatment-related peripheral VFDs were observed. No severe AEs or AEs resulting in discontinuation of vigabatrin therapy were reported. An abnormality in magnetic resonance images was observed in one patient during the extension period. Vigabatrin was deemed to be clinically effective and well tolerated in Japanese patients with infantile spasms. Copyright © 2017 The Author. Published by Elsevier Inc. All rights reserved.

  2. Deriving High-Precision Radial Velocities

    Science.gov (United States)

    Figueira, Pedro

    This chapter describes briefly the key aspects behind the derivation of precise radial velocities. I start by defining radial velocity precision in the context of astrophysics in general and exoplanet searches in particular. Next I discuss the different basic elements that constitute a spectrograph, and how these elements and overall technical choices impact on the derived radial velocity precision. Then I go on to discuss the different wavelength calibration and radial velocity calculation techniques, and how these are intimately related to the spectrograph's properties. I conclude by presenting some interesting examples of planets detected through radial velocity, and some of the new-generation instruments that will push the precision limit further.

  3. ST Elevation Infarction after Heart Transplantation Induced by Coronary Spasms and Mural Thrombus Detected by Optical Coherence Tomography

    DEFF Research Database (Denmark)

    Clemmensen, Tor Skibsted; Holm, Niels Ramsing; Eiskjær, Hans

    2016-01-01

    The case illustrates the possible link between coronary spasms, intraluminal thrombus formation, and widespread organized and layered thrombi in HTx patients. Furthermore, the case underlines the clinical value of OCT as a novel method for high-resolution vessel imaging in heart-transplanted (HTx......, and the left anterior descending coronary artery. The patient was stabilized after percutaneous coronary intervention. As a mural thrombus often goes undetected by coronary angiography, OCT may prove benefit in HTx patients with myocardial infarction or suspected coronary spasms....

  4. Vigabatrin Therapy for Infantile Spasms in a Case of Cardiofaciocutaneous Syndrome with Cardiac Hypertrophy Developing during Adrenocorticotropic Hormone Treatment.

    Science.gov (United States)

    Hatori, Takayuki; Sugiyama, Yohei; Yamashita, Shinichiro; Hirakubo, Yuka; Nonaka, Kazuhito; Ichihashi, Ko

    2016-01-01

    In a patient with cardiofaciocutaneous syndrome complicated by intractable infantile spasms (West syndrome), cardiac hypertrophy developed during adrenocorticotropic hormone treatment. Various types of antiepileptic drugs, intravenous immunoglobulin, thyrotropin releasing hormone, and a ketogenic diet were ineffective in this case. However, vigabatrin both decreased clinical seizures and improved electroencephalogram findings. Although vigabatrin has not been approved for use in Japan, the results in the present case suggest that this drug should be considered as an alternative therapy for cases of infantile spasms associated with syndromes involving cardiomyopathy or its potential risk factors, such as cardiofaciocutaneous syndrome.

  5. Does treatment have an impact on incidence and risk factors for autism spectrum disorders in children with infantile spasms?

    Science.gov (United States)

    Bitton, Jonathan Y; Demos, Michelle; Elkouby, Katia; Connolly, Mary; Weiss, Shelly K; Donner, Elizabeth J; Whiting, Sharon; Ronen, Gabriel M; Bello-Espinosa, Luis; Wirrell, Elaine C; Mohamed, Ismail S; Dooley, Joseph M; Carmant, Lionel

    2015-06-01

    Infantile spasms (IS) are a severe form of childhood epilepsy associated with autism spectrum disorders (ASD) in up to 35% of cases. The objective of this post hoc analysis of our randomized control trial was to determine whether rapid diagnosis and treatment of IS could limit the incidence of ASD while identifying risk factors related to ASD outcome. Patients with IS were randomized in a standardized diagnostic and treatment protocol. Clinical and electroencephalogram (EEG) evaluations were completed at all eight visits over 5 years, while cognitive evaluations were administered at 0, 6, 24 and 60 months, respectively. Autism was initially screened by means of the Checklist for Autism in Toddlers (CHAT) at 24 months, and formally assessed at the 30-and 60-month follow-ups using the Autism Diagnostic Observation Schedule-Generic (ADOS-G). Of the 69 patients included in the study, 25 could not be assessed due to severe delay or death. Eleven of the 42 patients screened with CHAT, were found to be at risk of an ASD outcome. ADOS was performed in 44 and 10 were diagnosed with ASD. The CHAT proved to correlate highly with the ADOS (80% ppv). Only patients with symptomatic IS developed ASD (p = 0.003). Earlier diagnosis or successful treatment did not correlate with a reduced rate of ASD. Other risk factors were identified such as having chronic epileptic discharges in the frontotemporal areas after disappearance of hypsarrhythmia (p = 0.005 and p = 0.007) and being of nonwhite origin (p = 0.009). ASD was only observed in children with sympyomatic IS. Other clinical risk factors include chronic frontotemporal epileptic activity and being of non-white origin. Early diagnosis and treatment did not prevent ASD as an outcome of IS. However, patients at risk for ASD could be identified early on and should in the future benefit from early intervention to potentially improve their long-term outcome. Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.

  6. Prevention: Exercise

    Medline Plus

    Full Text Available ... Pain Other Scoliosis Back Pain and Emotional Distress Muscle Spasms Pinched Nerve Discitis Degenerative Conditions Bulge vs ... exercise focus on core strengthening, or building the muscles that provide support for your body. Pilates, yoga ...

  7. Prevention: Exercise

    Medline Plus

    Full Text Available Toggle navigation CONDITIONS Low Back Pain Acute Low Back Pain Chronic Low Back Pain SI Joint Pain Other Scoliosis Back Pain and Emotional Distress Muscle Spasms Pinched Nerve Discitis Degenerative Conditions Bulge vs ...

  8. Axial and Radial Oxylipin Transport.

    Science.gov (United States)

    Gasperini, Debora; Chauvin, Adeline; Acosta, Ivan F; Kurenda, Andrzej; Stolz, Stéphanie; Chételat, Aurore; Wolfender, Jean-Luc; Farmer, Edward E

    2015-11-01

    Jasmonates are oxygenated lipids (oxylipins) that control defense gene expression in response to cell damage in plants. How mobile are these potent mediators within tissues? Exploiting a series of 13-lipoxygenase (13-lox) mutants in Arabidopsis (Arabidopsis thaliana) that displays impaired jasmonic acid (JA) synthesis in specific cell types and using JA-inducible reporters, we mapped the extent of the transport of endogenous jasmonates across the plant vegetative growth phase. In seedlings, we found that jasmonate (or JA precursors) could translocate axially from wounded shoots to unwounded roots in a LOX2-dependent manner. Grafting experiments with the wild type and JA-deficient mutants confirmed shoot-to-root oxylipin transport. Next, we used rosettes to investigate radial cell-to-cell transport of jasmonates. After finding that the LOX6 protein localized to xylem contact cells was not wound inducible, we used the lox234 triple mutant to genetically isolate LOX6 as the only JA precursor-producing LOX in the plant. When a leaf of this mutant was wounded, the JA reporter gene was expressed in distal leaves. Leaf sectioning showed that JA reporter expression extended from contact cells throughout the vascular bundle and into extravascular cells, revealing a radial movement of jasmonates. Our results add a crucial element to a growing picture of how the distal wound response is regulated in rosettes, showing that both axial (shoot-to-root) and radial (cell-to-cell) transport of oxylipins plays a major role in the wound response. The strategies developed herein provide unique tools with which to identify intercellular jasmonate transport routes. © 2015 American Society of Plant Biologists. All Rights Reserved.

  9. Exceptional circles of radial potentials

    International Nuclear Information System (INIS)

    Music, M; Perry, P; Siltanen, S

    2013-01-01

    A nonlinear scattering transform is studied for the two-dimensional Schrödinger equation at zero energy with a radial potential. Explicit examples are presented, both theoretically and computationally, of potentials with nontrivial singularities in the scattering transform. The singularities arise from non-uniqueness of the complex geometric optics solutions that define the scattering transform. The values of the complex spectral parameter at which the singularities appear are called exceptional points. The singularity formation is closely related to the fact that potentials of conductivity type are ‘critical’ in the sense of Murata. (paper)

  10. Radial to axillary nerve transfer.

    Science.gov (United States)

    Vanaclocha, Vicente; Herrera, Juan Manuel; Rivera-Paz, Marlon; Martínez-Gómez, Deborah; Vanaclocha, Leyre

    2018-01-01

    Axillary nerve injury is common after brachial plexus injuries, particularly with shoulder luxation. Nerve grafting is the traditional procedure for postganglionic injuries. Nerve transfer is emerging as a viable option particularly in late referrals. At the proximal arm the radial and axillary nerves lie close by. Sacrificing one of the triceps muscle nerve branches induces little negative consequences. Transferring the long head of the triceps nerve branch is a good option to recover axillary nerve function. The surgical technique is presented in a video, stressing the steps to achieve a successful result. The video can be found here: https://youtu.be/WbVbpMuPxIE .

  11. Transient left ventricular dysfunction due to coronary spasm after spinal anesthesia with bupivacaine - a case report.

    Science.gov (United States)

    Elikowski, Waldemar; Małek-Elikowska, Małgorzata; Słomczyński, Marek; Horbacka, Karolina; Bartkowski, Jarosław; Kalawski, Bartosz

    2017-10-23

    Bupivacaine is a long-acting local anesthetic (LA) used for cutaneous infiltration, peripheral nerve blocks, epidural and spinal anesthesia. However, its application may result in cardiovascular complications such as: hypotension, bradycardia, cardiac arrest and toxic myocardial injury. The authors describe a 53-year-old male with a history of cigarette smoking, admitted for an elective inguinal hernia surgery. Before surgery, the patient received subarachnoid injection of bupivacaine (20 mg). After the operation, he developed transient hypotension. Blood pressure returned to normal after gelofusine infusion; no sympathomimetics were administered. The male denied chest pain; however, ECG showed ST segment elevation coexisting with left ventricular anterolateral hypokinesia and decreased longitudinal strain in echocardiography. A significant increase in troponin I level was suggestive rather of myocardial infarction than of takotsubo cardiomyopathy. Urgent coronary angiography revealed left anterior descending artery spasm, which remitted after intracoronary nitroglycerin injection. Normalization of ECG and echocardiography was observed within a few days. The authors indicate that the presented atypical adverse effect of bupivacaine manifested itself with delay and that coronary spasm proceeded without angina. A close observation of the patient after anesthetic procedure with LA should be extended over the postoperative period.

  12. Espasmo hemifacial familiar: relato de dois casos Familial hemifacial spasm: report of two cases

    Directory of Open Access Journals (Sweden)

    EGBERTO REIS BARBOSA

    1998-03-01

    Full Text Available Os autores relatam os achados clínicos e angiográficos de dois casos de espasmo hemifacial familiar. Esta é a quinta descrição sobre esse tema na literatura e apresenta mãe e filha com idades de 76 e 51 anos respectivamente, nas quais o lado esquerdo foi o acometido. Exames de angiorressonância realizados nas pacientes revelaram dolicobasilar com origem lateralizada à esquerda para ambos os casos. Também demonstraram artéria cerebelar póstero-inferior muito desenvolvida e irregularidades murais nas artérias vertebrais e basilar sugestivas de arteriosclerose na mãe e vasos intracranianos levemente alongados na filha. Dados de revisão da literatura e sobre a etiologia do espasmo hemifacial são enfocados.The authors report the clinical and angiographical findings of two cases of familial hemifacial spasm. This is the fifth description in the literature and presents mother and daughter at the ages of 76 and 51 respectively, in whom the left side was affected. They underwent exams of angioresonance that showed dolichobasilar with left side origin in both patients. The exams also demonstrated postero-inferior cerebellar artery very developed and irregularities in the walls of the vertebral and basilar arteries suggestive of arteriosclerosis in the mother and slightly elongated intracranial vessels in the daughter. Literature review and etiology data of the hemifacial spasm are focused.

  13. Predictive factors for relapse of epileptic spasms after adrenocorticotropic hormone therapy in West syndrome.

    Science.gov (United States)

    Hayashi, Yumiko; Yoshinaga, Harumi; Akiyama, Tomoyuki; Endoh, Fumika; Ohtsuka, Yoko; Kobayashi, Katsuhiro

    2016-01-01

    To investigate whether serial electroencephalographic (EEG) findings can predict relapse of epileptic spasms after synthetic adrenocorticotropic hormone (ACTH) therapy in patients with West syndrome (WS). Thirty-nine WS patients (8 cryptogenic and 31 symptomatic) were included in this study. These patients received ACTH therapy for the first time and were regularly followed up for more than three years at our hospital. Sixteen patients (41.0%) showed seizure relapse (relapse group) and 23 patients (59.0%) did not show relapse (non-relapse group). We used survival analysis to investigate the influence of etiology and presence of epileptic discharges after the ACTH therapy on seizure outcome. Immediately after the ACTH therapy, etiology was associated with seizure outcome (p=0.003). In the early stage (1 month after the ACTH therapy), only the presence of epileptic discharges (p=0.001) had a significant association with seizure outcome, regardless of etiology. Because all relapsed patients were in the symptomatic group, we performed the same statistical analysis on symptomatic WS patient data only. We found that the group with no epileptic discharges on EEG showed a significantly higher seizure-free rate than those with epileptic discharges in the early stage (p=0.0091). This study demonstrated that serial EEG findings after ACTH therapy are significantly related to relapse of epileptic spasms. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  14. Whole-exome sequencing improves the diagnosis yield in sporadic infantile spasm syndrome.

    Science.gov (United States)

    Dimassi, S; Labalme, A; Ville, D; Calender, A; Mignot, C; Boutry-Kryza, N; de Bellescize, J; Rivier-Ringenbach, C; Bourel-Ponchel, E; Cheillan, D; Simonet, T; Maincent, K; Rossi, M; Till, M; Mougou-Zerelli, S; Edery, P; Saad, A; Heron, D; des Portes, V; Sanlaville, D; Lesca, G

    2016-02-01

    Infantile spasms syndrome (ISs) is characterized by clinical spasms with ictal electrodecrement, usually occurring before the age of 1 year and frequently associated with cognitive impairment. Etiology is widely heterogeneous, the cause remaining elusive in 40% of patients. We searched for de novo mutations in 10 probands with ISs and their parents using whole-exome sequencing (WES). Patients had neither consanguinity nor family history of epilepsy. Common causes of ISs were excluded by brain magnetic resonance imaging (MRI), metabolic screening, array-comparative genomic hybridization (CGH) and testing for mutations in CDKL5, STXBP1, and for ARX duplications. We found a probably pathogenic mutation in four patients. Missense mutations in SCN2A (p.Leu1342Pro) and KCNQ2 (p.Ala306Thr) were found in two patients with no history of epilepsy before the onset of ISs. The p.Asn107Ser missense mutation of ALG13 had been previously reported in four females with ISs. The fourth mutation was an in-frame deletion (p.Phe110del) in NR2F1, a gene whose mutations cause intellectual disability, epilepsy, and optic atrophy. In addition, we found a possibly pathogenic variant in KIF3C that encodes a kinesin expressed during neural development. Our results confirm that WES improves significantly the diagnosis yield in patients with sporadic ISs. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  15. Cranial computed tomography in infantile spasms; Primary findings related to long-term mental prognosis

    Energy Technology Data Exchange (ETDEWEB)

    Howitz, P. (Dept. of Paediatrics, Univ. Hospital, Rigshospitalet, Copenhagen (Denmark)); Neergaard, K.; Pedersen, H. (Dept. of Radiology, Univ. Hospital, Rigshospitalet, Copenhagen (Denmark))

    1990-01-01

    Out of 109 children with infantile spasms (IS), prospectively tested during the years 1976 to 1979 in Denmark, 52 children were examined by cranial computed tomography (CT). The classification of IS into cryptogenic (CR), symptomatic (SY) and doubtful (DO) was done clinically without considering the CT-finding. Sixty per cent of the scannings were abnormal. Only 6/30 (20%) of the children in ACTH treatment were found to develop cerebral atrophy which means that this finding is not an obligatory side-effect of ACTH treatment of children with IS. Normal CT-findings were found in 50% of the CR and 50% of the SY + DO-groups, and could not be used as a prognostic tool for estimating the mental development. This was also the case for children with cerebral atrophy. Abnormal CT-findings (minus atrophy) were highly correlated to the group with clinical symptoms and indicate an extremely unsatisfying long-term mental prognosis. CT-scanning is a valuable tool for the examination of clearing children with infantile spasms. (authors).

  16. Auditory processing following infantile spasms: An event-related potential study.

    Science.gov (United States)

    Fosi, Tangunu; Werner, Klaus; Boyd, Stewart G; De Haan, Michelle; Scott, Rod C; Neville, Brian G

    2017-05-01

    To investigate acoustic auditory processing in patients with recent infantile spasms (IS). Patients (n = 22; 12 female; median age 8 months; range 5-11 months) had normal preceding development, brain magnetic resonance imaging (MRI), and neurometabolic testing (West syndrome of unknown cause, uWS). Controls were healthy babies (n = 22; 11 female; median age 6 months; range 3-12 months). Event-related potentials (ERPs) and psychometry (Bayley Scales of Infant Development, Second Edition, BSID-II) took place at a month following IS remission. Following a repeated pure tone, uWS patients showed less suppression of the N100 at the mid-temporal electrodes (p = 0.006), and a prolonged response latency (p = 0.019). Their novelty P300 amplitude over the mid-temporal electrodes was halved (p = 0.001). The peak of the novelty P300 to environmental broadband sounds emerged later over the left temporal lobe in patients (p = 0.015), the lag correlating with duration of spasms (r = 0.547, p = 0.015). BSID-II scores were lower in patients (p < 0.001), with no correlation to ERP. Complex acoustic information is processed poorly following IS. This would impair language. Treatment did not reverse this phenomenon, but may have limited its severity. The data are most consistent with altered connectivity of the cortical acoustic processing areas induced by IS. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

  17. Coronary spasm after the topical use of cocaine in nasal surgery.

    Science.gov (United States)

    Lenders, Guy D; Jorens, Philippe G; De Meyer, Tim; Vandendriessche, Tom; Verbrugghe, Walter; Vrints, Christiaan J

    2013-01-01

    Cocaine is a frequently used recreational drug which imposes important health problems with even life-threatening cardiotoxicity. The therapeutic use of cocaine is nowadays restricted to topical anesthesia in ophthalmological and nasal surgery but the possible hazards of this local anesthesia are not always fully appreciated. A 51-year old male patient with moderate cardiovascular risk profile underwent elective nasal surgery and cocaine was used as a local anesthetic. During surgery, ventricular arrhythmias and cardiogenic shock occurred, mimicking an ST-segment elevation myocardial infarction (STEMI) in sinus rhythm. Coronary angiography showed diffuse spasm of the right coronary artery (RCA) which disappeared with intracoronary nitrates. Urine analysis was positive for cocaine. The patient recovered completely with a normal echocardiography and ECG at discharge. Cocaine cardiotoxicity is not uncommon in the community but a particular situation arises when used in medicine as a topical anesthetic. This is the first case report, to our knowledge, of a cardiogenic shock mimicking a STEMI with documentation of diffuse coronary spasm after cocaine use in nasal surgery. One must be aware of the potential life-threatening complications in this low-risk surgery, moreover when safer alternatives are available.

  18. A Triplet Repeat Expansion Genetic Mouse Model of Infantile Spasms Syndrome, Arx(GCG)10+7, with Interneuronopathy, Spasms in Infancy, Persistent Seizures, and Adult Cognitive and Behavioral Impairment

    Science.gov (United States)

    Price, Maureen G.; Yoo, Jong W.; Burgess, Daniel L.; Deng, Fang; Hrachovy, Richard A.; Frost, James D.; Noebels, Jeffrey L.

    2009-01-01

    Infantile spasms syndrome (ISS) is a catastrophic pediatric epilepsy with motor spasms, persistent seizures, mental retardation, and in some cases, autism. One of its monogenic causes is an insertion mutation (c.304ins (GCG)7) on the X chromosome, expanding the first polyalanine tract of the interneuron-specific transcription factor ARX from 16 to 23 alanine codons. Null mutation of the Arx gene impairs GABA- and cholinergic interneuronal migration but results in a neonatal lethal phenotype. We developed the first viable genetic mouse model of ISS that spontaneously recapitulates salient phenotypic features of the human triplet-repeat expansion mutation. Arx (GCG)10+7 (“Arx Plus7”) pups display abnormal spasm-like myoclonus and other key EEG features, including multifocal spikes, electrodecremental episodes, and spontaneous seizures persisting into maturity. The neurobehavioral profile of Arx mutants was remarkable for lowered anxiety, impaired associative learning, and abnormal social interaction. Laminar decreases of Arx+ cortical interneurons and a selective reduction of calbindin-, but not parvalbumin- or calretinin-expressing interneurons in neocortical layers and hippocampus indicate that specific classes of synaptic inhibition are missing from the adult forebrain, providing a basis for the seizures and cognitive disorder. A significant reduction of calbindin, NPY-expressing and cholinergic interneurons in the mutant striatum suggest that dysinhibition within this network may contribute to the dyskinetic motor spasms. This mouse model narrows the range of critical pathogenic elements within brain inhibitory networks essential to recreate this complex neurodevelopmental syndrome. PMID:19587282

  19. Malignant Multivessel Coronary Spasm Complicated by Myocardial Infarction, Transient Complete Heart Block, Ventricular Fibrillation, Cardiogenic Shock and Ischemic Stroke

    Directory of Open Access Journals (Sweden)

    Viji S. Thomson

    2014-07-01

    Full Text Available Multivessel coronary spasm resulting to cardiogenic shock and malignant ventricular arrhythmias though rare has been reported in the literature. The disease seems to be more prevalent in Asians. There have been isolated reports of coronary spasm in patients with reactive airway disease. We report the first case of spontaneous multivessel spasm in a male patient with bronchial asthma of Arab ethnicity resulting in acute myocardial infarction complicated by cardiogenic shock, recurrent ventricular arrhythmias, and transient complete heart block. Literature review of similar cases suggests a strong association with bronchial asthma and a more malignant course in patients with reactive airway disease. The role of intracoronary nitroglycerin in proving the diagnosis even in patients in shock on maximal inotropic supports and intra-aortic balloon pump is highlighted and the importance of considering multivessel coronary spasm as a cause for acute coronary syndrome even in patients with conventional risk factors for atherosclerotic coronary artery disease is reinforced in the discussion of this case.

  20. Pathogenesis and new candidate treatments for infantile spasms and early life epileptic encephalopathies: A view from preclinical studies.

    Science.gov (United States)

    Galanopoulou, Aristea S; Moshé, Solomon L

    2015-07-01

    Early onset and infantile epileptic encephalopathies (EIEEs) are usually associated with medically intractable or difficult to treat epileptic seizures and prominent cognitive, neurodevelopmental and behavioral consequences. EIEEs have numerous etiologies that contribute to the inter- and intra-syndromic phenotypic variability. Etiologies include structural and metabolic or genetic etiologies although a significant percentage is of unknown cause. The need to better understand their pathogenic mechanisms and identify better therapies has driven the development of animal models of EIEEs. Several rodent models of infantile spasms have emerged that recapitulate various aspects of the disease. The acute models manifest epileptic spasms after induction and include the NMDA rat model, the NMDA model with prior prenatal betamethasone or perinatal stress exposure, and the γ-butyrolactone induced spasms in a mouse model of Down syndrome. The chronic models include the tetrodotoxin rat model, the aristaless related homeobox X-linked (Arx) mouse models and the multiple-hit rat model of infantile spasms. We will discuss the main features and findings from these models on target mechanisms and emerging therapies. Genetic models have also provided interesting data on the pathogenesis of Dravet syndrome and proposed new therapies for testing. The genetic associations of many of the EIEEs have also been tested in rodent models as to their pathogenicity. Finally, several models have tested the impact of subclinical epileptiform discharges on brain function. The impact of these advances in animal modeling for therapy development will be discussed. Copyright © 2015 Elsevier Inc. All rights reserved.

  1. Hemifacial Spasm

    Science.gov (United States)

    ... National Institute of Neurological Disorders and Stroke (NINDS) conducts and supports research related to hemifacial spams through grants to major research institutions ... Publications Definition Hemifacial ...

  2. Formulas for Radial Transport in Protoplanetary Disks

    Science.gov (United States)

    Desch, Steven J.; Estrada, Paul R.; Kalyaan, Anusha; Cuzzi, Jeffrey N.

    2017-05-01

    The quantification of the radial transport of gaseous species and solid particles is important to many applications in protoplanetary disk evolution. An especially important example is determining the location of the water snow lines in a disk, which requires computing the rates of outward radial diffusion of water vapor and the inward radial drift of icy particles; however, the application is generalized to evaporation fronts of all volatiles. We review the relevant formulas using a uniform formalism. This uniform treatment is necessary because the literature currently contains at least six mutually exclusive treatments of radial diffusion of gas, only one of which is correct. We derive the radial diffusion equations from first principles using Fick's law. For completeness, we also present the equations for radial transport of particles. These equations may be applied to studies of diffusion of gases and particles in protoplanetary and other accretion disks.

  3. Ulnar nerve entrapment complicating radial head excision

    Directory of Open Access Journals (Sweden)

    Kevin Parfait Bienvenu Bouhelo-Pam

    Full Text Available Introduction: Several mechanisms are involved in ischemia or mechanical compression of ulnar nerve at the elbow. Presentation of case: We hereby present the case of a road accident victim, who received a radial head excision for an isolated fracture of the radial head and complicated by onset of cubital tunnel syndrome. This outcome could be the consequence of an iatrogenic valgus of the elbow due to excision of the radial head. Hitherto the surgical treatment of choice it is gradually been abandoned due to development of radial head implant arthroplasty. However, this management option is still being performed in some rural centers with low resources. Discussion: The radial head plays an important role in the stability of the elbow and his iatrogenic deformity can be complicated by cubital tunnel syndrome. Conclusion: An ulnar nerve release was performed with favorable outcome. Keywords: Cubital tunnel syndrome, Peripheral nerve palsy, Radial head excision, Elbow valgus

  4. Radial smoothing and closed orbit

    International Nuclear Information System (INIS)

    Burnod, L.; Cornacchia, M.; Wilson, E.

    1983-11-01

    A complete simulation leading to a description of one of the error curves must involve four phases: (1) random drawing of the six set-up points within a normal population having a standard deviation of 1.3 mm; (b) random drawing of the six vertices of the curve in the sextant mode within a normal population having a standard deviation of 1.2 mm. These vertices are to be set with respect to the axis of the error lunes, while this axis has as its origins the positions defined by the preceding drawing; (c) mathematical definition of six parabolic curves and their junctions. These latter may be curves with very slight curvatures, or segments of a straight line passing through the set-up point and having lengths no longer than one LSS. Thus one gets a mean curve for the absolute errors; (d) plotting of the actually observed radial positions with respect to the mean curve (results of smoothing)

  5. Radial flow pulse jet mixer

    Science.gov (United States)

    VanOsdol, John G.

    2013-06-25

    The disclosure provides a pulse jet mixing vessel for mixing a plurality of solid particles. The pulse jet mixing vessel is comprised of a sludge basin, a flow surface surrounding the sludge basin, and a downcoming flow annulus between the flow surface and an inner shroud. The pulse jet mixing vessel is additionally comprised of an upper vessel pressurization volume in fluid communication with the downcoming flow annulus, and an inner shroud surge volume separated from the downcoming flow annulus by the inner shroud. When the solid particles are resting on the sludge basin and a fluid such as water is atop the particles and extending into the downcoming flow annulus and the inner shroud surge volume, mixing occurs by pressurization of the upper vessel pressurization volume, generating an inward radial flow over the flow surface and an upwash jet at the center of the sludge basin.

  6. Recursive formula to compute Zernike radial polynomials.

    Science.gov (United States)

    Honarvar Shakibaei, Barmak; Paramesran, Raveendran

    2013-07-15

    In optics, Zernike polynomials are widely used in testing, wavefront sensing, and aberration theory. This unique set of radial polynomials is orthogonal over the unit circle and finite on its boundary. This Letter presents a recursive formula to compute Zernike radial polynomials using a relationship between radial polynomials and Chebyshev polynomials of the second kind. Unlike the previous algorithms, the derived recurrence relation depends neither on the degree nor on the azimuthal order of the radial polynomials. This leads to a reduction in the computational complexity.

  7. Simultaneous multivessel coronary artery spasm demonstrated by quantitative analysis of thallium-201 single photon emission computed tomography

    International Nuclear Information System (INIS)

    Kugiyama, K.; Yasue, H.; Okumura, K.; Minoda, K.; Takaoka, K.; Matsuyama, K.; Kojima, A.; Koga, Y.; Takahashi, M.

    1987-01-01

    Thallium-201 myocardial scintigraphy with quantitative analysis of emission computed tomography was performed during episodes of angina in 19 patients with variant angina and nearly normal coronary arteriographic findings. Eleven patients (group I) were shown by arteriography to have spasm in 2 or more large coronary arteries. Eight patients (group II) had spasm in only 1 coronary artery. In 7 patients in group I, significant diffuse perfusion defects simultaneously appeared in multiple coronary artery regions on the scintigram (group IA). The extent and severity of the perfusion defect as measured by thallium-201 tomography were significantly greater in group IA than in group II (p less than 0.001 and p less than 0.01, respectively). The duration of transient ST-segment elevation during the attack in group IA was significantly longer than in group II (p less than 0.001). The incidence of ventricular arrhythmias, including ventricular tachycardia, or complete atrioventricular block during the anginal attack was significantly higher (p less than 0.05) in group IA than in group II. In all study patients, neither attack nor scintigraphic perfusion defect appeared on the repeat test after oral administration of nifedipine. In conclusion, multivessel coronary artery spasm simultaneously appears and causes the attack in many patients with variant angina and nearly normal coronary arteriographic findings, and myocardial ischemia due to simultaneous multivessel coronary spasm is likely to be more extensive and severe, persist longer and have a higher frequency of potentially dangerous arrhythmias than that due to spasm of only 1 coronary artery

  8. Long-term outcome in children with infantile spasms treated with vigabatrin: a cohort of 180 patients.

    Science.gov (United States)

    Djuric, Milena; Kravljanac, Ruzica; Tadic, Biljana; Mrlješ-Popovic, Nataša; Appleton, Richard E

    2014-12-01

    Evaluation of efficacy of vigabatrin as the first drug in infants with previously untreated infantile spasms (IS) and reporting the long-term outcome. We analyzed a cohort of 180 infants with infantile spasms treated with vigabatrin as the first drug. Following initial evaluation and a 48-h basal period for counting the spasms, vigabatrin was administered using the same protocol in all. After 14 days all infants were assessed for therapeutic response (primary outcome). Psychomotor development was evaluated by a psychologist and neurologist prior to the initiation of treatment and during the follow-up. Seizure outcomes were followed prospectively, by seizure types and epilepsy syndromes. Long-term (secondary) outcomes included neurologic status, occurrence of late epilepsy, and developmental/cognitive status. Vigabatrin terminated the spasms in 101 patients (56.9%) at a mean period of 5 days. Patients with normal psychomotor development prior to the onset of spasms responded best. After follow-up of 2.4 to 18.9 years (mean 10.64; standard deviation [SD] 4.40), 38.1% of responders, treated with vigabatrin, had severe neurologic dysfunction, 42% had epilepsy, and 42.2% had unfavorable intellectual outcome. The group with symptomatic etiology and abnormal neurologic status at presentation demonstrated a significantly worse prognosis and a more unfavorable outcome than cryptogenic or idiopathic cases (85.1% and 81.6% versus 14.9% and 0%-p = 0.001). Idiopathic patients treated with vigabatrin were all intellectually normal, except the youngest patient who had borderline cognitive function. The most important prognostic factors were the underlying etiology and preexisting developmental profile. Long-term outcome in the patients treated with vigabatrin was similar to the outcome in patients treated with adrenocorticotropic hormone (ACTH) or corticosteroids, as reported in earlier studies. The long-term prognosis of idiopathic cases treated with vigabatrin was favorable

  9. Novel European SLC1A4 variant: infantile spasms and population ancestry analysis.

    Science.gov (United States)

    Conroy, Judith; Allen, Nicholas M; Gorman, Kathleen; O'Halloran, Eoghan; Shahwan, Amre; Lynch, Bryan; Lynch, Sally A; Ennis, Sean; King, Mary D

    2016-08-01

    SLC1A4 deficiency is a recently described neurodevelopmental disorder associated with microcephaly, global developmental delay, abnormal myelination, thin corpus callosum and seizures. It has been mainly reported in the Ashkenazi-Jewish population with affected individuals homozygous for the p.Glu256Lys variant. Exome sequencing performed in an Irish proband identified a novel homozygous nonsense SLC1A4 variant [p.Trp453*], confirming a second case of SLC1A4-associated infantile spasms. As this is the first European identified, population ancestry analysis of the Exome Aggregation Consortium database was performed to determine the wider ethnic background of SLC1A4 deficiency carriers. p.Glu256Lys was found in Hispanic and South Asian populations. Other potential disease-causing variants were also identified. Investigation for SLC1A4 deficiency should be performed regardless of ethnicity and extend to include unexplained early-onset epileptic encephalopathy.

  10. Coronary Artery Spasm during Cryoballoon Ablation in a Patient with Atrial Fibrillation.

    Science.gov (United States)

    Yajima, Kazuhiro; Yamase, Yuichiro; Oishi, Hideo; Ikehara, Noriyuki; Asai, Yasushi

    2018-03-15

    The patient was a 63-year-old man with drug-resistant atrial fibrillation who developed coronary spasm during cryoballoon ablation (CBA). CBA was started from the left inferior pulmonary vein. ST elevations in II, III, and aVf, with reciprocal ST depressions in V2-5, occurred in association with chest pain just after balloon rewarming and deflation, and the patient's blood pressure fell to 50 mmHg. Coronary angiography revealed 90% diffuse stenosis from the orifice of segment 1 to segment 4 in the right coronary artery. The stenosis and ST elevations improved after the intracoronary injection of nitroglycerine. Using continuous peripheral intravenous coronary vasodilation, we electrically isolated the other pulmonary veins with CBA without incident.

  11. Incidence of tonic spasms as the initial presentation of pediatric multiple sclerosis in Slovenia.

    Science.gov (United States)

    Bizjak, Neli; Osredkar, Damjan; Meglič, Nuška Pečarič; Benedik, Mirjana Perković

    2017-07-01

    Tonic spasms (TS) are involuntary movement patterns that can present in patients with multiple sclerosis (MS). They have been first described decades ago, but are frequently missed and misdiagnosed, particularly in the pediatric MS patients and if appearing ahead of hallmark neurological signs and symptoms of MS. Slovenia is a country with the population of about 2 million people. In the years from 1992 to 2016, we have treated 57 sequential pediatric patients with MS at our hospital, which is the only tertiary medical institution for treating children with MS in the country. We present the only two MS patients, a 17-year-old girl and a 14-year-old boy, whose first manifestation of MS were TS. This allowed us to estimate the incidence of TS in pediatric MS patients in Slovenia. Copyright © 2017 Elsevier B.V. All rights reserved.

  12. Management of cardiac arrest caused by coronary artery spasm: epinephrine/adrenaline versus nitrates.

    Science.gov (United States)

    Kiss, Gabor; Corre, Olivier; Gueret, Gildas; Nguyen Ba, Vinh; Gilard, Martine; Boschat, Jaques; Arvieux, Charles Chistian

    2009-01-01

    Cardiopulmonary resuscitation guidelines imply the use of epinephrine/adrenaline during cardiopulmonary arrest. However, in cardiac arrest situations resulting from coronary artery spasm (CAS), the use of epinephrine/adrenaline could be deleterious. A 49-year-old patient underwent an emergency coronarography with an attempt to stent the coronary arteries. Radiologic imaging revealed a positive methylergonovine maleate (Methergine, Novartis Pharmaceuticals, East Hanover, NJ) test, with subocclusive CAS in several coronary vessels leading to electromechanical dissociation. Cardiopulmonary resuscitation was performed, and intracoronary boluses of isosorbide dinitrate were given to treat CAS. Epinephrine/adrenaline was not administered during resuscitation. Spontaneous circulation was obtained after cardioversion for ventricular fibrillation, and the patient progressively regained consciousness. Resuscitation guidelines do not specify the use of trinitrate derivatives in cardiac arrest situations caused by CAS. The pros and cons of the use of nitrates and epinephrine/adrenaline during cardiac arrest caused by CAS are analyzed in this case report.

  13. [Clinical characteristics and prognosis analysis of vitamin B6 responsive infantile spasms].

    Science.gov (United States)

    Xue, Jiao; Yang, Zhixian; Wu, Ye; Xiong, Hui; Zhang, Yuehua; Liu, Xiaoyan

    2016-02-01

    To analyze clinical characteristics, treatment and prognosis in a cohort of children with vitamin B6 responsive infantile spasms. Ten patients were diagnosed as vitamin B6 responsive infantile spasms in Peking University First Hospital between January 2012 and May 2015.The clinical manifestations, diagnosis and treatment process, video-electroencephalogram, magnetic resonance imaging (MRI), epilepsy related genes and prognosis were retrospectively analyzed. Of the 10 patients, 5 were male, and 5 were female. Eight of them were normal at birth, and the other 2 patients had intracranial hemorrhage or anoxia.The age of epilepsy onset was from 3.5 to 8.0 months.All patients presented spasms primarily.Interictal electroencephalogram (EEG) showed hypsarrhythmia at seizures onset. MRI showed normal in 8 patients, and subarachnoid hemorrhage or multiple encephalomalacia foci after hemorrhage respectively in the other 2 patients. The results of blood biochemical, cerebrospinal fluid examination and urinary metabolic screening were negative. Epilepsy related genes including ALDH7A1 gene analysis showed wild type in all patients. Two patients were classified as symptomatic and eight might be idiopathic or cryptogenic. The initial dose of vitamin B6 was 10.0 mg/(kg·d). The interval between seizures onset and taking vitamin B6 was 0 to 4.0 months. Seizures disappeared completely within a week after administration of vitamin B6 in 9 patients and in 1.5 months in one patient.Of the 8 patients whose seizures were controlled completely during the follow-up period, 7 patients' EEG recovered within 1.5 to 4.0 months and then continued to be normal. The EEG of the rest of a patient returned to normal, but showed abnormal discharges after stopping taking vitamin B6. Two patients' EEG continued abnormal and seizures recurred due to vitamin B6 withdrawal. At the last follow-up, seizures were controlled in all patients. Drug treatment in one case had stopped. Vitamin B6 was used in 9

  14. Drug-Induced Myocardial Infarction Secondary to Coronary Artery Spasm in Teenagers and Young Adults

    Directory of Open Access Journals (Sweden)

    Menyar Ayman

    2006-01-01

    Full Text Available There is no published registry for drug-induced acute myocardial infarction (AMI with subsequent patent coronary angiogram in teenagers. To highlight the mechanism and impact of drug-induced MI with patent coronary arteries among teenagers who have relatively few coronary risk factors in comparison with older patients, we conducted a review of the literature. In this review most of the pertinent published (English and non-English articles through the Medline, Scopus, Cochrane Database of Systematic Reviews, and EBSCO Host research databases from 1970 to 2005 have been revised. Teenagers and young adults with AMI and subsequent patent coronary angiogram were included. In those cases drug-induced coronary spasm was highlighted. Among 220 articles (>12000 cases related with AMI with normal coronary angiogram, 50 articles (~100 cases reported the role of drug in AMI secondary to coronary artery spasm (CAS. There is no well-conducted trial for AMI secondary to CAS in young adults but only a series of case reports, and the diagnosis in most of cases was based on the clinical and laboratory findings without provocation. CAS was associated with 12 illicit substances in teenagers (i.e., cocaine, marijuana, alcohol, butane, and amphetamine. Smoking is not only the initiative but also might harbor other illicit substances that increase the risk for CAS. Cocaine-associated AMI is the most frequent in various research papers. CAS was reported with 19 types of medications (i.e., over-the-counter, chemotherapy, antimigraine, and antibiotics without strong relation to age. Despite drug-induced AMI being not a common event, attention to smoking and drugs in teenagers and young adults will have major therapeutic and prognostic implications.

  15. Sexual well-being in patients with blepharospasm, spasmodic torticollis and hemifacial spasm: a pilot study

    Directory of Open Access Journals (Sweden)

    Paola Perozzo

    2016-10-01

    Full Text Available Mood, anxiety and other psychological symptoms are common in dystonic patients suffering from blepharospam (BSP and spasmodic torticollis (ST. Since sexual well-being is an important aspect of mental health, here, we investigated whether these patients may also experience a worsening of their sexual life. In particular, quality of sexual life was evaluated in patients suffering from BSP (N=30, ST (N=30, and in a control group of patient with Hemifacial spasm (HFS; N=30, undergoing botulinum toxin type A therapy. A group of 30 age-matched healthy volunteers constituted an additional control group. Patients were evaluated just before the periodic injection of botulinum toxin. Sexual functioning was assessed using the Sexual Functioning Inventory (SFI, a reduced form of the Gollombok Rust Inventory, previously employed in patients with Parkinson’s disease. Depression (Beck Depression Inventory and anxiety (STAI-X1/X2 were also assessed. Results revealed that sexual functioning was significantly affected in patients with BSP, ST, and HFS with respect to healthy controls. Dystonic patients manifested more sexual dysfunction than patients with HFS. Overall, females had a poorer quality of sexual life than males and, among females, women with BSP were the most dysfunctional. Psychological symptoms were present in patients with dystonia, but not in patients with HFS. As discussed in the paper, several factors might be taken into account to explain worse quality of sexual life in patients with dystonia compared to patients with hemifacial spasm. Among them an important role might be played by the central origin of dystonia pathophysiology (i.e. altered activity of cortico-striato-thalamic-cortical circuits. Future investigations are necessary to further explore these preliminary findings, considering that this is the first time that sexual well-being is evaluated in patients with BSP, ST and HFS, and comparable data are not available.

  16. [Clinical observation of acupuncture plus electroacupuncture for hand spasm in stroke patients].

    Science.gov (United States)

    Tian, Meng; Lou, Tianwei; Leng, Jun

    2017-09-12

    To observe the effect difference between acupuncture combined with electroacupuncture (EA) and simple acupuncture for hand spasm in stroke patients. Sixty patients were randomly assigned into an acupuncture group and a combination group, 30 cases in each one. Patients in the two groups were treated with acupuncture at the affected Jianyu (LI 15), Binao (LI 14), Jianliao (TE 14), Quchi (LI 11), Shousanli (LI 10), Waiguan (TE 5), Futu (ST 32), Liangqiu (ST 34), Xuehai (SP 10), Zusanli (ST 36), Shangjuxu (ST 37), Xiajuxu (ST 39), Yinlingquan (SP 9), Sanyinjiao (SP 6), Taixi (KI 3), Taichong (LR 3), and the points at the middle of all the dorsal muscles between metacarpal bones. EA with discontinuous wave was used in the combination group at Waiguan (TE 5) and the middle point of the dorsal muscle between the second and the third metacarpal bones. The treatment was given for 40 min, once a day for 3 courses, five treatment per week, 4 weeks as a course. The modified Ashworth scale (MAS), the Fugl-Meyer finger motor function rating scale and the modified Barthel index (BI) were observed before and after treatment in the two group. The MAS scores after treatment significantly decreased compared with those before treatment in the two groups (both P Fugl-Meyer scores and BI scores after treatment increased in the two groups (all P <0.05), with higher scores in the combination group (both P <0.05). EA combined with acupuncture can more apparently alleviate hand spasm, promote the recovery of hand function, improve the quality of life for stroke patients than simple acupuncture.

  17. Response to second treatment after initial failed treatment in a multicenter prospective infantile spasms cohort

    Science.gov (United States)

    Knupp, Kelly G.; Leister, Erin; Coryell, Jason; Nickels, Katherine C.; Ryan, Nicole; Juarez-Colunga, Elizabeth; Gaillard, William D.; Mytinger, John R.; Berg, Anne T.; Millichap, John; Nordli, Douglas R.; Joshi, Sucheta; Shellhaas, Renée A.; Loddenkemper, Tobias; Dlugos, Dennis; Wirrell, Elaine; Sullivan, Joseph; Hartman, Adam L.; Kossoff, Eric H.; Grinspan, Zachary M.; Hamikawa, Lorie

    2018-01-01

    Objective Infantile spasms (IS) represent a severe epileptic encephalopathy presenting in the first 2 years of life. Recommended first-line therapies (hormonal therapy or vigabatrin) often fail. We evaluated response to second treatment for IS in children in whom the initial therapy failed to produce both clinical remission and electrographic resolution of hypsarhythmia and whether time to treatment was related to outcome. Methods The National Infantile Spasms Consortium established a multicenter, prospective database enrolling infants with new diagnosis of IS. Children were considered nonresponders to first treatment if there was no clinical remission or persistence of hypsarhythmia. Treatment was evaluated as hormonal therapy (adrenocorticotropic hormone [ACTH] or oral corticosteroids), vigabatrin, or “other.” Standard treatments (hormonal and vigabatrin) were compared to all other nonstandard treatments. We compared response rates using chi-square tests and multivariable logistic regression models. Results One hundred eighteen infants were included from 19 centers. Overall response rate to a second treatment was 37% (n = 44). Children who received standard medications with differing mechanisms for first and second treatment had higher response rates than other sequences (27/49 [55%] vs. 17/69 [25%], p < 0.001). Children receiving first treatment within 4 weeks of IS onset had a higher response rate to second treatment than those initially treated later (36/82 [44%] vs. 8/34 [24%], p = 0.040). Significance Greater than one third of children with IS will respond to a second medication. Choosing a standard medication (ACTH, oral corticosteroids, or vigabatrin) that has a different mechanism of action appears to be more effective. Rapid initial treatment increases the likelihood of response to the second treatment. PMID:27615012

  18. Altered Default Mode Network on Resting-State fMRI in Children with Infantile Spasms

    Directory of Open Access Journals (Sweden)

    Ya Wang

    2017-05-01

    Full Text Available Infantile spasms (IS syndrome is an age-dependent epileptic encephalopathy, which occurs in children characterized by spasms, impaired consciousness, and hypsarrhythmia. Abnormalities in default mode network (DMN might contribute to the loss of consciousness during seizures and cognitive deficits in children with IS. The purpose of the present study was to investigate the changes in DMN with functional connectivity (FC and amplitude of low-frequency fluctuation (ALFF, the two methods to discover the potential neuronal underpinnings of IS. The consistency of the two calculate methods of DMN abnormalities in IS patients was also our main focus. To avoid the disturbance of interictal epileptic discharge, our testing was performed within the interictal durations without epileptic discharges. Resting-state fMRI data were collected from 13 patients with IS and 35 sex- and age-matched healthy controls. FC analysis with seed in posterior cingulate cortex (PCC was used to compare the differences between two groups. We chose PCC as the seed region because PCC is the only node in the DMN that directly interacts with virtually all other nodes according to previous studies. Furthermore, the ALFF values within the DMN were also calculated and compared between the two groups. The FC results showed that IS patients exhibited markedly reduced connectivity between posterior seed region and other areas within DMN. In addition, part of the brain areas within the DMN showing significant difference of FC had significantly lower ALFF signal in the patient group than that in the healthy controls. The observed disruption in DMN through the two methods showed that the coherence of brain signal fluctuation in DMN during rest was broken in IS children. Neuronal functional impairment or altered integration in DMN would be one neuroimaging characteristic, which might help us to understand the underlying neural mechanism of IS. Further studies are needed to determine whether

  19. Interictal High Frequency Oscillations in an Animal Model of Infantile Spasms

    Science.gov (United States)

    Frost, James D.; Lee, Chong L.; Le, John T.; Hrachovy, Richard A.; Swann, John W.

    2012-01-01

    While infantile spasms is the most common catastrophic epilepsy of infancy and early-childhood, very little is known about the basic mechanisms responsible for this devastating disorder. In experiments reported here, spasms were induced in rats by the chronic infusion of TTX into the neocortex beginning on postnatal day 10–12. Studies of focal epilepsy suggest that high frequency EEG oscillations (HFOs) occur interictally at sites that are most likely responsible for seizure generation. Thus, our goal was to determine if HFOs occurred and where they occurred in cortex in the TTX model. We also undertook multiunit recordings to begin to analyze the basic mechanisms responsible for HFOs. Our results show that HFOs occur most frequently during hypsarrhythmia and NREM sleep and are most prominent contralateral to the TTX infusion site in the homotopic cortex and anterior to this region in frontal cortex. While HFOs were largest and most frequent in these contralateral regions, they were also commonly recorded synchronously across multiple and widely-spaced recordings sites. The amplitude and spatial distribution of interictal HFOs were found to be very similar to the high frequency bursts seen at seizure onset. However, the latter differed from the interictal events in that the high frequency activity was more intense at seizure onset. Microwire recordings showed that neuronal unit firing increased abruptly with the generation of HFOs. A similar increase in neuronal firing occurred at the onset of the ictal events. Taken together, results suggest that neocortical networks are abnormally excitable, particularly contralateral to TTX infusion, and that these abnormalities are not restricted to small areas of cortex. Multiunit firing coincident with HFOs are fully consistent with a neocortical hyperexcitability hypothesis particularly since they both occur at seizure onset. PMID:22342513

  20. Response to second treatment after initial failed treatment in a multicenter prospective infantile spasms cohort.

    Science.gov (United States)

    Knupp, Kelly G; Leister, Erin; Coryell, Jason; Nickels, Katherine C; Ryan, Nicole; Juarez-Colunga, Elizabeth; Gaillard, William D; Mytinger, John R; Berg, Anne T; Millichap, John; Nordli, Douglas R; Joshi, Sucheta; Shellhaas, Renée A; Loddenkemper, Tobias; Dlugos, Dennis; Wirrell, Elaine; Sullivan, Joseph; Hartman, Adam L; Kossoff, Eric H; Grinspan, Zachary M; Hamikawa, Lorie

    2016-11-01

    Infantile spasms (IS) represent a severe epileptic encephalopathy presenting in the first 2 years of life. Recommended first-line therapies (hormonal therapy or vigabatrin) often fail. We evaluated response to second treatment for IS in children in whom the initial therapy failed to produce both clinical remission and electrographic resolution of hypsarhythmia and whether time to treatment was related to outcome. The National Infantile Spasms Consortium established a multicenter, prospective database enrolling infants with new diagnosis of IS. Children were considered nonresponders to first treatment if there was no clinical remission or persistence of hypsarhythmia. Treatment was evaluated as hormonal therapy (adrenocorticotropic hormone [ACTH] or oral corticosteroids), vigabatrin, or "other." Standard treatments (hormonal and vigabatrin) were compared to all other nonstandard treatments. We compared response rates using chi-square tests and multivariable logistic regression models. One hundred eighteen infants were included from 19 centers. Overall response rate to a second treatment was 37% (n = 44). Children who received standard medications with differing mechanisms for first and second treatment had higher response rates than other sequences (27/49 [55%] vs. 17/69 [25%], p treatment within 4 weeks of IS onset had a higher response rate to second treatment than those initially treated later (36/82 [44%] vs. 8/34 [24%], p = 0.040). Greater than one third of children with IS will respond to a second medication. Choosing a standard medication (ACTH, oral corticosteroids, or vigabatrin) that has a different mechanism of action appears to be more effective. Rapid initial treatment increases the likelihood of response to the second treatment. Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.

  1. Comparison of Standard Catheters Versus Radial Artery-Specific Catheter in Patients Who Underwent Coronary Angiography Through Transradial Access.

    Science.gov (United States)

    Chen, On; Goel, Sunny; Acholonu, Michael; Kulbak, Guy; Verma, Shivani; Travlos, Efstratios; Casazza, Richard; Borgen, Elliot; Malik, Bilal; Friedman, Michael; Moskovits, Norbert; Frankel, Robert; Shani, Jacob; Ayzenberg, Sergey

    2016-08-01

    switched to femoral access (3 in each group) secondary to radial artery spasm. In conclusion, the radial artery-specific catheter was shown to have significantly lower fluoroscopy times but higher failure rates compared with the standard catheters. Copyright © 2016 Elsevier Inc. All rights reserved.

  2. Radial nerve entrapement in osseous tunnel without clinical symptoms

    Directory of Open Access Journals (Sweden)

    Purnima Patni

    2011-01-01

    Full Text Available Entrapment of a nerve in the callus of a healing fracture is not a common entity, but it does exist. The entrapment usually presents without neurological deficit. It is difficult to suspect the radial nerve injury if we need to operate on the same site. We present a case of entrapment of radial nerve in the callus of a supracondylar humerus fracture with cubitus varus deformity. The surgery for correction of the deformity led to the damage of the nerve. In retrospect a careful assessment of the x-rays showed two 3-4 mm diameter holes. Awareness of this finding would have given us sufficient indication of nerve entrapment to prevent this mishap.

  3. Radial bone graft usage for nasal septal reconstruction

    Directory of Open Access Journals (Sweden)

    Yakup Cil

    2011-01-01

    Full Text Available Background: Although various techniques have been described for correction of crooked and saddle nose deformities, these problems are challenging with high recurrence and revision rates. Conventional septal surgery may not be adequate for nose reconstruction in crooked and saddle nose deformities. Materials and Methods: Between December 2005 and October 2009, six patients with crooked nose and five patients with saddle nose deformities underwent corrective surgery in our clinic. All patients were male, and the mean age was 21 years (range, 19-23 years. We used rigid radial bone graft to prevent redeviation and recurrence following corrective nasal septal surgery. Results: The mean follow-up period was 28 months, ranging from 18 to 46 months. Mean operation time was 4 hours (3-4.5. All patients healed uneventfully. None of the patients required secondary surgery. Conclusions: We believe that radial bone grafts offer a long lasting support in treatment of challenging cases with crooked and saddle nose deformities.

  4. Photoelectric Radial Velocities, Paper XIX Additional Spectroscopic ...

    Indian Academy of Sciences (India)

    ian velocity curve that does justice to the measurements, but it cannot be expected to have much predictive power. Key words. Stars: late-type—stars: radial velocities—spectroscopic binaries—orbits. 0. Preamble. The 'Redman K stars' are a lot of seventh-magnitude K stars whose radial velocities were first observed by ...

  5. Concepts of radial and angular kinetic energies

    DEFF Research Database (Denmark)

    Dahl, Jens Peder; Schleich, W.P.

    2002-01-01

    We consider a general central-field system in D dimensions and show that the division of the kinetic energy into radial and angular parts proceeds differently in the wave-function picture and the Weyl-Wigner phase-space picture, Thus, the radial and angular kinetic energies are different quantities...

  6. Combination radial and thrust magnetic bearing

    Science.gov (United States)

    Blumenstock, Kenneth A. (Inventor)

    2002-01-01

    A combination radial and thrust magnetic bearing is disclosed that allows for both radial and thrust axes control of an associated shaft. The combination radial and thrust magnetic bearing comprises a rotor and a stator. The rotor comprises a shaft, and first and second rotor pairs each having respective rotor elements. The stator comprises first and second stator elements and a magnet-sensor disk. In one embodiment, each stator element has a plurality of split-poles and a corresponding plurality of radial force coils and, in another embodiment, each stator element does not require thrust force coils, and radial force coils are replaced by double the plurality of coils serving as an outer member of each split-pole half.

  7. CT findings in patients with infantile epilepsy on ACTH therapy. A report of 17 cases including one case of infantile spasm with subdural hematoma

    Energy Technology Data Exchange (ETDEWEB)

    Watanabe, K. (Nagoya Univ. (Japan). Faculty of Medicine); Hara, K.; Hakamada, A.; Miyazaki, S.

    1981-05-01

    A case of infantile spasms in which subdural hematoma developed after ACTH-Z therapy was reported. The results of CT evaluated before and after the therapy in 17 cases of infantile epilepsy including infantile spasms. Cerebral atrophy due to ACTH-Z therapy was remarkable, especially in the infants under one year old. We should vary careful in employing ACTH-Z therapy for infants of this age.

  8. Role of the interosseous membrane and annular ligament in stabilizing the proximal radial head.

    Science.gov (United States)

    Anderson, Ashley; Werner, Frederick W; Tucci, Emily R; Harley, Brian J

    2015-12-01

    The purpose of our study was to determine the relative contributions of the annular ligament, proximal band, central band, and distal band of the interosseous membrane in preventing dislocation of the proximal radius. In part 1 of the study, 8 forearms were loaded transversely with the forearm intact, and the central band, proximal band, and annular ligament were sequentially sectioned to determine the percentage contribution of each structure in preventing transverse radial displacement. In part 2, 12 forearms were cyclically supinated and pronated while optical sensors measured radial and ulnar motion. Transverse radial head motion was computed as the distal band, central band, and proximal band (and annular ligament) were sequentially sectioned. In part 1, there was no significant difference in the percentage contribution of each structure in preventing radial transverse displacement. In part 2, only after sectioning of the central band did significant radial head displacement occur. Greater displacements occurred in supination than in pronation. Dislocation of the proximal radius occurred in 2 arms after sectioning of all 3 structures. Under pure transverse displacement, the central band, annular ligament, and proximal band equally contributed to stabilizing the radius. However, during forearm rotation, the central band contributed more to radial head stability than the annular ligament and proximal band. Our study contributes to our knowledge of forearm biomechanics, demonstrating the importance of the central band in providing proximal radial head stability. Forceful supination should be avoided after surgical procedures designed to stabilize the radial head. Copyright © 2015 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.

  9. Cri du chat syndrome and complex karyotype in a patient with infantile spasms, hypsarrhythmia, nonketotic hyperglycinemia, and heterotopia.

    Science.gov (United States)

    Tsao, Chang Y; Wenger, Gail D; Bartholomew, Dennis W

    2005-04-15

    Seizures are rarely reported in association with deletion or duplication syndromes of the short arm of chromosome 5, or with chromosome 5 rings. We report on the clinical and cytogenetic findings in a girl with Cri du chat syndrome associated with complex abnormalities in chromosome 5, dysmorphic features, flexor infantile spasms, hypsarrhythmia, nonketotic hyperglycinemia, and heterotopia in her brain. Peripheral blood cytogenetic analysis indicates a mosaic karyotype with de novo deletion of varying amounts of 5p and pericentric inversion of the same chromosome 5. The deleted segment on 5p includes the region implicated in the catlike cry as well as sequences implicated in development of facial dysmorphism and mental retardation. This is the first case with Cri du chat syndrome associated with nonketotic hyperglycinemia, infantile spasms, hypsarrhythmia, and heterotopia. (c) 2005 Wiley-Liss, Inc.

  10. Successful use of botulinum toxin a in intractable, severe muscle spasms in spinal cord injury: A case report

    Directory of Open Access Journals (Sweden)

    Madhuri A Lokapur

    2017-01-01

    Full Text Available Botulinum toxin is a protein produced by Clostridium botulinum, which inhibits muscle contraction by transiently blocking the release of acetylcholine at the neuromuscular junction. At a neuromuscular junction, the toxin inactivates some of the fusion proteins, such as SNAP-25, syntaxin, or synaptobrevin, which are essential for cellular function. This process involves the temporary inhibition of presynaptic acetylcholine release; consequently, its effects are restricted to motor neurons that depend on the cholinergic transmission (muscular plate, gland innervating cells. Injections of botulinum toxin A have been shown to be useful in the treatment of etiologically diverse types of muscle spasms. Ultrasonography (USG has been used as a guide for confirming muscle fasciculations and also is an effective tool for confirming precise needle positioning and correct drug placement. We describe a case of a 25-year-old man with meningomyelocele and paraparesis with painful muscle spasms in bilateral thighs treated by USG-guided botulinum toxin injection.

  11. Radial electric fields for improved tokamak performance

    International Nuclear Information System (INIS)

    Downum, W.B.

    1981-01-01

    The influence of externally-imposed radial electric fields on the fusion energy output, energy multiplication, and alpha-particle ash build-up in a TFTR-sized, fusing tokamak plasma is explored. In an idealized tokamak plasma, an externally-imposed radial electric field leads to plasma rotation, but no charge current flows across the magnetic fields. However, a realistically-low neutral density profile generates a non-zero cross-field conductivity and the species dependence of this conductivity allows the electric field to selectively alter radial particle transport

  12. Revascularização do miocárdio com enxerto livre de artéria radial: experiência inicial

    Directory of Open Access Journals (Sweden)

    Giorgio PIERACCIANI

    1997-07-01

    patient (2 to 5, were performed: in 9 patients (45% to the diagonal branch, in 6 (30% to the obtuse marginal branch, in 4 (20% to the right coronary artery and 1(5% to the left anterior descending artery. The left internal mammary artery (LIMA was also used in every patient, the right IMA in 7 (35% and additional saphenous vein grafts in another 13 (65%. Calcium channel blockers were used orally to prevent RA spasm. There were no hospital deaths nor during follow up of 10 months. Seventeen immediate coronary angiographies have been performed. RA grafts were patent in 16 (94.1%. There was no ischemic sequelae of the superior limb secondary to graft harvesting. Our results are similar to many other recent published series, and no morbidity or mortality increase has been noticed with RA used for Coronary artery bypass grafting.

  13. Ineffectiveness of topiramate and levetiracetam in infantile spasms non-responsive to steroids. Open labeled randomized prospective study.

    Science.gov (United States)

    Mahmoud, Adel A; Rizk, Tamer M; Mansy, Ahmed A; Ali, Jaffar A; Al-Tannir, Mohamad A

    2013-04-01

    To compare the effectiveness of 2 novel antiepileptic drugs, topiramate and levetiracetam, as a second line treatment for infantile spasm when oral steroids fail. Forty infants under 2 years with clinically- and EEG-proven infantile spasms that did not respond to prednisone (2mg/kg/day in 2 divided doses) were recruited and randomized into 2 groups. They were randomly assigned to either topiramate (group 1; 1mg/kg/day for 3 days then increased by 1mg/kg/day every third day up to 6mg/kg/day) or levetiracetam (group 2; 10mg/kg/day for 5 days and then increased by 10mg/kg/day every 5 days up to 60mg/kg/day). The study was conducted in the Pediatric Neurology Department at the National Neuroscience Institute of King Fahad Medical City, Riyadh, Kingdom of Saudi Arabia between January 2008 and December 2010. Of the 20 patients included in the final data analysis, 11 (55%) were administered topiramate and 9 (45%) levetiracetam. Eighteen patients did not respond to the first drug, and subsequently to the other drug when crossed-over. Two patients with infantile spasm responded to either one drug without crossover. Their EEGs improved with time. The present study demonstrated the ineffectiveness of topiramate and levetiracetam suggesting current treatment modalities are grossly inadequate underscoring the urgent need for more research efforts to overcome current deficiencies. Two patients with cryptogenic infantile spasm responded to treatment suggesting the potential for treatment of such patients with these 2 drugs, and merits further multicenter investigation.

  14. Radial diagnostics in the system of ecological monitoring in trauma

    Science.gov (United States)

    Siniakova, Olga G.; Ishmuhametov, Airat I.; Proscurina, Gulnar B.; Sharifullin, Faad A.

    1997-08-01

    Both creating of effective identification and evaluation mechanisms of environmental factors hazardous for health, and revealing their influence degree on the human health play an important role in ecological monitoring. The grate importance in a solution of many ecological problems belongs to medicine, first of all, to its social-preventive brunch. In this reference trauma remains the extremely important problem. Annually more than 10 million persons sustain traumas. Alongside with occupational, transport trauma, a significant number of trauma cases occur due to the impact of various ecological factors, including natural disasters, mass poisonings and other reasons. Trauma results in severe changes in human body organs and systems; the timely detection and correct evaluation of these changes are the key points for the choice of treatment. Among diagnostic methods used for this purpose, the methods of radial diagnostics play an important role. Various radial methods-- x-ray, radionuclide, ultrasonic, magnetic resonance imaging, computer tomography (CT)--are used to detect the functional and structural changes of vital organs and systems in trauma. Each of these methods has its advantages and shortages. The reported study was devoted to the analysis of using the photon systems (gamma-camera and computer tomography) in application of radionuclide and CT methods of radial diagnostics in trauma.

  15. [AICA anatomic variation as a factor of worse prognosis for the surgical treatment of hemi-facial spasm].

    Science.gov (United States)

    Reizinho, Carla; Casimiro, Miguel; Luís, Ana; Dominguez, Manuel

    2013-01-01

    Hemifacial spasm is a neurovascular compression syndrome. These consist in a contacting vessel (most often an artery) to a cranial nerve in cerebelar-pontine angle. The most common is trigeminal neuralgia caused by contact between the superior cerebellar artery and the trigeminal nerve, and less commonly hemifacial spasm, vertiginous syndrome by contact of the antero inferior cerebelar artery with the eighth cranial nerve, glossopharyngeal neuralgia by contact of the postero inferior cerebelar artery and the IX cranial nerve, etc. These syndromes typically occur after the fifth decade of life, when the arterial tortuosity increases due to the arteriosclerosis process. They are however associated anatomical variations of the origin and course of the arteries, which facilitate contact with the nerves of the cerebellar-pontine angle. In hemifacial spasm, the vessel most often related is antero inferior cerebelar and the authors describe a case of a rare anatomical variant in the course of the artery that motivated the development of the disease, which was identified intraoperatively on a surgical approach to the cerebellar-pontine for vascular microdescompression.

  16. Cranial computed tomography in infantile spasms. Findings related to etiology, treatment and long-term developmental outcome

    Energy Technology Data Exchange (ETDEWEB)

    Tsukahara, Mitsuaki [Iwate Medical Univ., Morioka (Japan). School of Medicine

    1995-12-01

    Cranial computed tomographic (CT) scans obtained from 40 pediatric patients with infantile spasms were assessed in relation to the presumed etiology, ACTH therapy, and long-term mental outcome. Fifty-five percent (22/40) of cases had abnormal findings on CT scans. The major structural changes on CT scans were generalized brain atrophy (type I with 5 subtypes) and ependymal calcifications (type II). A wide diversity of etiologic factors including structural anomalies, tuberous sclerosis, and perinatal and postnatal brain damage were identified. Six patients, on whom serial CT-scannings were performed before and after ACTH therapy, had worsening of cerebral atrophy which was reversible in all but one patient after stopping the ACTH therapy. No clear correlation was found between findings on CT scans and seizure outcome. Ninety-two percent of the cases showed some degree of mental and developmental retardation; 67 percent (12/18) of the patients with severe mental retardation had abnormal findings on CT scans. CT-scanning of the brain was found to be a valuable tool for the examination of the underlying structural abnormalities in children with infantile spasms. The data presented may also be useful for the design and interpretation of therapeutic drug trials in patients with infantile spasms. (author).

  17. Effects of steroids therapy and ACTH therapy on the central nervous system evaluated by CT. Nephrotic syndrome and infantile spasms

    Energy Technology Data Exchange (ETDEWEB)

    Xano, E.; Yamashita, F.; Aoki, N.i; Matsuishi, T. (Kurume Univ., Fukuoka (Japan). School of Medicine); Yamamoto, M.

    1981-05-01

    CT scanning revealed cerebral atrophy of various degrees in 12 of 15 cases of nephrotic syndrome in which ACTH therapy was given and also in all the 8 cases of infantile spasms in which ACTH therapy was given. CT findings of cerebral atrophy were analysed by computor and expressed in terms of cerebrospinal fluid (CSF)-space/intracranial space ratio (%). The ratio was 6.6 +- 3.9% in nephrotic syndrome, 16.4 +- 7.8% in infantile spasms, and 1.6 +- 0.8% in control, showing statistically significant differences among them. Abnormal CT findings of 12 cases of nephrotic syndrome returned to normal after withdrawal of steroids in 9 cases (75%). CT findings in 8 cases of infantile spasms also improved in all the cases after withdrawal of ACTH therapy. The results suggested that cerebral atrophy on CT scan is a reversible change. However, the effect of steroids and ACTH on the central nervous system is not sufficiently known yet. We should be very careful about long-term administration of great amount of steroids or ACTH to infants and children.

  18. Scaling laws for radial foil bearings

    Science.gov (United States)

    Honavara Prasad, Srikanth

    The effects of fluid pressurization, structural deformation of the compliant members and heat generation in foil bearings make the design and analysis of foil bearings very complicated. The complex fluid-structural-thermal interactions in foil bearings also make modeling efforts challenging because these phenomena are governed by highly non-linear partial differential equations. Consequently, comparison of various bearing designs require detailed calculation of the flow fields (velocities, pressures), bump deflections (structural compliance) and heat transfer phenomena (viscous dissipation in the fluid, frictional heating, temperature profile etc.,) resulting in extensive computational effort (time/hardware). To obviate rigorous computations and aid in feasibility assessments of foil bearings of various sizes, NASA developed the "rule of thumb" design guidelines for estimation of journal bearing load capacity. The guidelines are based on extensive experimental data. The goal of the current work is the development of scaling laws for radial foil bearings to establish an analytical "rule of thumb" for bearing clearance and bump stiffness. The use of scale invariant Reynolds equation and experimentally observed NASA "rule of thumb" yield scale factors which can be deduced from first principles. Power-law relationships between: a. Bearing clearance and bearing radius, and b. bump stiffness and bearing radius, are obtained. The clearance and bump stiffness values obtained from scaling laws are used as inputs for Orbit simulation to study various cases. As the clearance of the bearing reaches the dimensions of the material surface roughness, asperity contact breaks the fluid film which results in wear. Similarly, as the rotor diameter increases (requiring larger bearing diameters), the load capacity of the fluid film should increase to prevent dry rubbing. This imposes limits on the size of the rotor diameter and consequently bearing diameter. Therefore, this thesis aims

  19. TRUE MASSES OF RADIAL-VELOCITY EXOPLANETS

    Energy Technology Data Exchange (ETDEWEB)

    Brown, Robert A., E-mail: rbrown@stsci.edu [Space Telescope Science Institute (United States)

    2015-06-01

    We study the task of estimating the true masses of known radial-velocity (RV) exoplanets by means of direct astrometry on coronagraphic images to measure the apparent separation between exoplanet and host star. Initially, we assume perfect knowledge of the RV orbital parameters and that all errors are due to photon statistics. We construct design reference missions for four missions currently under study at NASA: EXO-S and WFIRST-S, with external star shades for starlight suppression, EXO-C and WFIRST-C, with internal coronagraphs. These DRMs reveal extreme scheduling constraints due to the combination of solar and anti-solar pointing restrictions, photometric and obscurational completeness, image blurring due to orbital motion, and the “nodal effect,” which is the independence of apparent separation and inclination when the planet crosses the plane of the sky through the host star. Next, we address the issue of nonzero uncertainties in RV orbital parameters by investigating their impact on the observations of 21 single-planet systems. Except for two—GJ 676 A b and 16 Cyg B b, which are observable only by the star-shade missions—we find that current uncertainties in orbital parameters generally prevent accurate, unbiased estimation of true planetary mass. For the coronagraphs, WFIRST-C and EXO-C, the most likely number of good estimators of true mass is currently zero. For the star shades, EXO-S and WFIRST-S, the most likely numbers of good estimators are three and four, respectively, including GJ 676 A b and 16 Cyg B b. We expect that uncertain orbital elements currently undermine all potential programs of direct imaging and spectroscopy of RV exoplanets.

  20. The many radial access learning curves.

    Science.gov (United States)

    Hillegass, William B

    2017-04-01

    The radial approach to endovascular procedures has a series of learning curves: diagnostic heart catheterization, low-risk settings and "straightforward" percutaneous coronary intervention, high-risk settings, and complex coronary intervention, and peripheral vascular angiography and intervention. For diagnostic and low-risk interventional procedures, incremental improvements in technical success and safety are observed in the initial 200 procedures for most operators compared to highly experienced operators. Formal didactic training and ongoing support/review from an experienced radial operator(s) may expedite surmounting the series of radial learning curves while maintaining optimal procedural success and safety. Advances in technology and understanding will require the most experienced radial operators to continually embrace their next learning curve. © 2017 Wiley Periodicals, Inc.

  1. Radial Velocity Fluctuations of RZ Psc

    Science.gov (United States)

    Potravnov, I. S.; Gorynya, N. A.; Grinin, V. P.; Minikulov, N. Kh.

    2014-12-01

    The behavior of the radial velocity of the UX Ori type star RZ Psc is studied. The existence of an inner cavity with a radius of about 0.7 a.u. in the circumstellar disk of this star allows to suggest the presence of a companion. A study of the radial velocity of RZ Psc based on our own measurements and published data yields no periodic component in its variability. The two most accurate measurements of V r , based on high resolution spectra obtained over a period of three months, show that the radial velocity is constant over this time interval to within 0.5 km/s. This imposes a limit of M p ≤10 M Jup on the mass of the hypothetical companion. Possible reasons for the observed strong fluctuations in the radial velocity of this star are discussed.

  2. Radial pulsations in DB white dwarfs?

    Science.gov (United States)

    Kawaler, Steven D.

    1993-01-01

    Theoretical models of DB white dwarfs are unstable against radial pulsation at effective temperatures near 20,000-30,000 K. Many high-overtone modes are unstable, with periods ranging from 12 s down to the acoustic cutoff period of approximately 0.1 s. The blue edge for radial instability lies at slightly higher effective temperatures than for nonradial pulsations, with the temperature of the blue edge dependent on the assumed efficiency of convection. Models with increased convective efficiency have radial blue edges that are increasingly closer to the nonradial blue edge; in all models the instability persists into the nonradial instability strip. Radial pulsations therefore may exist in the hottest DB stars that lie below the DB gap; the greatest chance for detection would be observations in the ultraviolet. These models also explain why searches for radial pulsations in DA white dwarfs have failed: the efficient convection needed to explain the blue edge for nonradial DA pulsation means that the radial instability strip is 1000 K cooler than found in previous investigations. The multiperiodic nature of the expected pulsations can be used to advantage to identify very low amplitude modes using the uniform spacing of the modes in frequency. This frequency spacing is a direct indicator of the mass of the star.

  3. Association of Live Donor Nephrectomy and Reversal of Renal Artery Spasm

    Directory of Open Access Journals (Sweden)

    Jalal Azmandian

    2014-01-01

    Full Text Available Background: Kidney transplantation is the best treatment option for kidney failure. Major medical progress has been made in the field of renal transplantation over the last 40 years. The surgical procedure has been standardized and the complication rate is low. Overall, the outcome of renal transplantation is excellent and has improved over time. Vascular complications after renal transplantation are the most frequent type of complication following urological complications. Renal artery spasm (RAS following manipulation of renal artery is a common problem during live donor nephrectomy (LDN. The aim of this study was to determine whether or not it is necessary to wait for reverse of RAS and resumption of urinary flow before nephrectomy. Materials and Methods: In this clinical trial 16 cases of LDN who developed RAS during surgery received intra-arterial injection of 40 mg papaverine. In 8 cases surgery continued towards nephrectomy and in other 8 cases we waited for reverse of RAS. All analyses were performed using SPSS-11. Results: In both groups urinary flow started a few minutes (Mean, 12 min after declamping of transplanted kidney and normal renal consistency and color were achieved. There was no significant difference between urinary volume during 12 h after transplantation in two groups. Conclusion: The results showed that it might not be necessary to wait for reverse of RAS before LDN. Both patient (less anesthesia complications and hospital (less expenses will benefit from this time saving.

  4. Diagnosis, treatment, and outcomes of infantile spasms in the Trisomy 21 population.

    Science.gov (United States)

    Beatty, Christopher W; Wrede, Joanna E; Blume, Heidi K

    2017-02-01

    To determine if there are differences in the timing of diagnosis and response to treatment between infants with infantile spasms (IS) and Trisomy 21 (T21) and those with idiopathic IS. This was a retrospective study evaluating the time from onset of IS to diagnosis, treatment of IS, time from treatment to resolution of IS, and development of epilepsy in children with T21 and IS compared to children with idiopathic IS. Thirteen children with T21 and IS were identified over a 10 year period and compared to 32 children in the control group. There was no significant difference in age of onset, time between onset and diagnosis, or acute response to treatment. However, the children with idiopathic IS were more likely to go on to develop epilepsy than those with T21 and IS (41% vs. 0, p=0.006). The children with T21 and IS were diagnosed and treated similarly to those patients with idiopathic IS. There were no significant differences in the age of onset, time between the onset and diagnosis of IS, or acute treatment response of IS between the T21 and control groups. However those with T21 and IS had a lower risk of subsequent epilepsy following IS than those with idiopathic IS. IS in the T21 population appears to be inherently different from IS of unknown etiology. Copyright © 2017 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  5. Developing an animal model for infantile spasms: pathogenesis, problems and progress

    Science.gov (United States)

    Marsh, Eric D.; Golden, Jeffrey A.

    2009-01-01

    Infantile spasms (IS), the most common of the early epileptic encephalopathies, afflicts thousands of children each year and results in significant disability. Also known as West syndrome, IS is characterized by intractable stereotyped seizures, poor developmental outcome and a characteristic electroencephalogram (EEG) pattern. IS often progresses into another epileptic encephalopathy known as Lennox-Gastaut syndrome, and continues with the patient being burdened by lifelong epilepsy and varying degrees of mental retardation. Little is known about the biological basis of IS. As the etiologies of IS are diverse, the multiple causes must converge into a final common pathway that results in this specific epilepsy phenotype. Finding a model or models to test this final pathway is necessary both to understand why the greatest susceptibility to seizure development occurs during infancy and early childhood, and what underlies the decreased cognitive potential associated with IS. Furthermore, appropriate models would permit better testing of potential therapies directed specifically at IS. This review will describe the clinical features and etiologies of IS; the ideal features that IS models should contain; and the IS models that exist currently. Finally, we will discuss the limitations of these models and the potential avenues for future research on IS. PMID:19553693

  6. Radial artery occlusion after transradial approach to cardiac catheterization.

    Science.gov (United States)

    Wagener, John F; Rao, Sunil V

    2015-03-01

    Radial artery occlusion (RAO) is the most common complication of the transradial approach (TRA) to cardiac catheterization, with a reported incidence between 0.8 % and 30 %. RAO is likely the result of acute thrombus formation and complicated by neointimal hyperplasia. Most RAO are asymptomatic with rare cases of acute hand or digit ischemia reported in the literature. The role of testing for dual circulation to the hand in determining the safety of TRA as it relates to symptomatic RAO is controversial; however, modifiable risk factors like low sheath-to-artery ratio, adequate anticoagulation, and non-occlusive ("patent") hemostasis are likely to prevent RAO. This review examines the incidence of RAO, potential mechanisms leading to RAO, and strategies to prevent and treat RAO.

  7. Anomalies of radial and ulnar arteries

    Directory of Open Access Journals (Sweden)

    Rajani Singh

    Full Text Available Abstract During dissection conducted in an anatomy department of the right upper limb of the cadaver of a 70-year-old male, both origin and course of the radial and ulnar arteries were found to be anomalous. After descending 5.5 cm from the lower border of the teres major, the brachial artery anomalously bifurcated into a radial artery medially and an ulnar artery laterally. In the arm, the ulnar artery lay lateral to the median nerve. It followed a normal course in the forearm. The radial artery was medial to the median nerve in the arm and then, at the level of the medial epicondyle, it crossed from the medial to the lateral side of the forearm, superficial to the flexor muscles. The course of the radial artery was superficial and tortuous throughout the arm and forearm. The variations of radial and ulnar arteries described above were associated with anomalous formation and course of the median nerve in the arm. Knowledge of neurovascular anomalies are important for vascular surgeons and radiologists.

  8. Non-invasive, multimodal analysis of cortical activity, blood volume and neurovascular coupling in infantile spasms using EEG-fNIRS monitoring.

    Science.gov (United States)

    Bourel-Ponchel, Emilie; Mahmoudzadeh, Mahdi; Delignières, Aline; Berquin, Patrick; Wallois, Fabrice

    2017-01-01

    Although infantile spasms can be caused by a variety of etiologies, the clinical features are stereotypical. The neuronal and vascular mechanisms that contribute to the emergence of infantile spasms are not well understood. We performed a multimodal study by simultaneously recording electroencephalogram and functional Near-infrared spectroscopy in an intentionally heterogeneous population of six children with spasms in clusters. Regardless of the etiology, spasms were accompanied by two phases of hemodynamic changes; an initial change in the cerebral blood volume (simultaneously with each spasm) followed by a neurovascular coupling in all children except for the one with a large porencephalic cyst. Changes in cerebral blood volume, like the neurovascular coupling, occurred over frontal areas in all patients regardless of any brain damage suggesting a diffuse hemodynamic cortical response. The simultaneous motor activation and changes in cerebral blood volume might result from the involvement of the brainstem. The inconstant neurovascular coupling phase suggests a diffuse activation of the brain likely resulting too from the brainstem involvement that might trigger diffuse changes in cortical excitability.

  9. Physical mechanism determining the radial electric field and its radial structure in a toroidal plasma

    International Nuclear Information System (INIS)

    Ida, Katsumi; Miura, Yukitoshi; Itoh, Sanae

    1994-10-01

    Radial structures of plasma rotation and radial electric field are experimentally studied in tokamak, heliotron/torsatron and stellarator devices. The perpendicular and parallel viscosities are measured. The parallel viscosity, which is dominant in determining the toroidal velocity in heliotron/torsatron and stellarator devices, is found to be neoclassical. On the other hand, the perpendicular viscosity, which is dominant in dictating the toroidal rotation in tokamaks, is anomalous. Even without external momentum input, both a plasma rotation and a radial electric field exist in tokamaks and heliotrons/torsatrons. The observed profiles of the radial electric field do not agree with the theoretical prediction based on neoclassical transport. This is mainly due to the existence of anomalous perpendicular viscosity. The shear of the radial electric field improves particle and heat transport both in bulk and edge plasma regimes of tokamaks. (author) 95 refs

  10. Statistical analysis of radial interface growth

    International Nuclear Information System (INIS)

    Masoudi, A A; Hosseinabadi, S; Khorrami, M; Davoudi, J; Kohandel, M

    2012-01-01

    Recent studies have questioned the application of standard scaling analysis to study radially growing interfaces (Escudero 2008 Phys. Rev. Lett. 100 116101; 2009 Ann. Phys. 324 1796). We show that the radial Edwards–Wilkinson (EW) equation belongs to the same universality as that obtained in the planar geometry. In addition, we use numerical simulations to calculate the interface width for both random deposition with surface relaxation (RDSR) and restricted solid on solid (RSOS) models, assuming that the system size increases linearly with time (due to radial geometry). By applying appropriate rules for each model, we show that the interface width increases with time as t β , where the exponent β is the same as those obtained from the corresponding planar geometries. (letter)

  11. Radial anisotropy ambient noise tomography of volcanoes

    Science.gov (United States)

    Mordret, Aurélien; Rivet, Diane; Shapiro, Nikolai; Jaxybulatov, Kairly; Landès, Matthieu; Koulakov, Ivan; Sens-Schönfelder, Christoph

    2016-04-01

    The use of ambient seismic noise allows us to perform surface-wave tomography of targets which could hardly be imaged by other means. The frequencies involved (~ 0.5 - 20 s), somewhere in between active seismic and regular teleseismic frequency band, make possible the high resolution imaging of intermediate-size targets like volcanic edifices. Moreover, the joint inversion of Rayleigh and Love waves dispersion curves extracted from noise correlations allows us to invert for crustal radial anisotropy. We present here the two first studies of radial anisotropy on volcanoes by showing results from Lake Toba Caldera, a super-volcano in Indonesia, and from Piton de la Fournaise volcano, a hot-spot effusive volcano on the Réunion Island (Indian Ocean). We will see how radial anisotropy can be used to infer the main fabric within a magmatic system and, consequently, its dominant type of intrusion.

  12. Carpal alignment in distal radial fractures

    Directory of Open Access Journals (Sweden)

    Jain Pankaj

    2002-05-01

    Full Text Available Abstract Background Carpal malalignment following the malunited distal radial fracture is described to develop as an adaptation to realign the hand to the malunion. It worsens gradually after healing of the fracture due to continued loading of the wrist. It is also reported to develop during the immobilization itself rather than after fracture healing. The present work was aimed to study the natural course and the quantitative assessment of such adaptive carpal realignment following distal radial fracture. Methods In a prospective study, 118 distal radial fractures treated with different modalities were followed-up with serial radiographs for a year for assessment of various radiological parameters. Results Two patterns of carpal malalignment were identified depending upon the effective radio-lunate flexion (ERLF measured on pre-reduction radiographs. The midcarpal malalignment was seen in 98 radial fractures (83% with the lunate following the dorsiflexed fracture fragment and a measured ERLF of less than 25°. The second pattern of radio-carpal malalignment showed the fracture fragment to dorsiflex without taking the lunate with a measured ERLF of more than 25°. The scaphoid did not follow the fracture fragment in both the patterns of malalignment. Conclusion It is better to assess distal radial fractures for any wrist ligamentous injury on the post-reduction film with the restored radial anatomy than on the pre-reduction film since most carpal malalignments get corrected with the reduction of the fracture. Similar carpal malalignment reappear with the redisplacement of the fracture as seen in pre-reduction radiographs and develops during the immobilization rather than as a later compensatory mechanism for the malunion.

  13. Differentiating non-motor symptoms in Parkinson's disease from controls and hemifacial spasm.

    Directory of Open Access Journals (Sweden)

    Ming-Hui Yong

    Full Text Available BACKGROUND AND AIMS: Non-motor symptoms (NMS are important manifestations of Parkinson's disease (PD that reduce patients' health-related quality of life. Some NMS may also be caused by age-related changes, or manifested as a psychological reaction to a chronic neurological condition. This case-control study compared the NMS burden among PD patients, healthy controls and hemifacial spasm (HFS patients. In addition, we determined the NMS that discriminated between PD and non-PD subjects. METHODS: 425 subjects were recruited from a tertiary hospital in Singapore (200 PD patients, 150 healthy controls and 75 HFS patients. NMS burden in subjects was measured using the Non-Motor Symptoms Scale (NMSS. RESULTS: NMSS total score was significantly higher in PD patients (37.9±2.6 compared to healthy controls (11.2±0.9 (p<0.0001 and HFS patients (18.0±2.1 (p<0.0001. In addition, NMSS total score was significantly higher in HFS patients compared to healthy controls (p = 0.003. PD patients experienced a higher NMS burden than healthy controls in all domains, and a higher NMS burden than HFS patients in all but attention/memory and urinary domains. NMS burden for HFS and healthy controls differed only in the sleep/fatigue and urinary domains. Using stepwise logistic regression, problems of 'constipation', 'restless legs', 'dribbling saliva', 'altered interest in sex' and 'change in taste or smell' were found to have significant discriminative power in differentiating between PD patients and healthy controls and between PD patients and HFS patients. CONCLUSION: PD patients experienced a greater overall NMS burden compared to both healthy controls and HFS patients. HFS patients demonstrated a higher NMS burden than controls, and some NMS may be common to chronic neurological conditions while others are more specific to PD. Differentiating patients using NMS domains may help refine the clinical management of NMS in PD patients.

  14. Modified Ashworth scale and spasm frequency score in spinal cord injury: reliability and correlation.

    Science.gov (United States)

    Baunsgaard, C B; Nissen, U V; Christensen, K B; Biering-Sørensen, F

    2016-09-01

    Intra- and inter-rater reliability study. To assess intra- and inter-rater reliability of the Modified Ashworth Scale (MAS) and Spasm Frequency Score (SFS) in lower extremities in a population of spinal cord-injured persons, as well as correlations between the two scales. Clinic for Spinal Cord Injuries, Rigshospitalet, Hornbaek, Denmark. Thirty-one persons participated in the study and were tested four times in total with MAS and SFS by three experienced raters. Cohen's kappa (κ), simple and quadratic weighted (nominal and ordinal scale level of measurement), was used as a measure of reliability and Spearman's rank correlation coefficient for correlation between MAS and SFS. Neurological level ranged from C2 to L2 and American Spinal Injury Association impairment scale A to D. Time since injury was (mean±s.d.) 3.4±6.5 years. Age was 48.3±20.2 years. Cause of injury was traumatic in 55% and non-traumatic for 45% of the participants. Antispastic medication was used by 61%. MAS showed intra-rater κsimple=-0.11 to 0.46 and κweighted=-0.11 to 0.83. Inter-rater κsimple=-0.06 to 0.32 and κweighted=0.08 to 0.74. SFS showed intra-rater κweighted=0.94 and inter-rater κweighted=0.93. Correlation between MAS and SFS showed non-significant correlation coefficients from-0.11 to 0.90. Reliability of MAS is highly affected by the weighting scheme. With a weighted-κ it was overall reliable and simple-κ overall unreliability. Repeated tests should always be performed by the same rater and in a very standardized manner. SFS was found reliable. MAS and SFS are poorly correlated, and ratings were inversely distributed and suggest that it assesses different aspects of spasticity.

  15. Multimodal Image-Based Virtual Reality Presurgical Simulation and Evaluation for Trigeminal Neuralgia and Hemifacial Spasm.

    Science.gov (United States)

    Yao, Shujing; Zhang, Jiashu; Zhao, Yining; Hou, Yuanzheng; Xu, Xinghua; Zhang, Zhizhong; Kikinis, Ron; Chen, Xiaolei

    2018-02-21

    To address the feasibility and predictive value of multimodal image-based virtual reality in detecting and assessing features of neurovascular confliction (NVC), particularly regarding the detection of offending vessels, degree of compression exerted on the nerve root, in patients who underwent microvascular decompression for nonlesional trigeminal neuralgia and hemifacial spasm (HFS). This prospective study includes 42 consecutive patients who underwent microvascular decompression for classic primary trigeminal neuralgia or HFS. All patients underwent preoperative 1.5-T magnetic resonance imaging (MRI) with T2-weighted three-dimensional (3D) sampling perfection with application-optimized contrasts by using different flip angle evolutions, 3D time-of-flight magnetic resonance angiography, and 3D T1-weighted gadolinium-enhanced sequences in combination, whereas 2 patients underwent extra experimental preoperative 7.0-T MRI scans with the same imaging protocol. Multimodal MRIs were then coregistered with open-source software 3D Slicer, followed by 3D image reconstruction to generate virtual reality (VR) images for detection of possible NVC in the cerebellopontine angle. Evaluations were performed by 2 reviewers and compared with the intraoperative findings. For detection of NVC, multimodal image-based VR sensitivity was 97.6% (40/41) and specificity was 100% (1/1). Compared with the intraoperative findings, the κ coefficients for predicting the offending vessel and the degree of compression were >0.75 (P impact on detection of small-caliber offending vessels with relatively slow flow speed in cases of HFS. Multimodal image-based VR using 3D sampling perfection with application-optimized contrasts by using different flip angle evolutions in combination with 3D time-of-flight magnetic resonance angiography sequences proved to be reliable in detecting NVC and in predicting the degree of root compression. The VR image-based simulation correlated well with the real

  16. Epileptic spasms in clusters and associated syndromes other than West syndrome: A study of 48 patients.

    Science.gov (United States)

    Caraballo, Roberto H; Fortini, Sebastian; Reyes, Gabriela; Carpio Ruiz, Aliria; Sanchez Fuentes, Salvador Vazquez; Ramos, Belen

    2016-07-01

    To study the different epilepsy syndromes that included epileptic spasms (ES) in clusters without hypsarrhythmia (WoH). Between 2/1990 and 7/2013, we registered 48 patients with the electroclinical diagnostic criteria of ES in clusters WoH. We recognized two subgroups. In the first subgroup of 30 patients, ES started at a mean age of 10.6 months (range, 2-40 months). Ictal EEG recordings showed diffuse high-amplitude slow waves in 15 patients, diffuse slow waves followed by voltage attenuation in six patients, diffuse fast rhythms in five, diffuse slow waves with superimposed fast rhythms in three, and diffuse sharp waves in one. In the second subgroup of 18 patients, nine had electroclinical features of Lennox-Gastaut syndrome, four had epilepsy with myoclonic and atonic seizures, two had Dravet syndrome, one 6-year-old boy had a non-convulsive status epilepticus characterized by atypical absences associated with ES, one had epilepsy with migrating seizures of infancy, and one patient had clinical features of subacute sclerosing panencephalitis with ES. ES started at a mean age of 6.3 years (range, 0.5-13 years). The ictal EEG recording during the ES showed diffuse fast rhythms in 10 cases, diffuse slow waves with superimposed fast rhythms in four, and diffuse slow waves in four. Our study shows two subgroups of children with ESWoH. The first subgroup had a well-defined electroclinical syndrome predominantly in infancy, and in the second subgroup ES was one more seizure type associated with an epileptic encephalopathy other than West syndrome predominantly occurring in childhood. Copyright © 2016 Elsevier B.V. All rights reserved.

  17. Infantile spasms (West syndrome: update and resources for pediatricians and providers to share with parents

    Directory of Open Access Journals (Sweden)

    Wheless James W

    2012-07-01

    Full Text Available Abstract Background Infantile spasms (IS; West syndrome is a severe form of encephalopathy that typically affects infants younger than 2 years old. Pediatricians, pediatric neurologists, and other pediatric health care providers are all potentially key early contacts for families who have an infant with IS. The objective of this article is to assist pediatric health care providers in the detection of the disease and in the counseling and guidance of families who have an infant with IS. Methods Treatment guidelines, consensus reports, and original research studies are reviewed to provide an update regarding the diagnosis and treatment of infants with IS. Web sites were searched for educational and supportive resource content relevant to providers and families of patients with IS. Results Early detection of IS and pediatrician referral to a pediatric neurologist for further evaluation and initiation of treatment may improve prognosis. Family education and the establishment of a multidisciplinary continuum of care are important components of care for the majority of patients with IS. The focus of the continuum of care varies across diagnosis, initiation of treatment, and short- and long-term needs. Several on-line educational and supportive resources for families and caregivers of patients with IS were identified. Conclusions Given the possibility of poor developmental outcomes in IS, including the emergence of other seizure disorders and cognitive and developmental problems, early recognition, referral, and treatment of IS are important for optimal patient outcomes. Dissemination of and access to educational and supportive resources for families and caregivers across the lifespan of the child with IS is an urgent need. Pediatric health care providers are well positioned to address these needs.

  18. Magnetic resonance imaging of vascular compression in trigeminal neuralgia and hemifacial spasms

    International Nuclear Information System (INIS)

    Nagaseki, Yoshishige; Horikoshi, Tohru; Omata, Tomohiro; Sugita, Masao; Nukui, Hideaki; Sakamoto, Hajime; Kumagai, Hiroshi; Sasaki, Hideo; Tsuji, Reizou.

    1991-01-01

    We show how neurosurgical planning can benefit from the better visualization of the precise vascular compression of the nerve provided by the oblique-sagittal and gradient-echo method (OS-GR image) using magnetic resonance images (MRI). The scans of 3 patients with trigeminal neuralgia (TN) and of 15 with hemifacial spasm (HFS) were analyzed for the presence and appearance of the vascular compression of the nerves. Imaging sequences consisted of an OS-GR image (TR/TE: 200/20, 3-mm-thick slice) cut along each nerve shown by the axial view, which was scanned at the angle of 105 degrees taken between the dorsal line of the brain stem and the line corresponding to the pontomedullary junction. In the OS-GR images of the TN's, the vascular compressions of the root entry zone (REZ) of the trigeminal nerve were well visualized as high-intensity lines in the 2 cases whose vessels were confirmed intraoperatively. In the other case, with atypical facial pain, vascular compression was confirmed at the rostral distal site on the fifth nerve, apart from the REZ. In the 15 cases of HFS, twelve OS-GR images (80%) demonstrated vascular compressions at the REZ of the facial nerves from the direction of the caudoventral side. During the surgery for these 12 cases, in 11 cases (excepting the 1 case whose facial nerve was not compressed by any vessels), vascular compressions were confirmed corresponding to the findings of the OS-GR images. Among the 10 OS-GR images on the non-affected side, two false-positive findings were visualized. It is concluded that OS-GR images obtained by means of MRI may serve as a useful planning aid prior to microvascular decompression for cases of TN and HFS. (author)

  19. Ultrasonic scanner for radial and flat panels

    Science.gov (United States)

    Spencer, R. L.; Hill, E. K. (Inventor)

    1973-01-01

    An ultrasonic scanning mechanism is described that scans panels of honeycomb construction or with welded seams. It incorporates a device which by simple adjustment is adapted to scan either a flat panel or a radial panel. The supporting structure takes the form of a pair of spaced rails. An immersion tank is positioned between the rails and below their level. A work holder is mounted in the tank and is adapted to hold the flat or radial panel. A traveling bridge is movable along the rails and a carriage is mounted on the bridge.

  20. Reble, a radially converging electron beam accelerator

    International Nuclear Information System (INIS)

    Ramirez, J.J.; Prestwich, K.R.

    1976-01-01

    The Reble accelerator at Sandia Laboratories is described. This accelerator was developed to provide an experimental source for studying the relevant diode physics, beam propagation, beam energy deposition in a gas using a radially converging e-beam. The nominal parameters for Reble are 1 MV, 200 kA, 20 ns e-beam pulse. The anode and cathode are concentric cylinders with the anode as the inner cylinder. The radial beam can be propagated through the thin foil anode into the laser gas volume. The design and performance of the various components of the accelerator are presented

  1. Radial Plasma Flow Switch ^=A7

    Science.gov (United States)

    Terry, R. E.; Thornhill, J. W.

    1996-11-01

    A radial plasma flow switch configuration for use with longer quarter cycle time Marx bank drivers is characterized by 2D MHD calculations (MACH 2). A primary plasma armature implodes radially into a trap to establish the conduction phase. A secondary armature born from this plasma then commutes current to the load region at an Alfven speed characteristic of the mass splitting between the trap and the output port. The efficiency of current and energy transfer to simple inductive loads and plasma radiation source (PRS) loads is examined with respect to different models of anomalous resistivity and several geometries for controlling the motion of the primary plasma armature.

  2. Radial velocity observations of VB10

    Science.gov (United States)

    Deshpande, R.; Martin, E.; Zapatero Osorio, M. R.; Del Burgo, C.; Rodler, F.; Montgomery, M. M.

    2011-07-01

    VB 10 is the smallest star known to harbor a planet according to the recent astrometric study of Pravdo & Shaklan [1]. Here we present near-infrared (J-band) radial velocity of VB 10 performed from high resolution (R~20,000) spectroscopy (NIRSPEC/KECK II). Our results [2] suggest radial velocity variability with amplitude of ~1 km/s, a result that is consistent with the presence of a massive planet companion around VB10 as found via long-term astrometric monitoring of the star by Pravdo & Shaklan. Employing an entirely different technique we verify the results of Pravdo & Shaklan.

  3. Patients with Pelvic Floor Muscle Spasm Have a Superior Response to Pelvic Floor Physical Therapy at Specialized Centers.

    Science.gov (United States)

    Polackwich, Alan Scott; Li, Jianbo; Shoskes, Daniel A

    2015-10-01

    Chronic prostatitis/chronic pelvic pain syndrome is a common condition that often requires multimodal therapy. Patients with chronic pelvic pain syndrome have a high incidence of pelvic floor spasm, which can be treated with pelvic floor physical therapy. However, this is a specialized skill. We compared outcomes of pelvic floor physical therapy as part of multimodal therapy in patients with chronic pelvic pain syndrome between those treated at our institution and elsewhere. We identified patients from our chronic pelvic pain syndrome registry with pelvic floor spasm who were seen between 2010 and 2014 for more than 1 visit. Patient phenotype was assessed with the UPOINT system and symptom severity was determined by the National Institutes of Health CPSI. A 6-point decrease in CPSI was used to define patient improvement. A total of 82 patients fit the study criteria. Mean age was 41.6 years (range 19 to 75) and median symptom duration was 24 months (range 3 to 240). Mean CPSI was 26.8 (range 10 to 41), the median number of positive UPOINT domains was 3 (range 1 to 6) and 27 patients (32.9%) were treated locally. At followup 9 patients had refused pelvic floor physical therapy, and 24 and 48 had undergone pelvic floor physical therapy elsewhere and at CCF, respectively. The mean change in CPSI was 1.11 ± 4.1 in patients who refused, -3.46 ± 6.7 in those treated elsewhere and -11.3 ± 7.0 in those treated at CCF (p physical therapy at CCF (OR 4.23, p = 0.002) and symptom duration (OR 0.52, p = 0.03) predicted improvement. Pelvic floor physical therapy can be effective for chronic pelvic pain syndrome in patients with pelvic floor spasm. However, the outcome depends on specialty training and experience of therapists. Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  4. Comparison of electrocardiography and thallium-201 myocardial scintigraphy for the detection of ergonovine-induced coronary artery spasm: angiographic correlation

    International Nuclear Information System (INIS)

    Shanes, J.G.; Pavel, D.; Blend, M.

    1987-01-01

    This study was performed to determine the sensitivity of thallium imaging vs ECG monitoring for detecting coronary artery spasm noninvasively following intravenous ergonovine administration as compared to simultaneous coronary angiography. Thirty-two patients with insignificant coronary artery disease and chest pain underwent 12-lead ECG monitoring, thallium imaging, and coronary arteriography following the administration of 0.05, 0.1, 0.2, and 0.3 mg of ergonovine given 5 minutes apart or until chest pain occurred. One minute following the last dose of ergonovine, 2.5 mCi of thallium-201 was injected intravenously, and a final ECG was recorded and repeat coronary arteriography performed. Within 10 minutes following the injection of thallium, imaging was performed in the 40-degree and 70-degree left anterior oblique and anterior projections. The ECG, thallium study, and coronary arteriogram were read blindly and results were compared. The ECG, angiogram, and thallium study were read as positive if the following occurred, respectively: greater than or equal to 1 mm ST segment elevation, depression, or T wave reversal; greater than 50% vessel narrowing,; and reversible perfusion defect. Five patients were excluded from analysis because of either catheter-induced spasm, suboptimal thallium studies, or protocol violations. Of the 27 patients included for analysis, six had chest pain, five had a positive angiogram, five had a positive thallium study, and one had a positive ECG. The sensitivity of thallium vs ECG monitoring was 80% vs 25%, and the accuracy was 92% vs 80%. We conclude that thallium imaging greatly increases the noninvasive detection of ergonovine-induced coronary spasm as compared with the ECG with no loss of accuracy

  5. Improving the inter-rater agreement of hypsarrhythmia using a simplified EEG grading scale for children with infantile spasms.

    Science.gov (United States)

    Mytinger, John R; Hussain, Shaun A; Islam, Monica P; Millichap, John J; Patel, Anup D; Ryan, Nicole R; Twanow, Jaime-Dawn E; Heyer, Geoffrey L

    2015-10-01

    There is poor inter-rater agreement in determining the presence or absence of hypsarrhythmia among patients with infantile spasms. Yet, remission of hypsarrhythmia has been used as a clinical and research outcome measure. Two important features of hypsarrhythmia are the burden of epileptiform discharges and the amplitudes of background slow waves. We hypothesized that an electroencephalogram (EEG) grading scale emphasizing epileptiform discharge burden and the amplitudes of background slow waves would improve inter-rater agreement in interpreting hypsarrhythmia. Our aim was to assess inter-rater agreement of hypsarrhythmia using a novel and simplified EEG grading scale called the 'BASED' (Burden of Amplitudes and Epileptiform Discharges) score and compare this to the traditional method of EEG analysis. Twenty patients with infantile spasms were prospectively evaluated and electroclinical outcomes were determined. Forty EEG clips (20 pre-treatment and 20 post-treatment), representing the most severely abnormal five minute sleep epoch of each study, were assessed by three reviewers blinded to treatment and clinical outcome. Fleiss' kappa (К) was used to assess the inter-rater agreement in the interpretation of hypsarrhythmia when using the BASED score compared to the traditional method of EEG analysis. Reviewers had favorable inter-rater agreement using the BASED score in interpreting hypsarrhythmia (К: 0.87) compared to when using the traditional method of EEG analysis to interpret hypsarrhythmia (К: 0.09). The three reviewers all agreed on the presence or absence of hypsarrhythmia in 37/40 (93%) epochs using the BASED score but in only 15/40 (38%) epochs using the traditional method of EEG analysis, p=infantile spasms clinical trials must better define criteria for hypsarrhythmia. Copyright © 2015 The Authors. Published by Elsevier B.V. All rights reserved.

  6. Risk of vigabatrin-associated brain abnormalities on MRI in the treatment of infantile spasms is dose-dependent.

    Science.gov (United States)

    Hussain, Shaun A; Tsao, Jackie; Li, Menglu; Schwarz, Madeline D; Zhou, Raymond; Wu, Joyce Y; Salamon, Noriko; Sankar, Raman

    2017-04-01

    Although the link between vigabatrin (VGB) and retinotoxicity is well known, little attention has been focused on the risk of VGB-associated brain abnormalities on magnetic resonance imaging (MRI) (VABAM), namely reversible-and largely asymptomatic-signal changes in the thalami, basal ganglia, brainstem tegmentum, and cerebellar nuclei. Using a large infantile spasms cohort, we set out to identify predictors of these phenomena. Children with infantile spasms were retrospectively identified. Brain MRI reports were serially reviewed without knowledge of VGB exposure. Upon VABAM discovery, records were systematically reviewed to ascertain presence of symptoms attributable to VGB. Separately, progress notes were sequentially reviewed to identify and quantify VGB exposure. We identified 507 brain MRI studies among 257 patients with infantile spasms. VGB treatment was documented in 143 children, with detailed exposure data available for 104, of whom 45 had at least one MRI study during VGB treatment. Among the limited subset of asymptomatic children who underwent MRI (n = 40), 6 exhibited VABAM. Risk of asymptomatic VABAM was dose-dependent, as peak (but not cumulative) VGB dosage was strongly associated with asymptomatic VABAM (p = 0.0028). In an exploratory analysis, we encountered 4 children with symptomatic VABAM among 104 patients with detailed VGB exposure data. Risk of symptomatic VABAM was seemingly dose-independent, and potentially associated with concomitant hormonal therapy (i.e., prednisolone and adrenocorticotropic hormone [ACTH]) (p = 0.039). We have demonstrated dose-dependent risk of asymptomatic VABAM and uncovered a possible association between symptomatic VABAM and concomitant hormonal therapy. Caution should be exercised in the use of high VGB dosage (i.e., >175 mg/kg/day), and further study is warranted to confirm the potential impact of hormonal therapy. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

  7. One-year results of cemented bipolar radial head prostheses for comminuted radial head fractures

    Directory of Open Access Journals (Sweden)

    Laun, Reinhold

    2015-12-01

    Full Text Available Introduction: Comminuted radial head fractures (Mason type III continue to pose a challenge to orthopedic surgeons. When internal fixation is not possible, radial head arthroplasty has been advocated as the treatment of choice. The purpose of this retrospective study was to evaluate clinical and radiological short-term results of patients with Mason type III radial head fractures treated with a cemented bipolar radial prosthesis. Methods: Twelve patients received cemented bipolar radial head hemiarthroplasty for comminuted radial head fractures. In all patients a CT scan was obtained prior to surgical treatment to assess all associated injuries. Postoperatively an early motion protocol was applied. All patients were evaluated clinically and radiologically at an average of 12.7 months.Results: According to the Mayo Modified Wrist Score, the Mayo Elbow Performance Score, the functional rating index of Broberg and Morrey, and the DASH Score good to excellent results were obtained. Grip strength and range of motion were almost at the level of the unaffected contralateral side. Patient satisfaction was high, no instability or signs of loosening of the implant, and only mild signs of osteoarthritis were seen.Conclusion: Overall good to excellent short-term results for primary arthroplasty for comminuted radial head fractures were observed. These encouraging results warrant the conduction of further studies with long-term follow-up and more cases to see if these short-term results can be maintained over time.

  8. The extraordinary spectral properties of radially periodic ...

    Indian Academy of Sciences (India)

    R. Narasimhan (Krishtel eMaging) 1461 1996 Oct 15 13:05:22

    coupling constant, such that a rather precise picture of the spectrum of radially periodic. Schrödinger operators has now been obtained. Keywords. Schr ödinger operator; self-adjointness; embedded eigenvalue; exponential decay; dense point spectrum. 0. Introduction and preliminaries. The Schrödinger equation i. ∂. ∂t.

  9. Revealing the radial modes in vortex beams

    CSIR Research Space (South Africa)

    Sephton, Bereneice C

    2016-10-01

    Full Text Available is neglected in this generation approach. Here, we show that a consequence of this is that vortex beams carry very little energy in the desired zeroth radial order, as little as only a few percent of the incident power. We demonstrate this experimentally...

  10. Radial interchange motions of plasma filaments

    DEFF Research Database (Denmark)

    Garcia, O.E.; Bian, N.H.; Fundamenski, W.

    2006-01-01

    on a biperiodic domain perpendicular to the magnetic field. It is demonstrated that a blob-like plasma structure develops dipolar vorticity and electrostatic potential fields, resulting in rapid radial acceleration and formation of a steep front and a trailing wake. While the dynamical evolution strongly depends...

  11. Three versus four radial keratotomy incisions.

    Science.gov (United States)

    Melles, G R; Go, A T; Beekhuis, W H; van Rij, G; Binder, P S

    1992-01-01

    Radial keratotomy (RK) is currently performed with four or eight semi-radial incisions. To evaluate the effect of a theoretically more stable three-incision RK pattern, centripetal incisions were made in 16 human donor eyes (eight pairs), using a double-edged diamond blade set to 90% of central pachymetry and a 3.5 mm optical clear zone. Intraocular pressure was maintained at 15 mm Hg during surgery and while keratometry readings were made. One randomly selected eye of each pair had three radial incisions made at 12, 4 and 8 o'clock; the other eye had four radial incisions at 12, 3, 6, and 9 o'clock. Corneal flattening was 6.08 diopters (D) with four incisions and 4.84 D with three incisions (P less than .05). Astigmatism increased 0.44 D and 0.69 D, respectively (P greater than .1). Histologically measured mean incision depth (77.4%) did not differ significantly between the groups (P greater than .1). This study shows that 80% of the effect of a four-incision RK pattern can be obtained with a theoretically more stable three-incision pattern.

  12. Spectral problem for the radial Schroedinger equation

    International Nuclear Information System (INIS)

    Vshivtsev, A.S.; Tatarintsev, A.V.; Prokopov, A.V.; Sorokin, V. N.

    1998-01-01

    For the first time, a procedure for determining spectra on the basis of generalized integral transformations is implemented for a wide class of radial Schroedinger equations. It is shown that this procedure works well for known types of potentials. Concurrently, this method makes it possible to obtain new analytic results for the Cornell potential. This may prove important for hadron physics

  13. Computing modal dispersion characteristics of radially Asymmetric ...

    African Journals Online (AJOL)

    We developed a matrix theory that applies to with non-circular/circular but concentric layers fibers. And we compute the dispersion characteristics of radially unconventional fiber, known as Asymmetric Bragg fiber. An attempt has been made to determine how the modal characteristics change as circular Bragg fiber is ...

  14. Periarticular Morphine-Induced Sphincter of Oddi Spasm Causing Severe Pain and Bradycardia in an Awake Patient Under Spinal Anesthesia: An Important Diagnostic Consideration.

    Science.gov (United States)

    Koumpan, Yuri; Engen, Dale; Tanzola, Robert; Saha, Tarit

    2016-10-01

    Sphincter of Oddi spasm from opioids has been documented, presenting as severe epigastric pain and potentially overlooked in a differential diagnosis. We present a case of sphincter of Oddi spasm from periarticular morphine in a patient under spinal anesthesia, causing severe distress and treated effectively with glucagon. It is important for anesthesiologists using opioids to consider it as a cause of perioperative pain and be familiar with treatment as it may be refractory by conventional use of opioids for pain relief. It is also important to consider the systemic effects of periarticular absorption, as evident by our case.

  15. Randomized, Single-Blind, Parallel Clinical Trial on Efficacy of Oral Prednisolone Versus Intramuscular Corticotropin on Immediate and Continued Spasm Control in West Syndrome.

    Science.gov (United States)

    Wanigasinghe, Jithangi; Arambepola, Carukshi; Sri Ranganathan, Shalini; Sumanasena, Samanmalie; Attanapola, Gangani

    2015-09-01

    A single-center, single-blind, parallel-group, randomized clinical trial was performed to test the null hypothesis that adrenocorticotropic hormone is not superior to high-dose prednisolone for treatment of newly diagnosed West syndrome. Newly diagnosed infants with West syndrome were randomized to receive 14 days of oral prednisolone (40-60 mg/day) or a synthetically prepared intramuscular long-acting adrenocorticotropic hormone (40-60 IU/every other day [0.5-0.75 mg]) according to the United Kingdom Infantile Spasm Study protocol. They were blindly evaluated for infantile spasm remission by day 14, electroclinical remission (spasm cessation + resolution of hypsarrhythmia on a 30-minute electroencephalograph) by day 14 and continued spasm freedom for 28 days. Ninety-seven patients were enrolled in the study, with 48 of them receiving prednisolone and 49 receiving ACTH. There was no significant difference in the baseline characteristics or risk factors for the two treatment groups. By day 14, cessation of infantile spasms occurred in 28/48 (58.3%) infants on prednisolone compared with only 18/49 (36.7%) infants given adrenocorticotropic hormone (P = 0.03) and electroclinical remission in 21 on prednisolone compared with nine on adrenocorticotropic hormone (P = 0.007). Sustained spasm control for 28 consecutive days following electroclinical remission occurred in 15 children on prednisolone compared with six on adrenocorticotropic hormone (P = 0.008). The total number of days required for spasm cessation was significantly less in those treated with prednisolone (3.85 days ± 2.4) compared with adrenocorticotropic hormone (8.65 days ± 3.7) (P = 0.001). Among patients who did not achieve remission, there was a non-significant trend toward greater quantitative reduction of spasms with prednisolone than with adrenocorticotropic hormone (P = 0.079). Synthetic adrenocorticotropic hormone of 40-60 IU/every other day did not yield superior rates of electroencephalographic

  16. Right radial nerve dysfunction following laparoscopic sigmoid colectomy

    Directory of Open Access Journals (Sweden)

    Yoshikazu Takinami

    2014-10-01

    Full Text Available Here, we report a case of right radial nerve dysfunction following laparoscopic sigmoid colectomy under general anesthesia. A 75-year-old man was intubated without excessive retroflexion, and his upper body was held in place by lateral body positioners with protective cushions over the chest and acromioclavicular joints. The patient’s head was maintained at the center and held on the operation table with a memory-foam pillow to prevent hyperextension of the neck. The arms, abducted 80° with the forearms supinated, were held in place on the armrests with protective cushions. The surgical position was a 20° head-down lithotomy position with the right side of the body lowered by 15°. Surgery was completed successfully with no complications, and anesthesia time was 7 h and 37 min. After surgery, however, the patient complained of numbness and hypoesthesia on the radial and ulnar side, respectively, of the right arm from the elbow to the fingertips, with the boundary running between fingers 3 and 4. Dysesthesia was observed in the right fingertips of fingers 1–3. After 3 months of silver spike point low-frequency electrotherapy, hypoesthesia improved, while dysesthesia partially improved, in the dorsal area between right fingers 1 and 2.

  17. Tetanus: Pathophysiology, Treatment, and the Possibility of Using Botulinum Toxin against Tetanus-Induced Rigidity and Spasms

    Directory of Open Access Journals (Sweden)

    Bjørnar Hassel

    2013-01-01

    Full Text Available Tetanus toxin, the product of Clostridium tetani, is the cause of tetanus symptoms. Tetanus toxin is taken up into terminals of lower motor neurons and transported axonally to the spinal cord and/or brainstem. Here the toxin moves trans-synaptically into inhibitory nerve terminals, where vesicular release of inhibitory neurotransmitters becomes blocked, leading to disinhibition of lower motor neurons. Muscle rigidity and spasms ensue, often manifesting as trismus/lockjaw, dysphagia, opistotonus, or rigidity and spasms of respiratory, laryngeal, and abdominal muscles, which may cause respiratory failure. Botulinum toxin, in contrast, largely remains in lower motor neuron terminals, inhibiting acetylcholine release and muscle activity. Therefore, botulinum toxin may reduce tetanus symptoms. Trismus may be treated with botulinum toxin injections into the masseter and temporalis muscles. This should probably be done early in the course of tetanus to reduce the risk of pulmonary aspiration, involuntary tongue biting, anorexia and dental caries. Other muscle groups are also amenable to botulinum toxin treatment. Six tetanus patients have been successfully treated with botulinum toxin A. This review discusses the use of botulinum toxin for tetanus in the context of the pathophysiology, symptomatology, and medical treatment of Clostridium tetani infection.

  18. Tetanus: Pathophysiology, Treatment, and the Possibility of Using Botulinum Toxin against Tetanus-Induced Rigidity and Spasms

    Science.gov (United States)

    Hassel, Bjørnar

    2013-01-01

    Tetanus toxin, the product of Clostridium tetani, is the cause of tetanus symptoms. Tetanus toxin is taken up into terminals of lower motor neurons and transported axonally to the spinal cord and/or brainstem. Here the toxin moves trans-synaptically into inhibitory nerve terminals, where vesicular release of inhibitory neurotransmitters becomes blocked, leading to disinhibition of lower motor neurons. Muscle rigidity and spasms ensue, often manifesting as trismus/lockjaw, dysphagia, opistotonus, or rigidity and spasms of respiratory, laryngeal, and abdominal muscles, which may cause respiratory failure. Botulinum toxin, in contrast, largely remains in lower motor neuron terminals, inhibiting acetylcholine release and muscle activity. Therefore, botulinum toxin may reduce tetanus symptoms. Trismus may be treated with botulinum toxin injections into the masseter and temporalis muscles. This should probably be done early in the course of tetanus to reduce the risk of pulmonary aspiration, involuntary tongue biting, anorexia and dental caries. Other muscle groups are also amenable to botulinum toxin treatment. Six tetanus patients have been successfully treated with botulinum toxin A. This review discusses the use of botulinum toxin for tetanus in the context of the pathophysiology, symptomatology, and medical treatment of Clostridium tetani infection. PMID:23299659

  19. The Successful Treatment of Opioid Withdrawal-Induced Refractory Muscle Spasms with 5-HTP in a Patient Intolerant to Clonidine.

    Science.gov (United States)

    Dais, Jennifer; Khosia, Ankur; Doulatram, Gulshan

    2015-01-01

    Instituting drug holidays for chronic opioid using patients is becoming commonplace for pain practitioners initiating procedures such as intrathecal pump or spinal cord stimulator trials. As such, pain practitioners need to be adept in their management of acute opioid withdrawal. Successfully weaning an opioid dependent patient off of chronic opioids requires a thorough knowledge of the available adjuvants to assist in this process. However, that selection can become exhausted by adjuvant side effects or by ineffective attenuation of opioid withdrawal symptoms. In that case, novel drugs, or novel application of currently available medications must be sought after to assist in the drug holiday. We present a case in which refractory muscle spasms secondary to opioid withdrawal were successfully treated with an over-the-counter supplement that is not typically used for the attenuation of opioid withdrawal symptoms. In a patient intolerant to the side effects of clonidine, we were able to successfully wean chronic opiates by treating refractory muscle spasms with the serotonin precursor, 5-hydroxytryptophan (5-HTP). We hypothesize that our success with this medication gives further credence to the role of serotonin in opioid withdrawal somatic symptomatology, and supports the need for future research to clarify the role of serotonin precursors or serotonin modulating drugs as potential alternatives in those unable to follow standard treatment protocols.

  20. Espasmos infantis: experiência em treze casos Infantile spasms: experience in thriteen cases

    Directory of Open Access Journals (Sweden)

    LUIZ FERNANDO FONSECA

    2000-06-01

    Full Text Available Os espasmos infantis são crises típicas da primeira infância e constituem patologia grave, com prognóstico sombrio. Apresentamos a experiência no tratamento de 13 casos novos atendidos no Serviço de Neurologia Infantil do Centro Geral de Pediatria FHEMIG de Belo Horizonte no ano de 1997, bem como revisão da literatura sobre o assunto. Após propedêutica adequada encontramos 12 casos considerados sintomáticos e 1 criptogenético. Todos os casos foram tratados com ACTH durante 6 semanas, associado a drogas antiepilépticas orais de manutenção em mono ou politerapia. Os resultados com o ACTH foram excelentes num momento inicial, com resposta completa em todos os casos e efeitos colaterais que não contra-indicaram o tratamento. Porém houve índice de recorrência de 55%, sendo usada como droga de segunda linha a vigabatrina em 5 casos, com controle de 4 deles. Todos os casos apresentaram atraso do desenvolvimento neuropsicomotor.Infantile spasms are typical crisis found in the childhood being a serious pathology with an obscure prognosis. We present the experience of the Service of Neuropediatrics in the Pediatric General Center, Belo Horizonte -- Brasil, during the year of 1997, with a review of the literature about. After appropriate propaedeutics, were found 12 cases considered symptomatics and 1 case cryptogenic. All these cases were treated with ACTH during 6 weeks associated with oral antiepileptic drugs in mono or polytherapy for maintenance. We had excellent results using ACTH at the beginning of the treatment, obtaining good results in all of the cases with some side effects that were not important enough to interrupt the treatment. However, there was a recurrence rate of 55% and vigabatrin was used as second option, controlling 4 of the 5 cases. All the cases showed retardation of the neuropsychomotor development.

  1. Neurodevelopmental outcome of infantile spasms: A systematic review and meta-analysis.

    Science.gov (United States)

    Widjaja, Elysa; Go, Cristina; McCoy, Blathnaid; Snead, O Carter

    2015-01-01

    The aims of this systematic review and meta-analysis were to assess (i) estimates of good neurodevelopmental outcome in infantile spasms (IS), (ii) if neurodevelopmental outcome has changed since the publication of the first guideline on medical treatment of IS in 2004 and (iii) effect of lead time to treatment (LTTT). The Medline, Embase, Cochrane, PsycINFO, Web of Science and Scopus databases, and reference lists of retrieved articles were searched. Studies inclusion criteria were: (i) >5 patients with IS, (ii) mean/median follow-up of >6 months, (iii) neurodevelopmental outcome, and (iv) randomized and observational studies. The data extracted included proportion of good neurodevelopmental outcome, year of publication, cryptogenic or symptomatic IS and LTTT. Of the 1436 citations screened, 55 articles were included in final analysis, with a total of 2967 patients. The pooled estimate for good neurodevelopmental outcome was 0.236 (95% CI: 0.193-0.286). There was no difference between the proportions of good neurodevelopmental outcome for the 21 studies published after 2004 [0.264 (95% CI: 0.197-0.344)] compared to the 34 studies published before 2004 [0.220 (95% CI: 0.168-0.283)] (Q value=0.862, p=0.353). The pooled estimate of good neurodevelopmental outcome for cryptogenic IS [0.543 (95% CI: 0.458-0.625)] was higher than symptomatic IS [0.125 (95% CI: 0.09-0.171)] (Q value=69.724, p4weeks for good neurodevelopmental outcome of 8 studies was 1.519 (95% CI: 1.064-2.169). Neurodevelopmental outcome was overall poor in patients with IS and has not changed since the publication of first guideline on IS. Although cryptogenic IS has better prognosis than symptomatic IS, the outcome for cryptogenic IS remained poor. There was heterogeneity in neurodevelopmental outcome ascertainment methods, highlighting the need for a more standardized and comprehensive assessment of cognitive, behavioural, emotional and functional outcomes. Copyright © 2014 Elsevier B.V. All rights

  2. Research on Radial Motion Characteristic of the Cropping Hammer in Radial-Forging Cropping Method

    Directory of Open Access Journals (Sweden)

    Lijun Zhang

    2015-01-01

    Full Text Available The radial loading form applied to the bar is very important for reducing or avoiding the impact and vibration of the radial-forging cropping system and obtaining the high-quality cross section. A new radial stroke loading curve of the cropping hammer based on the cycloid form is proposed and the dynamic model of radial stroke loading mechanism is built. With the aim of obtaining the equivalent stiffness of the bar with V-shaped notch, which is a key parameter affecting the dynamic characteristic of radial stroke loading mechanism, the analytic model of the bar is built and the simulation experiments are designed by means of the orthogonal test method. The analytical results show that the diameter of the bar has the significant influence on the equivalent stiffness of the bar. Furthermore, the equivalent stiffness of the bar with V-shaped notch can be directly calculated according to the equivalent stiffness of smooth bar when h/d0.15. By using the cycloid stroke curve, the cropping experimental results for 45 steel bars and 20 steel bars show that the radial impact and vibration of the cropping system are decreased and the bar cross-section qualities have been significantly improved.

  3. Efecto a corto plazo de la vigabatrina en los espasmos infantiles Short term effect of vigabatrin in infantile spasms

    Directory of Open Access Journals (Sweden)

    Albia J. Pozo Alonso

    2007-03-01

    Full Text Available El objetivo de este trabajo fue valorar el efecto a corto plazo de la vigabatrina en 18 pacientes con el diagnóstico de espasmos infantiles. Trece pacientes fueron tratados en monoterapia, y 6 de ellos de primera intención. La dosis máxima promedio utilizada de vigabatrina fue de 130 mg/ (kg∙día (rango de 75 a 170 mg/ [kg∙día]. Los espasmos epilépticos cesaron en el 44,4 % de los casos a los 18,4 días como promedio tras el inicio del tratamiento con vigabatrina (rango de 3 a 43 días. La dosis promedio de respuesta a la vigabatrina fue de 103 mg/ (kg∙día (rango de 50 a 156 mg/ [kg∙día]. En el 16,7 % de los niños se logró la disminución de las crisis en más del 90 % y en el 5,6 % se redujeron los espasmos epilépticos en más del 50 %. Los espasmos epilépticos persistieron en el 33,3 %. Se obtuvo mejoría electroencefalográfica en el 55,6 % de los casos y en el 5,6 % desaparecieron las descargas. La hipsarritmia desapareció en el 75 % de los pacientes. Se debe continuar empleando la vigabatrina en monoterapia o como terapia adjunta en pacientes con espasmos infantiles.The purpose of this paper was to assess the short term effect of vigabatrin in 18 patients that were diagnosed infantile spasms. Thirteen of them were treated with monotherapy, and six of them were treated of first intention. The average maximum dose of vigabatrin was 130 mg/(kg∙day (range 75-170 mg/[kg∙day]. The epileptic spasms ceased in 44.4 % of the cases at 18.4 days as an average after the beginning of the treatment with vigabatrin (range 3 to 43 days. The average dose of response to vigabatrin was 103 mg/(kg∙day (range 50 to 156 mg/[kg∙day]. In 16.7 % of the children it was possible to reduce the crises more than 90 % , whereas in 5.6 % the epileptic spasms decreased more than 50 %. The epileptic spasms persisted in 33.3 %. An electroencephalographic improvement was observed in 55.6 % of the cases, and in 5.6 % the discharges vanished

  4. A lack of clinically apparent vision loss among patients treated with vigabatrin with infantile spasms: The UCLA experience.

    Science.gov (United States)

    Schwarz, Madeline D; Li, Menglu; Tsao, Jackie; Zhou, Raymond; Wu, Yvonne W; Sankar, Raman; Wu, Joyce Y; Hussain, Shaun A

    2016-04-01

    Vigabatrin (VGB) is one of two FDA-approved medications for treatment of infantile spasms. Despite demonstrated efficacy, its use has been curtailed by reports indicating a substantial risk of VGB-associated visual field loss (VAVFL). As these reports have conflicted with our clinical observations in routine practice, we systematically reviewed the experiences of patients treated with VGB at UCLA to estimate the prevalence of clinically apparent VAVFL. Patients with video-EEG-confirmed infantile spasms evaluated at our center between February 2007 and February 2014 were retrospectively identified. Among patients with VGB exposure, we documented relevant clinical factors and determined the duration of therapy, peak dosage, and cumulative dosage. Based on a review of serial neurologic and ophthalmologic reports and aided by electroretinography (ERG) assessments when available, we ascertained whether each patient had evidence of clinically apparent vision impairment (i.e., recognized by a neurologist or ophthalmologist during any follow-up visit) and whether or not the vision loss was attributed to VGB exposure (i.e., evidence of bilateral, symmetric, and peripheral visual field loss), either by the treating physician or on retrospective review by the study team. During the study period, 257 patients with video-EEG-confirmed infantile spasms were identified. One hundred and forty-three (56%) patients received VGB. Although visual loss of any cause was common among patients with (31%) and without (32%) VGB exposure, there were no cases in which visual field defects were plausibly linked to VGB. We estimate that the risk of clinically significant VAVFL does not exceed 3.2% (95% CI upper bound). Vision loss was never characterized as exclusively peripheral and was always better explained by other causes (e.g., hemianopsia following hemispherectomy and cortical vision impairment after hypoxic ischemic encephalopathy). Precise quantitative exposure data were available for

  5. Wrist extension strength required for power grip: a study using a radial nerve block model.

    Science.gov (United States)

    Suzuki, T; Kunishi, T; Kakizaki, J; Iwakura, N; Takahashi, J; Kuniyoshi, K

    2012-06-01

    The aim of this study was to investigate the correlation of wrist extension strength (WES) and grip strength (GS) using a radial nerve block, and to determine the WES required to prevent the "wrist flexion phenomenon" (antagonistic WES) when making a fist. We tested 14 arms in seven healthy males. WES and GS were measured before blocking as standard WES and standard GS. All participants then had radial nerve blocks with mepivacaine hydrochloride. During the recovery process from radial nerve blockade, WES and GS were recorded every 5 minutes. There was a very strong correlation between WES and GS (p < 0.0001). The mean antagonistic WES was 51% of standard WES, and the mean GS, recorded at the same time, was 66% of standard GS.

  6. Precise Near-Infrared Radial Velocities

    Science.gov (United States)

    Plavchan, Peter; Gao, Peter; Gagne, Jonathan; Furlan, Elise; Brinkworth, Carolyn; Bottom, Michael; Tanner, Angelle; Anglada-Escude, Guillem; White, Russel; Davison, Cassy; Mills, Sean; Beichman, Chas; Johnson, John Asher; Ciardi, David; Wallace, Kent; Mennesson, Bertrand; Vasisht, Gautam; Prato, Lisa; Kane, Stephen; Crawford, Sam; Crawford, Tim; Sung, Keeyoon; Drouin, Brian; Lin, Sean; Leifer, Stephanie; Catanzarite, Joe; Henry, Todd; von Braun, Kaspar; Walp, Bernie; Geneser, Claire; Ogden, Nick; Stufflebeam, Andrew; Pohl, Garrett; Regan, Joe

    2016-01-01

    We present the results of two 2.3 μm near-infrared (NIR) radial velocity (RV) surveys to detect exoplanets around 36 nearby and young M dwarfs. We use the CSHELL spectrograph (R ~ 46,000) at the NASA InfraRed Telescope Facility (IRTF), combined with an isotopic methane absorption gas cell for common optical path relative wavelength calibration. We have developed a sophisticated RV forward modeling code that accounts for fringing and other instrumental artifacts present in the spectra. With a spectral grasp of only 5 nm, we are able to reach long-term radial velocity dispersions of ~20-30 m s-1 on our survey targets.

  7. WWER radial reflector modeling by diffusion codes

    International Nuclear Information System (INIS)

    Petkov, P. T.; Mittag, S.

    2005-01-01

    The two commonly used approaches to describe the WWER radial reflectors in diffusion codes, by albedo on the core-reflector boundary and by a ring of diffusive assembly size nodes, are discussed. The advantages and disadvantages of the first approach are presented first, then the Koebke's equivalence theory is outlined and its implementation for the WWER radial reflectors is discussed. Results for the WWER-1000 reactor are presented. Then the boundary conditions on the outer reflector boundary are discussed. The possibility to divide the library into fuel assembly and reflector parts and to generate each library by a separate code package is discussed. Finally, the homogenization errors for rodded assemblies are presented and discussed (Author)

  8. SpicyNodes Radial Map Engine

    Science.gov (United States)

    Douma, M.; Ligierko, G.; Angelov, I.

    2008-10-01

    The need for information has increased exponentially over the past decades. The current systems for constructing, exploring, classifying, organizing, and searching information face the growing challenge of enabling their users to operate efficiently and intuitively in knowledge-heavy environments. This paper presents SpicyNodes, an advanced user interface for difficult interaction contexts. It is based on an underlying structure known as a radial map, which allows users to manipulate and interact in a natural manner with entities called nodes. This technology overcomes certain limitations of existing solutions and solves the problem of browsing complex sets of linked information. SpicyNodes is also an organic system that projects users into a living space, stimulating exploratory behavior and fostering creative thought. Our interactive radial layout is used for educational purposes and has the potential for numerous other applications.

  9. Numerical simulation of radial compressor stage

    Science.gov (United States)

    Syka, T.; Luňáček, O.

    2013-04-01

    Article describes numerical simulations of air flow in radial compressor stage in NUMECA CFD software. In simulations geometry variants with and without seals are used. During tasks evaluating was observed seals influence on flow field and performance parameters of compressor stage. Also is described CFDresults comparison with results from design software based on experimental measurements and monitoring of influence of seals construction on compressor stage efficiency.

  10. Numerical simulation of radial compressor stage

    OpenAIRE

    Luňáček O.; Syka T.

    2013-01-01

    Article describes numerical simulations of air flow in radial compressor stage in NUMECA CFD software. In simulations geometry variants with and without seals are used. During tasks evaluating was observed seals influence on flow field and performance parameters of compressor stage. Also is described CFDresults comparison with results from design software based on experimental measurements and monitoring of influence of seals construction on compressor stage efficiency.

  11. Numerical simulation of radial compressor stage

    Directory of Open Access Journals (Sweden)

    Luňáček O.

    2013-04-01

    Full Text Available Article describes numerical simulations of air flow in radial compressor stage in NUMECA CFD software. In simulations geometry variants with and without seals are used. During tasks evaluating was observed seals influence on flow field and performance parameters of compressor stage. Also is described CFDresults comparison with results from design software based on experimental measurements and monitoring of influence of seals construction on compressor stage efficiency.

  12. Learning Methods for Radial Basis Functions Networks

    Czech Academy of Sciences Publication Activity Database

    Neruda, Roman; Kudová, Petra

    2005-01-01

    Roč. 21, - (2005), s. 1131-1142 ISSN 0167-739X R&D Projects: GA ČR GP201/03/P163; GA ČR GA201/02/0428 Institutional research plan: CEZ:AV0Z10300504 Keywords : radial basis function networks * hybrid supervised learning * genetic algorithms * benchmarking Subject RIV: BA - General Mathematics Impact factor: 0.555, year: 2005

  13. Radial zoning in large LMFBRs. Part I

    International Nuclear Information System (INIS)

    King, M.J.; Barthold, W.P.

    1975-08-01

    The effect of radial zoning on optimization of the nuclear power peaking factor is considered for large LMFBRs of the 5000 MW(t) class. The dependence of the power peaking factor on the ratios of outer-to-inner core volumes and enrichments was investigated for static conditions and burn cycles corresponding to first core loadings and equilibrium cores. Carbide and nitride fuels were considered

  14. Radial oxygen gradients over rat cortex arterioles

    OpenAIRE

    Galler, Michael

    2011-01-01

    Purpose: We present the results of the visualisation of radial oxygen gradients in rats’ cortices and their use in neurocritical management. Methods: PO2 maps of the cortex of 10 wistar rats were obtained with a camera (SensiMOD, PCO, Kehlheim, Germany). Those pictures were analyzed and edited by a custom-made software. We chose a vessel for examination. A matrix, designed to evaluate the cortical O2 partial pressure, was placed vertically to the artery and afterwards multiple regio...

  15. Moment approach to tandem mirror radial transport

    International Nuclear Information System (INIS)

    Siebert, K.D.; Callen, J.D.

    1986-02-01

    A moment approach is proposed for the study of tandem mirror radial transport in the resonant plateau regime. The salient features of the method are described with reference to axisymmetric tokamak transport theory. In particular, the importance of momentum conservation to the establishment of the azimuthal variations in the electrostatic potential is demonstrated. Also, an ad hoc drift kinetic equation is solved to determine parallel viscosity coefficients which are required to close the moment system

  16. Photoelectric Radial Velocities, Paper XIX Additional Spectroscopic ...

    Indian Academy of Sciences (India)

    m star nearly 1 south of HD 188058; it has the Tycho designa- tion 2152−5699−1, and was found byTycho to have V = 10m.52, B − V = 0m.57. Although such a colour index would suggest that there should be no difficulty in measuring the star's radial velocity with the spectrometers, three separate efforts at. OHP resulted in ...

  17. Fuel radial design using Path Relinking

    International Nuclear Information System (INIS)

    Campos S, Y.

    2007-01-01

    The present work shows the obtained results when implementing the combinatory optimization technique well-known as Path Re linking (Re-linkage of Trajectories), to the problem of the radial design of nuclear fuel assemblies, for boiling water reactors (BWR Boiling Water Reactor by its initials in English), this type of reactors is those that are used in the Laguna Verde Nucleo electric Central, Veracruz. As in any other electric power generation plant of that make use of some fuel to produce heat and that it needs each certain time (from 12 to 14 months) to make a supply of the same one, because this it wears away or it burns, in the nucleolectric plants to this activity is denominated fuel reload. In this reload different activities intervene, among those which its highlight the radial and axial designs of fuel assemblies, the patterns of control rods and the multi cycles study, each one of these stages with their own complexity. This work was limited to study in independent form the radial design, without considering the other activities. These phases are basic for the fuel reload design and of reactor operation strategies. (Author)

  18. Symmetries of Trautman retarded radial coordinates

    Science.gov (United States)

    Kolanowski, Maciej; Lewandowski, Jerzy

    2018-02-01

    We consider spacetime described by an observer that uses a Trautman retarded radial coordinate system. Given a metric tensor, we find all the local symmetries of the coordinates. They set a 10D family that can be parametrized by Poincaré algebra. This result is similar to the symmetries of an observer using the Gaussian normal spacetime radial coordinates and experiencing algebra deformation induced by the spacetime Riemann tensor. A new, surprising property of the retarded coordinates is a generic lack of smoothness in the symmetries. We show that, in general, the symmetries are not twice differentiable. In other words, a family of smooth symmetries is smaller than in the Gaussian normal spacetime coordinate case. We demonstrate examples of that non-smoothness and find the necessary conditions for the differentiability to the second order. We also discuss the consequences and relevance of that result for the geometric relational observables program. One can interpret our result by the fact that Trautman coordinates provide gauge conditions stronger than the Gaussian spacetime radial gauge.

  19. Fast radial basis functions for engineering applications

    CERN Document Server

    Biancolini, Marco Evangelos

    2017-01-01

    This book presents the first “How To” guide to the use of radial basis functions (RBF). It provides a clear vision of their potential, an overview of ready-for-use computational tools and precise guidelines to implement new engineering applications of RBF. Radial basis functions (RBF) are a mathematical tool mature enough for useful engineering applications. Their mathematical foundation is well established and the tool has proven to be effective in many fields, as the mathematical framework can be adapted in several ways. A candidate application can be faced considering the features of RBF:  multidimensional space (including 2D and 3D), numerous radial functions available, global and compact support, interpolation/regression. This great flexibility makes RBF attractive – and their great potential has only been partially discovered. This is because of the difficulty in taking a first step toward RBF as they are not commonly part of engineers’ cultural background, but also due to the numerical complex...

  20. Development of a Radial Deconsolidation Method

    Energy Technology Data Exchange (ETDEWEB)

    Helmreich, Grant W. [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Montgomery, Fred C. [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Hunn, John D. [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States)

    2015-12-01

    A series of experiments have been initiated to determine the retention or mobility of fission products* in AGR fuel compacts [Petti, et al. 2010]. This information is needed to refine fission product transport models. The AGR-3/4 irradiation test involved half-inch-long compacts that each contained twenty designed-to-fail (DTF) particles, with 20-μm thick carbon-coated kernels whose coatings were deliberately fabricated such that they would crack under irradiation, providing a known source of post-irradiation isotopes. The DTF particles in these compacts were axially distributed along the compact centerline so that the diffusion of fission products released from the DTF kernels would be radially symmetric [Hunn, et al. 2012; Hunn et al. 2011; Kercher, et al. 2011; Hunn, et al. 2007]. Compacts containing DTF particles were irradiated at Idaho National Laboratory (INL) at the Advanced Test Reactor (ATR) [Collin, 2015]. Analysis of the diffusion of these various post-irradiation isotopes through the compact requires a method to radially deconsolidate the compacts so that nested-annular volumes may be analyzed for post-irradiation isotope inventory in the compact matrix, TRISO outer pyrolytic carbon (OPyC), and DTF kernels. An effective radial deconsolidation method and apparatus appropriate to this application has been developed and parametrically characterized.

  1. Uso de manometria computadorizada para estudo do espasmo do segmento faringoesofágico em pacientes com voz traqueoesofágica inadequada antes e após aplicação de toxina botulínica Computerized manometry use to evaluate spasm in pharyngoesophageal segment in patients with poor tracheoesophageal speech before and after treatment with botulinum toxin

    Directory of Open Access Journals (Sweden)

    Carlos T. Chone

    2009-04-01

    Full Text Available Voz traqueoesofágica (VTE com prótese fonatória (PF é método eficaz e reproduzível na reabilitação vocal após laringectomia total (LT, impedida pelo espasmo do segmento faringoesofágico (SFE. A manometria computadorizada (MC é novo método objetivo e direto de avaliação do SFE. OBJETIVO: Análise objetiva do espasmo do SFE, com MC, antes e após aplicação de toxina botulínica (TB. DESENHO DO ESTUDO: Prospectivo clínico. MATERIAL E MÉTODOS: Análise de oito pacientes consecutivos submetidos à LT com VTE e PF, sem emissão vocal, com espasmo do SFE à videofluoroscopia, considerado padrão ouro para detecção de espasmo. Todos trataram o espasmo com injeção de 100 unidades de TB no SFE. Avaliação constituiu-se de videofluoroscopia e MC do SFE, antes e após aplicação de TB. RESULTADOS: Houve diminuição na pressão do SFE à MC, após injeção de TB em todos. A média de pressão do SFE à MC, nos oito pacientes, antes da aplicação de TB foi de 25.36 mmHg e após foi de 14.31 mmHg (p=0,004. Houve emissão vocal sem esforço e melhora do espasmo do SFE à videofluoroscopia após o uso da TB. CONCLUSÃO: Foi observada diminuição na pressão do SFE após injeção da TB à MC em todos os pacientes, com melhora do espasmo à videofluoroscopia.Tracheoesophageal voice (TEV with voice prosthesis (VP is an efficient and reproducible method used in vocal rehabilitation after total laryngectomy (TL, prevented by spasms in the pharyngoesophageal segment (PES. Computerized Manometry (CM is a new, direct and objective method used to assess the PES. AIM: to carry out an objective analysis of the PES, with CM, before and after the injection of botulinum toxin (BT. STUDY DESIGN: clinical-prospective. MATERIALS AND METHODS: analysis of eight patients consecutively submitted to TL with TEV and VP, without vocal emission, with PES spasms seen through videofluoroscopy, considered the gold standard for spasm detection. All had their

  2. Analysis of radial vibrations of poroelastic circular cylindrical shells ...

    African Journals Online (AJOL)

    Waves propagating in radial direction of a poroelastic circular cylinder are termed as radial vibrations. Radial vibrations of poroelastic circular cylindrical shell of infinite extent immersed in an inviscid elastic fluid are examined employing Biot's theory. Biot's model consists of an elastic matrix permeated by a network of ...

  3. Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.

    Science.gov (United States)

    Go, C Y; Mackay, M T; Weiss, S K; Stephens, D; Adams-Webber, T; Ashwal, S; Snead, O C

    2012-06-12

    To update the 2004 American Academy of Neurology/Child Neurology Society practice parameter on treatment of infantile spasms in children. MEDLINE and EMBASE were searched from 2002 to 2011 and searches of reference lists of retrieved articles were performed. Sixty-eight articles were selected for detailed review; 26 were included in the analysis. RECOMMENDATIONS were based on a 4-tiered classification scheme combining pre-2002 evidence and more recent evidence. There is insufficient evidence to determine whether other forms of corticosteroids are as effective as adrenocorticotropic hormone (ACTH) for short-term treatment of infantile spasms. However, low-dose ACTH is probably as effective as high-dose ACTH. ACTH is more effective than vigabatrin (VGB) for short-term treatment of children with infantile spasms (excluding those with tuberous sclerosis complex). There is insufficient evidence to show that other agents and combination therapy are effective for short-term treatment of infantile spasms. Short lag time to treatment leads to better long-term developmental outcome. Successful short-term treatment of cryptogenic infantile spasms with ACTH or prednisolone leads to better long-term developmental outcome than treatment with VGB. Low-dose ACTH should be considered for treatment of infantile spasms. ACTH or VGB may be useful for short-term treatment of infantile spasms, with ACTH considered preferentially over VGB. Hormonal therapy (ACTH or prednisolone) may be considered for use in preference to VGB in infants with cryptogenic infantile spasms, to possibly improve developmental outcome. A shorter lag time to treatment of infantile spasms with either hormonal therapy or VGB possibly improves long-term developmental outcomes.

  4. Proteomic analysis of adrenocorticotropic hormone treatment of an infantile spasm model induced by N-methyl-D-aspartic acid and prenatal stress.

    Directory of Open Access Journals (Sweden)

    Jing Wang

    Full Text Available Infantile spasms is an age-specific epileptic syndrome associated with poor developmental outcomes and poor response to nearly all traditional antiepileptic drugs except adrenocorticotropic hormone (ACTH. We investigated the protective mechanism of ACTH against brain damage. An infantile spasm rat model induced by N-methyl-D-aspartate (NMDA in neonate rats was used. Pregnant rats were randomly divided into the stress-exposed and the non-stress exposed groups, and their offspring were randomly divided into ACTH-treated spasm model, untreated spasm model, and control groups. A proteomics-based approach was used to detect the proteome differences between ACTH-treated and untreated groups. Gel image analysis was followed by matrix-assisted laser desorption/ionization time-of-flight mass spectrometric protein identification and bioinformatics analysis. Prenatal stress exposure resulted in more severe seizures, and ACTH treatment reduced and delayed the onset of seizures. The most significantly up-regulated proteins included isoform 1 of tubulin β-5 chain, cofilin-1 (CFL1, synaptosomal-associated protein 25, malate dehydrogenase, N(G,N(G-dimethylarginine dimethylaminohydrolase 1, annexin A3 (ANXA3, and rho GDP-dissociation inhibitor 1 (ARHGDIA. In contrast, tubulin α-1A chain was down-regulated. Three of the identified proteins, ARHGDIA, ANXA3, and CFL1, were validated using western blot analysis. ARHGDIA expression was assayed in the brain samples of five infantile spasm patients. These proteins are involved in the cytoskeleton, synapses, energy metabolism, vascular regulation, signal transduction, and acetylation. The mechanism underlying the effects of ACTH involves the molecular events affected by these proteins, and protein acetylation is the mechanism of action of the drug treatment.

  5. Proteomic Analysis of Adrenocorticotropic Hormone Treatment of an Infantile Spasm Model Induced by N-Methyl-d-Aspartic Acid and Prenatal Stress

    Science.gov (United States)

    Wang, Jing; Wang, Juan; Zhang, Ying; Yang, Guang; Zhou, Wen-Jing; Shang, Ai-Jia; Zou, Li-Ping

    2012-01-01

    Infantile spasms is an age-specific epileptic syndrome associated with poor developmental outcomes and poor response to nearly all traditional antiepileptic drugs except adrenocorticotropic hormone (ACTH). We investigated the protective mechanism of ACTH against brain damage. An infantile spasm rat model induced by N-methyl-d-aspartate (NMDA) in neonate rats was used. Pregnant rats were randomly divided into the stress-exposed and the non-stress exposed groups, and their offspring were randomly divided into ACTH-treated spasm model, untreated spasm model, and control groups. A proteomics-based approach was used to detect the proteome differences between ACTH-treated and untreated groups. Gel image analysis was followed by matrix-assisted laser desorption/ionization time-of-flight mass spectrometric protein identification and bioinformatics analysis. Prenatal stress exposure resulted in more severe seizures, and ACTH treatment reduced and delayed the onset of seizures. The most significantly up-regulated proteins included isoform 1 of tubulin β-5 chain, cofilin-1 (CFL1), synaptosomal-associated protein 25, malate dehydrogenase, N(G),N(G)-dimethylarginine dimethylaminohydrolase 1, annexin A3 (ANXA3), and rho GDP-dissociation inhibitor 1 (ARHGDIA). In contrast, tubulin α-1A chain was down-regulated. Three of the identified proteins, ARHGDIA, ANXA3, and CFL1, were validated using western blot analysis. ARHGDIA expression was assayed in the brain samples of five infantile spasm patients. These proteins are involved in the cytoskeleton, synapses, energy metabolism, vascular regulation, signal transduction, and acetylation. The mechanism underlying the effects of ACTH involves the molecular events affected by these proteins, and protein acetylation is the mechanism of action of the drug treatment. PMID:23028951

  6. Proteomic analysis of adrenocorticotropic hormone treatment of an infantile spasm model induced by N-methyl-D-aspartic acid and prenatal stress.

    Science.gov (United States)

    Wang, Jing; Wang, Juan; Zhang, Ying; Yang, Guang; Zhou, Wen-Jing; Shang, Ai-Jia; Zou, Li-Ping

    2012-01-01

    Infantile spasms is an age-specific epileptic syndrome associated with poor developmental outcomes and poor response to nearly all traditional antiepileptic drugs except adrenocorticotropic hormone (ACTH). We investigated the protective mechanism of ACTH against brain damage. An infantile spasm rat model induced by N-methyl-D-aspartate (NMDA) in neonate rats was used. Pregnant rats were randomly divided into the stress-exposed and the non-stress exposed groups, and their offspring were randomly divided into ACTH-treated spasm model, untreated spasm model, and control groups. A proteomics-based approach was used to detect the proteome differences between ACTH-treated and untreated groups. Gel image analysis was followed by matrix-assisted laser desorption/ionization time-of-flight mass spectrometric protein identification and bioinformatics analysis. Prenatal stress exposure resulted in more severe seizures, and ACTH treatment reduced and delayed the onset of seizures. The most significantly up-regulated proteins included isoform 1 of tubulin β-5 chain, cofilin-1 (CFL1), synaptosomal-associated protein 25, malate dehydrogenase, N(G),N(G)-dimethylarginine dimethylaminohydrolase 1, annexin A3 (ANXA3), and rho GDP-dissociation inhibitor 1 (ARHGDIA). In contrast, tubulin α-1A chain was down-regulated. Three of the identified proteins, ARHGDIA, ANXA3, and CFL1, were validated using western blot analysis. ARHGDIA expression was assayed in the brain samples of five infantile spasm patients. These proteins are involved in the cytoskeleton, synapses, energy metabolism, vascular regulation, signal transduction, and acetylation. The mechanism underlying the effects of ACTH involves the molecular events affected by these proteins, and protein acetylation is the mechanism of action of the drug treatment.

  7. Preventing pressure ulcers

    Science.gov (United States)

    ... Coulson I, eds. Treatment of Skin Disease: Comprehensive Therapeutic Strategies . 4th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap ... Caring for muscle spasticity or spasms Eating extra calories when sick - adults ...

  8. CT findings and prognosis of 70 full-term infants having spasm due to hypoxic ischemic encephalography following asphyxia

    International Nuclear Information System (INIS)

    Ogita, Yasutoki; Kawakami, Tadashi; Tsunei, Mikio; Ohta, Yuko; Sone, Yoshiharu; Akamatsu, Hiroshi

    1984-01-01

    Relationship between cranial CT findings and prognosis at 12 months or more after birth was studied in 70 full-term (appropriate for date and large for date) infants who had spasm due to hypoxic ischemic encephalopathy following neonatal asphyxia. There was correlation between the prognosis of the infants and neonatal CT findings showing slight and marked low density areas in the brain parenchyma. However, it was sometimes difficult to estimate the prognosis when the low density area was moderate on CT. Therefore, follow-up CT at one and six months and one year after birth was required to examine changes in low density areas for the estimation of prognosis. The prognosis was unfavorable in cases of the disease accompanied by hemorrhage in the brain parenchyma or cerebral ventricle, persistent cerebral edema on neonatal CT, and low density areas in the atrophied brain by the follow-up CT. There was no consistent relationship between subarachnoid hemorrhage and the prognosis. (Namekawa, K.)

  9. CT findings and prognosis of 70 full-term infants having spasm due to hypoxic ischemic encephalography following asphyxia

    Energy Technology Data Exchange (ETDEWEB)

    Ogita, Yasutoki; Kawakami, Tadashi; Tsunei, Mikio; Ohta, Yuko; Sone, Yoshiharu; Akamatsu, Hiroshi

    1984-07-01

    Relationship between cranial CT findings and prognosis at 12 months or more after birth was studied in 70 full-term (appropriate to for date) infants who had spasm due to hypoxic ischemic encephalopathy following neonatal asphyxia. There was correlation between the prognosis of the infants and neonatal CT findings showing slight and marked low density areas in the brain parenchyma. However, it was sometimes difficult to estimate the prognosis when the low density area was moderate on CT. Therefore, follow-up CT at one and six months and one year after birth was required to examine changes in low density areas for the estimation of prognosis. The prognosis was unfavorable in cases of the disease accompanied by hemorrhage in the brain parenchyma or cerebral ventricle, persistent cerebral edema on neonatal CT, and low density areas in the atrophied brain by the follow-up CT. There was no consistent relationship between subarachnoid hemorrhage and the prognosis. (Namekawa, K.).

  10. Convex and Radially Concave Contoured Distributions

    Directory of Open Access Journals (Sweden)

    Wolf-Dieter Richter

    2015-01-01

    Full Text Available Integral representations of the locally defined star-generalized surface content measures on star spheres are derived for boundary spheres of balls being convex or radially concave with respect to a fan in Rn. As a result, the general geometric measure representation of star-shaped probability distributions and the general stochastic representation of the corresponding random vectors allow additional specific interpretations in the two mentioned cases. Applications to estimating and testing hypotheses on scaling parameters are presented, and two-dimensional sample clouds are simulated.

  11. CFTs of SLEs: the radial case

    Energy Technology Data Exchange (ETDEWEB)

    Bauer, Michel; Bernard, Denis

    2004-03-18

    We present a relation between conformal field theories (CFT) and radial stochastic Schramm-Loewner evolutions (SLE) similar to that we previously developed for the chordal SLEs. We construct an important local martingale using degenerate representations of the Virasoro algebra. We sketch how to compute derivative exponants and the restriction martingales in this framework. In its CFT formulation, the SLE dual Fokker-Planck operator acts as the two-particle Calogero Hamiltonian on boundary primary fields and as the dilatation operator on bulk primary fields localized at the fixed point of the SLE map.

  12. Radial fractional Laplace operators and Hessian inequalities

    Science.gov (United States)

    Ferrari, Fausto; Verbitsky, Igor E.

    In this paper we deduce a formula for the fractional Laplace operator ( on radially symmetric functions useful for some applications. We give a criterion of subharmonicity associated with (, and apply it to a problem related to the Hessian inequality of Sobolev type: ∫Rn |(u| dx⩽C∫Rn -uFk[u] dx, where Fk is the k-Hessian operator on Rn, 1⩽kFerrari et al. [5] contains the extremal functions for the Hessian Sobolev inequality of X.-J. Wang (1994) [15]. This is proved using logarithmic convexity of the Gaussian ratio of hypergeometric functions which might be of independent interest.

  13. Microvascular Decompression of Facial Nerve and Pexy of the Left Vertebral Artery for Left Hemifacial Spasm: 3-Dimensional Operative Video.

    Science.gov (United States)

    Cheng, Chun-Yu; Shetty, Rakshith; Martinez, Vicente; Sekhar, Laligam N

    2018-03-29

    A 73-yr-old man presented with intractable left hemifacial spasm of 4 yr duration. Brain magnetic resonance imaging showed significant compression of left facial nerve by the left vertebral artery (VA) and anterior inferior cerebellar artery (AICA).The patient underwent a left retrosigmoid craniotomy and a microvascular decompression of the cranial nerve (CN) VII. Intraoperatively, we found that the distal AICA had a protracted subarcuate extradural course.1 This was relieved by intra/extradural dissection. The left VA and the AICA loop were compressing the root exit zone of CN VII. The VA was mobilized, and pexy into the petrosal dura was done with 8-0 nylon sutures (Ethilon Nylon Suture, Ethicon Inc, a subsidiary of Johnson & Johnson, Somerville, New Jersey). Once this was done, the lateral spread disappeared.2 The AICA loop was decompressed with 2 pieces of Teflon felt (Bard PTFE felt, Bard peripheral Vascular Inc, a subsidiary of CR Bard Inc, Temp, Arizona). After this, wave V of the brainstem auditory evoked potential (BAEP) disappeared completely, with no recovery despite the application of the nicardipine on the internal auditory artery (IAA). The IAA appeared to be stretched by the microvascular decompression. Arachnoidal dissection was done to release the CN VIII and an additional felt piece was placed to elevate the AICA loop; the BAEP recovered completely. The patient had a complete disappearance of the hemifacial spasm postoperatively, and hearing was unchanged.This 3-D video shows the technical nuances of performing a vertebropexy, release of the AICA from its extradural subarcuate course, and the surgical maneuvers in the event of an unexpected change in neuromonitoring response. The suture technique of vertebropexy is preferred to a loop technique, to avoid kinking of the VA.3Informed consent was obtained from the patient prior to the surgery that included videotaping of the procedure and its distribution for educational purposes. All relevant

  14. Confinement of ripple-trapped slowing-down ions by a radial electric field

    International Nuclear Information System (INIS)

    Herrmann, W.

    1998-03-01

    Weakly collisional ions trapped in the toroidal field ripples at the outer plasma edge can be prevented to escape the plasma due to grad B-drift by a counteracting radial electric field. This leads to an increase in the density of ripple-trapped ions, which can be monitored by the analysis of charge exchange neutrals. The minimum radial electric field E r necessary to confine ions with energy E and charge q (q=-1: charge of the electron) is E r = -E/(q * R), where R is the major radius at the measuring point. Slowing-down ions from neutral injection are usually in the right energy range to be sufficiently collisionless in the plasma edge and show the confinement by radial electric fields in the range of tens of kV/m. The density of banana ions is almost unaffected by the radial electric field. Neither in L/H- nor in H/L-transitions does the density of ripple-trapped ions and, hence, the neutral particle fluxes, show jumps in times shorter than 1 ms. According to [1,2] the response time of the density and the fluxes to a sudden jump in the radial electric field is less than 200 μs, if the halfwidth of the electric field is larger or about 2 cm. This would exclude rapid jumps in the radial electric field at the transition. Whether the halfwidth of the electric field is that large during transition cannot be decided from the measurement of the fluxes alone. (orig.)

  15. Radial Extension, Prototypicality, and Tectonic Equivalence

    Directory of Open Access Journals (Sweden)

    Shaver Stephen R.

    2018-01-01

    Full Text Available In his book “Without Metaphor, No Saving God: Theology After Cognitive Linguistics”, Robert Masson describes a metaphoric process by which newly accepted truths emerge: for example, in the assertion “Jesus is the Messiah,” Christians reconfigure the field of meanings associated with an existing concept from the Hebrew scriptures (messiah by asserting its identification with Jesus. Masson dubs this process a “tectonic equivalence” or “tectonic shift.” In this paper I build on Masson‘s work by examining some of the shifts he describes as tectonic through the lens of the cognitive linguistics concepts of radial extension and polysemy. I propose that a lasting tectonic shift may be understood as a blend creating a radial extension that substantially alters the category structure of the original source frame so that the blended space comes to be understood as a central instance of that category. Such an approach allows a fruitful analysis of the similarities and differences among three example blends: god is a rock, jesus is the messiah, and jesus is god.

  16. Asymptotic Solutions of Serial Radial Fuel Shuffling

    Directory of Open Access Journals (Sweden)

    Xue-Nong Chen

    2015-12-01

    Full Text Available In this paper, the mechanism of traveling wave reactors (TWRs is investigated from the mathematical physics point of view, in which a stationary fission wave is formed by radial fuel drifting. A two dimensional cylindrically symmetric core is considered and the fuel is assumed to drift radially according to a continuous fuel shuffling scheme. A one-group diffusion equation with burn-up dependent macroscopic coefficients is set up. The burn-up dependent macroscopic coefficients were assumed to be known as functions of neutron fluence. By introducing the effective multiplication factor keff, a nonlinear eigenvalue problem is formulated. The 1-D stationary cylindrical coordinate problem can be solved successively by analytical and numerical integrations for associated eigenvalues keff. Two representative 1-D examples are shown for inward and outward fuel drifting motions, respectively. The inward fuel drifting has a higher keff than the outward one. The 2-D eigenvalue problem has to be solved by a more complicated method, namely a pseudo time stepping iteration scheme. Its 2-D asymptotic solutions are obtained together with certain eigenvalues keff for several fuel inward drifting speeds. Distributions of the neutron flux, the neutron fluence, the infinity multiplication factor kinf and the normalized power are presented for two different drifting speeds.

  17. Radial gravitational gliding on passive margins

    Science.gov (United States)

    Cobbold, P. R.; Szatmari, P.

    1991-03-01

    Gravitational gliding of uppermost sediments down a passive margin is possible if there is a basal layer of evaporite or other soft material to allow detachment. In examples from the Gulf of Mexico and the Brazilian margin, gliding has produced three main structural domains: an uppermost domain of downdip extension; an intermediate domain of rigid gliding; and a lowermost domain of downdip contraction. Domain boundaries are established by changes in slope. In this paper, we examine three kinds of gravitational gliding, depending on the paths followed by material particles. In ideal parallel gliding, particle paths are parallel straight lines, trending downslope. This should occur where the margin is perfectly straight. In ideal radial gliding, particle paths are radii of a circle and the margin is shaped like a circular cone. Natural margins will not have ideal shapes; but divergent gliding will tend to occur off coastal salients; convergent gliding, off coastal re-entrants. A simple kinematic model based on ductile behaviour illustrates some essential features of radial gliding. Changes in radius during divergent gliding produce strike-parallel extension; during convergent gliding, they produce strike-parallel contraction. Vertical strains also differ. Divergent gliding produces an uppermost domain of strong vertical thinning, balanced by extensions in all horizontal directions. Similarly, convergent gliding produces a lowermost domain of strong vertical thickening, balanced by contractions in all horizontal directions. These deformed states cannot be restored by simple techniques based on section balancing. We have done three experiments using analogue materials: sand, to model the brittle behaviour of sediments; silicone putty, to model the ductile behaviour of basal layers of evaporite. The experiments were properly scaled to account for gravitational forces. Experiment I reproduced convergent gliding above a basement with a conical upper surface. Strike

  18. Endoscopic versus open radial artery harvest and mammario-radial versus aorto-radial grafting in patients undergoing coronary artery bypass surgery

    DEFF Research Database (Denmark)

    Carranza, Christian L; Ballegaard, Martin; Werner, Mads U

    2014-01-01

    , it is unknown whether the endoscopic technique results in fewer complications or a graft patency comparable to open harvest. When the radial artery has been harvested, there are two ways to use the radial artery as a graft. One way is sewing it onto the aorta and another is sewing it onto the mammary artery...

  19. Randomized, Single-Blind, Parallel Clinical Trial on Efficacy of Oral Prednisolone Versus Intramuscular Corticotropin: A 12-Month Assessment of Spasm Control in West Syndrome.

    Science.gov (United States)

    Wanigasinghe, Jithangi; Arambepola, Carukshi; Ranganathan, Shalini Sri; Sumanasena, Samanmali

    2017-11-01

    We earlier completed a single-blind, parallel-group, randomized clinical trial to test the null hypothesis that adrenocorticotropic hormone (ACTH) is not superior to high-dose prednisolone for short-term control of West syndrome. We now present long-term follow-up data for spasm control for individuals who completed this earlier trial. Infants with untreated West syndrome were randomized to receive 14 days of prednisolone (40 to 60 mg/day) or intramuscular long-acting ACTH (40 to 60 IU every other day). They were evaluated at three, six, and 12 months to evaluate long-term spasm control. The total number of infants treated was 97 (48 prednisolone; 49 ACTH). All completed the treatment course. Eighty-five, 82, and 76 children were available for follow-up at three, six, and 12 months. The number lost to follow-up at each interval was not statistically different. Likelihood of spasm freedom at three months was significantly higher for prednisolone (64.6%) than for ACTH (38.8%) (P = 0.01; odds ratio = 2.9; 95% confidence interval = 1.3 to 6.6). At six months (P = 0.19) and twelve months (P = 0.13), the control of spasms was not statistically different, although a trend in favor of prednisolone was documented at both of these time points (58.3% versus 44.9% for ACTH at six months and 56.2% versus 40.8% with ACTH at 12 months). After initial remission by day 14 (n = 46), the likelihood of a relapse within the next 12 months was not statistically different between the two treatment groups (P = 0.1). Control of spasms at three months was significantly better if initially treated with prednisolone. Control of spasms at six and 12 months was not significantly different despite a trend favoring prednisolone. Risk of relapse following initial remission was similar in the two groups. Copyright © 2017 Elsevier Inc. All rights reserved.

  20. MR accuracy and arthroscopic incidence of meniscal radial tears

    Energy Technology Data Exchange (ETDEWEB)

    Magee, Thomas; Shapiro, Marc; Williams, David [Department of Radiology, Neuroimaging Institute, 27 East Hibiscus Blvd., Melbourne, FL 32901 (United States)

    2002-12-01

    A meniscal radial tear is a vertical tear that involves the inner meniscal margin. The tear is most frequent in the middle third of the lateral meniscus and may extend outward in any direction. We report (1) the arthroscopic incidence of radial tears, (2) MR signs that aid in the detection of radial tears and (3) our prospective accuracy in detection of radial tears. Design and patients. Three musculoskeletal radiologists prospectively read 200 consecutive MR examinations of the knee that went on to arthroscopy by one orthopedic surgeon. MR images were assessed for location and MR characteristics of radial tears. MR criteria used for diagnosis of a radial tear were those outlined by Tuckman et al.: truncation, abnormal morphology and/or lack of continuity or absence of the meniscus on one or more MR images. An additional criterion used was abnormal increased signal in that area on fat-saturated proton density or T2-weighted coronal and sagittal images. Prospective MR readings were correlated with the arthroscopic findings.Results. Of the 200 consecutive knee arthroscopies, 28 patients had radial tears reported arthroscopically (14% incidence). MR readings prospectively demonstrated 19 of the 28 radial tears (68% sensitivity) when the criteria for diagnosis of a radial tear were truncation or abnormal morphology of the meniscus. With the use of the additional criterion of increased signal in the area of abnormal morphology on fat-saturated T2-weighted or proton density weighted sequences, the prospective sensitivity was 25 of 28 radial tears (89% sensitivity). There were no radial tears described in MR reports that were not demonstrated on arthroscopy (i.e., there were no false positive MR readings of radial tears in these 200 patients). Radial tears are commonly seen at arthroscopy. There was a 14% incidence in this series of 200 patients who underwent arthroscopy. Prospective detection of radial tears was 68% as compared with arthroscopy when the criteria as

  1. Anomalous Medial Branch of Radial Artery: A Rare Variant

    Directory of Open Access Journals (Sweden)

    Surbhi Wadhwa

    2016-10-01

    Full Text Available Radial artery is an important consistent vessel of the upper limb. It is a useful vascular access site for coronary procedures and its reliable anatomy has resulted in an elevation of radial forearm flaps for reconstructive surgeries of head and neck. Technical failures, in both the procedures, are mainly due to anatomical variations, such as radial loops, ectopic radial arteries or tortuosity in the vessel. We present a rare and a unique anomalous medial branch of the radial artery spiraling around the flexor carpi radialis muscle in the forearm with a high rising superficial palmar branch of radial artery. Developmentally it probably is a remanent of the normal pattern of capillary vessel maintenance and regression. Such a case is of importance for reconstructive surgeons and coronary interventionists, especially in view of its unique medial and deep course.

  2. Radial propagation of microturbulence in tokamaks

    International Nuclear Information System (INIS)

    Garbet, X.; Laurent, L.; Roubin, J.P.; Samain, A.

    1992-01-01

    Energy confinement time in tokamaks exhibits a clear dependence on global plasma parameters. This is not the case for transport coefficients; their dependence on local plasma parameters cannot be precisely established. The aim of the present paper is to give a possible explanation of this behaviour; turbulence propagates radially because of departure from cylindrical geometry. This implies that the turbulence level at a given point and hence transport coefficients are not only functions of local plasma parameters. A quantitative estimate of the propagation velocity is derived from a Lagrangian formalism. Two cases are considered: the effect of toroidicity and the effect of non linear mode-mode coupling. The consequences of this model are discussed. This process does not depend on the type of instability. For the sake of simplicity only electrostatic perturbations are considered

  3. Radial particle distributions in PARMILA simulation beams

    International Nuclear Information System (INIS)

    Boicourt, G.P.

    1984-03-01

    The estimation of beam spill in particle accelerators is becoming of greater importance as higher current designs are being funded. To the present, no numerical method for predicting beam-spill has been available. In this paper, we present an approach to the loss-estimation problem that uses probability distributions fitted to particle-simulation beams. The properties of the PARMILA code's radial particle distribution are discussed, and a broad class of probability distributions are examined to check their ability to fit it. The possibility that the PARMILA distribution is a mixture is discussed, and a fitting distribution consisting of a mixture of two generalized gamma distributions is found. An efficient algorithm to accomplish the fit is presented. Examples of the relative prediction of beam spill are given. 26 references, 18 figures, 1 table

  4. Aniracetam improves radial maze performance in rats.

    Science.gov (United States)

    Martin, J R; Cumin, R; Aschwanden, W; Moreau, J L; Jenck, F; Haefely, W E

    1992-01-01

    The memory enhancing effect of the pyrrolidinone derivative aniracetam was investigated in rats trained in a delayed-response task in an 8-arm radial maze. Oral administration of aniracetam (100, 200, 400, or 800 mg kg-1) 16 h and again 1 h prior to a first trial of exposure to a given configuration of 4 baited arms resulted in a significant improvement in performance during a second trial in the maze given 3 h later in which there was access to all 8 arms but only the other 4 arms were baited. The pattern of baited arms was varied daily. The performance enhancement was greatest for the highest doses. These results extend the demonstration of the cognition enhancing effects of aniracetam to a spatial memory task in rats.

  5. Hydrostatic radial bearing of centrifugal pump

    International Nuclear Information System (INIS)

    Skalicky, A.

    1976-01-01

    A hydrostatic radial pump is described characterized by the fact that part of the medium off-taken from delivery is used as a lubricating medium. Two additional bodies are placed alongside a hydrostatic bearing with coils in between them and the pump shaft; the coils have an opposite pitch. The feed channel for the hydrostatic bearing pocket is linked to delivery. The coil outlets are connected to the pump suction unit. Two rotating coils placed alongside the hydrostatic bearing will considerably simplify the communication channel design and reduce the dependence on the pump shaft deflections. The addition of another rotating coil in the close vicinity of the pump shaft or directly on the shaft further increases the efficiency. The bearing can be used in designing vertical circulating pumps for the cooling circuits of nuclear reactors. (J.B.)

  6. Radial expansion for spinning conformal blocks

    CERN Document Server

    Costa, Miguel S.; Penedones, João; Trevisani, Emilio

    2016-07-12

    This paper develops a method to compute any bosonic conformal block as a series expansion in the optimal radial coordinate introduced by Hogervorst and Rychkov. The method reduces to the known result when the external operators are all the same scalar operator, but it allows to compute conformal blocks for external operators with spin. Moreover, we explain how to write closed form recursion relations for the coefficients of the expansions. We study three examples of four point functions in detail: one vector and three scalars; two vectors and two scalars; two spin 2 tensors and two scalars. Finally, for the case of two external vectors, we also provide a more efficient way to generate the series expansion using the analytic structure of the blocks as a function of the scaling dimension of the exchanged operator.

  7. Evolving spacetimes with purely radial tension

    Directory of Open Access Journals (Sweden)

    B. Nasre Esfahani

    2000-12-01

    Full Text Available   In this study time-dependent and spherically symmetric solutions of the Einstein field equations in an anisotropic background with a purely radial tension are presented. There exist three classes of solutions,1 An open spacetime with a wormhole at its center. 2 A conical spacetime. 3 A closed spacetime. These inhomogeneous solutions are reduced to FRW spacetimes in matter-dominated era, asymptotically. Therefore, they can be used to describe local inhomogeneities that are not considered in the standard model. For the wormhole solution. it is explicity shown that the considered matter is non-exotic, that is, it does not violate the energy conditions. Also, static solutions are studied. There is only one static solution,a conical spacetime. In this case, the matter satisfies the energy condition critically.

  8. [Functional anatomy of humero-radial articulation].

    Science.gov (United States)

    Cronier, P; Moreau, P; Mercier, P; Pillet, J

    1983-03-01

    From a theoretical study combined with dissection of twelve joints and the building of a mechanical model, the authors describe the contact zones between capitulum and conoìdal zone of the humerus on one hand, and cupule and radial bevel-edge on the other, and this, during the various phases of pro-supination. In supination, the contact is essentially forward. In pronation, it is mostly backward. In each position, it is approximately the same area of the cupule which is in contact with the capitulum. The best fit is in the functional position. Major displacement occurs with almost flat cupules, displacement is minimal with hollow cupules which correspond to rounder heads.

  9. Radial-Electric-Field Piezoelectric Diaphragm Pumps

    Science.gov (United States)

    Bryant, Robert G.; Working, Dennis C.; Mossi, Karla; Castro, Nicholas D.; Mane, Pooma

    2009-01-01

    In a recently invented class of piezoelectric diaphragm pumps, the electrode patterns on the piezoelectric diaphragms are configured so that the electric fields in the diaphragms have symmetrical radial (along-the-surface) components in addition to through-the-thickness components. Previously, it was accepted in the piezoelectric-transducer art that in order to produce the out-of-plane bending displacement of a diaphragm needed for pumping, one must make the electric field asymmetrical through the thickness, typically by means of electrodes placed on only one side of the piezoelectric material. In the present invention, electrodes are placed on both sides and patterned so as to produce substantial radial as well as through-the-thickness components. Moreover, unlike in the prior art, the electric field can be symmetrical through the thickness. Tests have shown in a given diaphragm that an electrode configuration according to this invention produces more displacement than does a conventional one-sided electrode pattern. The invention admits of numerous variations characterized by various degrees of complexity. Figure 1 is a simplified depiction of a basic version. As in other piezoelectric diaphragm pumps of similar basic design, the prime mover is a piezoelectric diaphragm. Application of a suitable voltage to the electrodes on the diaphragm causes it to undergo out-of-plane bending. The bending displacement pushes a fluid out of, or pulls the fluid into, a chamber bounded partly by the diaphragm. Also as in other diaphragm pumps in general, check valves ensure that the fluid flows only in through one port and only out through another port.

  10. Radial dose for low energy heavy ion beam

    International Nuclear Information System (INIS)

    Moribayashi, Kengo

    2016-01-01

    This paper presents the effect of incident heavy ion charge on radial dose. The mean charge of a heavy ion moving in a medium decreases with decreasing energies of this ion. As a result, this ion impact ionization cross sections, which affect radial dose, also decrease. For 1 MeV/u and 2 MeV/u, the relationship between radial dose and the mean charge of an incident ion is shown. (author)

  11. Vitreous veils and radial lattice in Marshall syndrome.

    Science.gov (United States)

    Brubaker, Jacob W; Mohney, Brian G; Pulido, Jose S; Babovic-Vuksanovic, Dusica

    2008-12-01

    To report the findings of membranous vitreous veils and radial lattice in a child with Marshall syndrome. Observational case report. Retrospective review of medical records and fundus photograph of a 6-year-old boy with Marshall syndrome. Vitreoretinal findings were significant for bilateral membranous vitreous veils and radial lattice degeneration. This case demonstrates the occurrence of vitreous veils and radial lattice degeneration in patients with Marshall syndrome.

  12. Hybrid radial-cones trajectory for accelerated magnetic resonance imaging

    Science.gov (United States)

    Johnson, Kevin M.

    2016-01-01

    Purpose To design and develop ultra-short echo time k-space sampling schemes, radial-cones, which enable high sampling efficiency while maintaining compatibility with parallel imaging and compressed sensing reconstructions. Theory and Methods Radial-cones is a trajectory which samples 3D k-space utilizing a single base cone distributed along radial dimensions through a cost function based optimization. Trajectories were generated for highly undersampled, short readout sampling and compared to 3D radial sampling in point spread function (PSF) analysis, digital and physical phantoms, and initial human volunteers. Parallel imaging reconstructions were evaluated with and without the use of compressed sensing based regularization. Results Compared to 3D radial sampling, radial-cones reduced the peak value and energy of PSF aliasing. In both digital and physical phantoms, this improved sampling behavior corresponded to a reduction in the root-mean square error with a further reduction utilizing compressed sensing. A slight increase in noise and corresponding increase in apparent resolution was observed with radial-cones. In in-vivo feasibility testing, radial-cones reconstructed images has markedly lower apparent artifacts. Ultimate gains in imaging performance were limited by off-resonance blurring. Conclusion Radial-cones is an efficient Non-Cartesian sampling scheme enabling short echo readout with a high level of compatibility with parallel imaging and compressed sensing. PMID:27017991

  13. Unusual presentation of a radial neck fracture in a child

    Directory of Open Access Journals (Sweden)

    Murali Poduval

    2013-01-01

    Full Text Available Fracture of the radial neck are uncommon injuries. In children, they may present as radial neck fractures, a components of forearm fracture dislocations, or as isolated fracture dislocations. Here, we present an unusual and previously undescribed variant of radial neck fracture with dislocation of the radial head to the medial side and ulnar nerve injury. The fracture dislocation was openly reduced and fixed with a small fragment plate. The fracture healed with some loss of rotational movements. At short followup of 6 months patient had useful elbow function but ulnar nerve did not recover.

  14. Intramural hemorrhage and endothelial changes in atherosclerotic coronary artery after repetitive episodes of spasm in x-ray-irradiated hypercholesterolemic pigs

    International Nuclear Information System (INIS)

    Nagasawa, K.; Tomoike, H.; Hayashi, Y.; Yamada, A.; Yamamoto, T.; Nakamura, M.

    1989-01-01

    To assess whether coronary spasm affects the progression of atherosclerosis and results in evolution of myocardial infarction, the role of coronary spasm on the fine structure of conduit coronary arteries was studied morphologically. Goettingen miniature pigs were fed a semisynthetic diet containing 2% cholesterol and 1.1% sodium cholate. One month after being on this diet, the pigs were anesthetized and the endothelium of a branch of the left coronary artery was denuded using a balloon catheter. X-ray irradiation in a dose of 1,500 rad was given twice selectively to the area denuded, after 4 and 5 months of cholesterol feeding. Five months after endothelial denudation, transient (group A) and repetitive episodes (group B) of coronary spasm were provoked by single and periodic (five times every 5 minutes) intracoronary injections of serotonin (10 micrograms/kg/injection), respectively. The extent of spasm by serotonin at the previously denuded site was 84 +/- 4% (n = 4) and 90 +/- 5% (n = 6) narrowing in groups A and B (p = NS between groups), respectively. Forty minutes after the final administration of serotonin, the left coronary artery was relaxed by nitroglycerin, and the heart was isolated and perfuse-fixed under physiological pressure. Intramural hemorrhage was noted at the spastic site in six pigs of group B but not in group A. The average percent luminal narrowing, on cross sections at the spastic site in group B, was significantly greater than in group A (56 +/- 7% vs. 23 +/- 5%, p less than 0.01). Scanning electron micrographs revealed that the endothelial lining was intact at the nonspastic site in both groups. In addition to the appearance of intercellular bridges at the spastic site in both groups, squeezing of endothelial cells and adhesion of white blood cells were present at the spastic site exclusively in group B

  15. Pseudarthrosis of radial shaft with dislocation of heads of radial and ulnar bones (case report

    Directory of Open Access Journals (Sweden)

    M. E. Puseva

    2013-01-01

    Full Text Available The authors presented a rare clinical case - the injury of forearm complicated by the formation of the pseudarthrosis of the radial shaft in combination with old dislocation of heads the radius and ulna. The differentiated approach to the choice of surgical tactics was proposed, which consists of several consistent stages: taking free autotransplant from the crest of iliac bone, resection of pseudarthrosis of radius with replacement of the bone defect by the graft for restoration of anatomic length, conducting combined strained osteosynthesis and elimination of dislocation of a head of radial and ulnar bones by transosseous osteosynthesis. The chosen treatment strategy allowed to restore the anatomy and function of the upper extremity.

  16. The Result of The Treatment of Infantile Spasm After Two Year Follow-Up Review Of 45 Cases in Ten Past Years

    Directory of Open Access Journals (Sweden)

    A Nasirian

    2001-06-01

    Full Text Available In the past ten years, 45 cases of infantile spasm treated in our center. From these patients, 15 cases received only steroid, other 15 cases only clonazepam, and the rest, a combination of clonazepam and steroid. All patients were observed for two years. This is a retrospective study, the data being extracted from patients record. A number of patients were treated in ambulatory and the others were patients of the hospital. The result of comparison of optimal control of seizures are as follow: 1 In only steroid group 27 percent of the patients were free of seizure, 2 In only clonazepam group 28 percent showed no seizure, 3 in combined clonazepam and steroid group 39% were seizure-free. Based on this trial we believe that treatment of infantile spasm with combined steroid and clonazepam is better than any of them alone. Considering these results, we believe that the treatment of infantile spasm with a combination of steroid and clonazepam is preferable to these drugs.

  17. The Fault in Their Stars-Accumulating Astrocytic Inclusions Associated With Clusters of Epileptic Spasms in Children With Global Developmental Delay.

    Science.gov (United States)

    Whitney, Robyn; AlMehmadi, Sameer; McCoy, Bláthnaid; Yau, Ivanna; Ochi, Ayako; Otsubo, Hiroshi; Weiss, Shelly K; Rutka, James; Hazrati, Lili-Naz; Snead, O Carter; Go, Cristina

    2017-08-01

    The presence of cerebral astrocytic inclusions recently has been described in a subset of children with early-onset refractory epilepsy, with or without structural brain malformations, and varying degrees of developmental delay. We describe two new individuals with epilepsy with astrocytic inclusions and suggest that in some children this disorder may represent a unique hemispheric epilepsy. We review previously reported individuals with epilepsy with astrocytic inclusions. Two children with early onset epilepsy with astrocytic inclusions had refractory clusters of epileptic spasms, developmental delay, abnormal neuroimaging, and hemispheric or diffuse interictal epileptiform discharges. In both children, the initial focal resection of the putative epileptogenic zone was unsuccessful and pathology failed to show astrocytic inclusions. Subsequently, both children underwent functional hemispherectomy due to ongoing clusters of epileptic spasms, and the presence of multilobar astrocytic inclusions was demonstrated. Postoperatively, both children have remained seizure free in the short-term with improved development. We highlight that functional hemispherectomy may be required for seizure control in a select subset of children with clusters of epileptic spasms, astrocytic inclusions, and global developmental delay. Given the small number of documented patients, however, ongoing collaboration is needed to better understand the pathophysiology of this condition and determine the optimal way to diagnose and manage these children. Crown Copyright © 2017. Published by Elsevier Inc. All rights reserved.

  18. Is muscle spasm as detected by FDG-PET a common cause of low back pain? probably not

    International Nuclear Information System (INIS)

    El-Haddad, G; Cheng, E; Kumar, R; Bhargava, A; Wintering, N; Zhuang, HM; Alavi, A; Xiu, Y

    2004-01-01

    Objectives:There are no studies reported in the literature describing the role of 18-F-fluorodeoxyglucose (FDG) positron emission tomography (PET) in low back pain (LBP). The purpose of this study was to investigate changes in metabolic activity of the soft tissues surrounding the lumbar spine in patients with a history of LBP, as well as to assess a future role for FDG PET imaging in these patients. We were specifically interested in investigating muscle spasm as the cause of LBP this population. Methods: A detailed questionnaire regarding LBP and other related medical history was given to patients just before the administration of FDG for a whole body PET study. PET scans were performed predominantly for the evaluation of known or suspected malignancies. PET scans findings and questionnaire results were independently tabulated and then compared. Results: A total of 43 patients returned completed questionnaires, 21 males, 22 females, and mean age of 52±18 years. A total of 8 patients demonstrated nonspecific mildly increased FDG activity in the region of the soft tissues and spinous processes of the lower thoracic and lumbar spine (3 patients with both acute and chronic LBP; 4 patients with chronic LBP but no acute symptoms at the time of the PET scan; 1 patient with no history of either acute or chronic LBP). In one patient with acute and chronic pain extending form the lower lumbar spine to the left hip, only increased right psoas muscle uptake was observed. Conclusion: The predominant finding in our study was mildly increased FDG activity in the soft tissues superficial to the lumbar spin. However, this activity was inconsistent, and its significance, if any, cannot be determined at this time. Since this was only a pilot study, additional subjects must be recruited. Of note is that no intense FDG activity was present in the lower back musculature of patients with either acute or chronic LBP. Therefore, muscle spasm as the cause of LBP may be overestimated as a

  19. Fuel radial design using Path Relinking; Diseno radial de combustible usando Path Relinking

    Energy Technology Data Exchange (ETDEWEB)

    Campos S, Y. [ININ, 52750 La Marquesa, Estado de Mexico (Mexico)

    2007-07-01

    The present work shows the obtained results when implementing the combinatory optimization technique well-known as Path Re linking (Re-linkage of Trajectories), to the problem of the radial design of nuclear fuel assemblies, for boiling water reactors (BWR Boiling Water Reactor by its initials in English), this type of reactors is those that are used in the Laguna Verde Nucleo electric Central, Veracruz. As in any other electric power generation plant of that make use of some fuel to produce heat and that it needs each certain time (from 12 to 14 months) to make a supply of the same one, because this it wears away or it burns, in the nucleolectric plants to this activity is denominated fuel reload. In this reload different activities intervene, among those which its highlight the radial and axial designs of fuel assemblies, the patterns of control rods and the multi cycles study, each one of these stages with their own complexity. This work was limited to study in independent form the radial design, without considering the other activities. These phases are basic for the fuel reload design and of reactor operation strategies. (Author)

  20. Neurological Study of Radial Nerve Conduction During Endoscopic Radial Artery Harvesting:An Intra‐Operative Evaluation

    Directory of Open Access Journals (Sweden)

    Gianluigi Bisleri

    2014-08-01

    Full Text Available Endoscopic radial artery harvesting (ERAH is a feasible and attractive minimally invasive approach for conduit procurement, however there have been concerns about a potential neurological damage occurring at the harvest limb site secondary to injury of the radial nerve during endoscopic harvesting. We present a case of ERAH in which we evaluated intraoperatively the characteristics of radial nerve conduction by means of electroneuromyography (ENM during harvesting. No pathological changes of nerve conduction were detected at the harvest limb site during surgery and postoperatively, thereby supporting the benefits of the endoscopic approach in terms of neurological outcomes following radial artery procurements with a less invasive approach.

  1. Design of radial reinforcement for prestressed concrete containments

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Shen, E-mail: swang@bechtel.com [Bechtel Power Corporation, 5275 Westview Drive, BP2-2C3, Frederick, MD 21703 (United States); Munshi, Javeed A., E-mail: jamunshi@bechtel.com [Bechtel Power Corporation, 5275 Westview Drive, BP2-2C3, Frederick, MD 21703 (United States)

    2013-02-15

    Highlights: ► A rigorous formulae is proposed to calculate radial stress within prestressed concrete containments. ► The proposed method is validated by finite element analysis in an illustrative practical example. ► A partially prestressed condition is more critical than a fully prestressed condition for radial tension. ► Practical design consideration is provided for detailing of radial reinforcement. -- Abstract: Nuclear containments are critical components for safety of nuclear power plants. Failure can result in catastrophic safety consequences as a result of leakage of radiation. Prestressed concrete containments have been used in large nuclear power plants with significant design internal pressure. These containments are generally reinforced with prestressing tendons in the circumferential (hoop) and meridional (vertical) directions. The curvature effect of the tendons introduces radial tensile stresses in the concrete shell which are generally neglected in the design of such structures. It is assumed that such tensile radial stresses are small as such no radial reinforcement is provided for this purpose. But recent instances of significant delaminations in Crystal River Unit 3 in Florida have elevated the need for reevaluation of the radial tension issue in prestressed containment. Note that currently there are no well accepted industry standards for design and detailing of radial reinforcement. This paper discusses the issue of radial tension in prestressed cylindrical and dome shaped structures and proposes formulae to calculate radial stresses. A practical example is presented to illustrate the use of the proposed method which is then verified by using state of art finite element analysis. This paper also provides some practical design consideration for detailing of radial reinforcement in prestressed containments.

  2. Combining axial and radial nanowire heterostructures: radial Esaki diodes and tunnel field-effect transistors.

    Science.gov (United States)

    Dey, Anil W; Svensson, Johannes; Ek, Martin; Lind, Erik; Thelander, Claes; Wernersson, Lars-Erik

    2013-01-01

    The ever-growing demand on high-performance electronics has generated transistors with very impressive figures of merit (Radosavljevic et al., IEEE Int. Devices Meeting 2009, 1-4 and Cho et al., IEEE Int. Devices Meeting 2011, 15.1.1-15.1.4). The continued scaling of the supply voltage of field-effect transistors, such as tunnel field-effect transistors (TFETs), requires the implementation of advanced transistor architectures including FinFETs and nanowire devices. Moreover, integration of novel materials with high electron mobilities, such as III-V semiconductors and graphene, are also being considered to further enhance the device properties (del Alamo, Nature 2011, 479, 317-323, and Liao et al., Nature 2010, 467, 305-308). In nanowire devices, boosting the drive current at a fixed supply voltage or maintaining a constant drive current at a reduced supply voltage may be achieved by increasing the cross-sectional area of a device, however at the cost of deteriorated electrostatics. A gate-all-around nanowire device architecture is the most favorable electrostatic configuration to suppress short channel effects; however, the arrangement of arrays of parallel vertical nanowires to address the drive current predicament will require additional chip area. The use of a core-shell nanowire with a radial heterojunction in a transistor architecture provides an attractive means to address the drive current issue without compromising neither chip area nor device electrostatics. In addition to design advantages of a radial transistor architecture, we in this work illustrate the benefit in terms of drive current per unit chip area and compare the experimental data for axial GaSb/InAs Esaki diodes and TFETs to their radial counterparts and normalize the electrical data to the largest cross-sectional area of the nanowire, i.e. the occupied chip area, assuming a vertical device geometry. Our data on lateral devices show that radial Esaki diodes deliver almost 7 times higher peak

  3. The Radial Velocity Experiment (RAVE) : First data release

    NARCIS (Netherlands)

    Steinmetz, M.; Zwitter, T.; Siebert, A.; Watson, F. G.; Freeman, K. C.; Munari, U.; Campbell, R.; Williams, M.; Seabroke, G. M.; Wyse, R. F. G.; Parker, Q. A.; Bienayme, O.; Roeser, S.; Gibson, B. K.; Gilmore, G.; Grebel, E. K.; Navarro, J. F.; Burton, D.; Cass, C. J. P.; Dawe, J. A.; Fiegert, K.; Hartley, M.; Russell, K. S.; Saunders, W.; Enke, H.; Bailin, J.; Binney, J.; Bland-Hawthorn, J.; Boeche, C.; Dehnen, W.; Eisenstein, D. J.; Evans, N. W.; Fiorucci, M.; Fulbright, J. P.; Gerhard, O.; Jauregi, U.; Kelz, A.; Mijovic, L.; Minchev, I.; Parmentier, G.; Penarrubia, J.; Quillen, A. C.; Read, M. A.; Ruchti, G.; Scholz, R. -D.; Siviero, A.; Smith, M.C.; Sordo, R.; Veltz, L.; Vidrih, S.; von Berlepsch, R.; Boyle, B. J.; Schilbach, E.; Helmi, A.

    2006-01-01

    We present the first data release of the Radial Velocity Experiment (RAVE), an ambitious spectroscopic survey to measure radial velocities and stellar atmosphere parameters (temperature, metallicity, and surface gravity) of up to one million stars using the Six Degree Field multiobject spectrograph

  4. The Radial Velocity Experiment (RAVE) : Second data release

    NARCIS (Netherlands)

    Zwitter, T.; Siebert, A.; Munari, U.; Freeman, K. C.; Siviero, A.; Watson, F. G.; Fulbright, J. P.; Wyse, R. F. G.; Campbell, R.; Seabroke, G. M.; Williams, M.; Steinmetz, M.; Bienayme, O.; Gilmore, G.; Grebel, E. K.; Helmi, A.; Navarro, J. F.; Anguiano, B.; Boeche, C.; Burton, D.; Cass, P.; Dawe, J.; Fiegert, K.; Hartley, M.; Russell, K.; Veltz, L.; Bailin, J.; Binney, J.; Bland-Hawthorn, J.; Brown, A.; Dehnen, W.; Evans, N. W.; Fiorentin, P. Re; Fiorucci, M.; Gerhard, O.; Gibson, B.; Kelz, A.; Kuijken, K.; Matijevic, G.; Minchev, I.; Parker, Q. A.; Penarrubia, J.; Quillen, A.; Read, M. A.; Reid, W.; Roeser, S.; Ruchti, G.; Scholz, R. -D.; Smith, M. C.; Sordo, R.; Tolstoi, E.; Tomasella, L.; Vidrih, S.; De Boer, E. Wylie

    We present the second data release of the Radial Velocity Experiment ( RAVE), an ambitious spectroscopic survey to measure radial velocities and stellar atmosphere parameters ( temperature, metallicity, surface gravity, and rotational velocity) of up to one million stars using the 6 dF multi-object

  5. Regression equations for the estimation of radial length from its ...

    African Journals Online (AJOL)

    HD) were measured using digital vernier caliper while the circumference of the radial head and the circumference at the radial tuberosity were measured using an anthropometric tape. Pearson correlation and Persian regression were used to derive the liner regression equations for the measured parameters that showed a ...

  6. The radial scar in contrast-enhanced MR mammography

    International Nuclear Information System (INIS)

    Baum, F.; Fischer, U.; Fuezesi, L.; Obenauer, S.; Vosshenrich, R.; Grabbe, E.

    2000-01-01

    Introduction: In patients with the mammographic findings of a radial scar, contrast enhanced (CE) MR mammography was evaluated in a retrospective study. Material and methods: In 24 women with radial opacities and black star configurations, CE MR mammography was performed. Examinations were done on a 1.5 T system using bilateral superficial coil (2D technique, T 1 -weighted FLASH-sequence, T R 336 ms, T E 5 ms, FA 90 ). Findings in mammography and MR mammography were compared with the histological results. Results: 15 radial scars (including 4 with additional ADH) and 9 carcinomas (6 in co-existence with a radial scar) presenting with a diameter of 3 mm to 13 mm were evaluated. There was no evidence of malignancy in MRI in 12 to 15 radial scars. In MR mammography 6 of the carcinomas fulfilled the criteria for malignant tumors. There were 3 borderline cases (scored 3 points) corresponding histologically to 1 radial scar, and to 2 false positives and 1 false negative. Conclusion: CE MR mammography is superior to other imaging modalities in the differentiation between radial scars and carcinomas. However, lesions suggestive of radial scars have to be removed surgically. (orig.) [de

  7. Incidence and Outcome of the Radial Nerve Injury following Open ...

    African Journals Online (AJOL)

    Background: Radial nerve injury is the most common peripheral nerve injury associated with humeral shaft fracture and can result in significant motor impairment of the arm and the wrist. Objectives: To evaluate the incidence, pattern and outcome of the radial nerve injury following open fracture of the humerus. Material and ...

  8. Radial Color Gradient in a Globular Cluster 1. M68

    Directory of Open Access Journals (Sweden)

    Sukyoung Yi

    1990-12-01

    Full Text Available Stars in M68 from the observed color-magnitude diagrams with CCD were integrated to find any radial gradient. The result shows that M68 has a slightly bluer core. The main cause of these calculated radial color variations seems to come from the random distribution of giants.

  9. Modelling and analysis of radial thermal stresses and temperature ...

    African Journals Online (AJOL)

    A theoretical investigation has been undertaken to study operating temperatures, heat fluxes and radial thermal stresses in the valves of a modern diesel engine with and without air-cavity. Temperatures, heat fluxes and radial thermal stresses were measured theoretically for both cases under all four thermal loading ...

  10. Humero-radial synostosis with ulnar defects in sibs.

    Science.gov (United States)

    Ramer, J C; Ladda, R L

    1989-06-01

    Sibs with virtually identical humero-radial synostosis (HRS) are presented and compared with 17 previously reported cases from the literature of recessively transmitted HRS. The range of anomalies described includes (in addition to humero-radial synostosis) ulnar hypoplasia, patellar hypoplasia, and chronic glomerulo-nephritis.

  11. Radiographic study of distal radial physeal closure in thoroughbred horses

    International Nuclear Information System (INIS)

    Vulcano, L.C.; Mamprim, M.J.; Muniz, L.M.R.; Moreira, A.F.; Luna, S.P.L.

    1997-01-01

    Monthly radiography was performed to study distal radial physeal closure in ten male and ten female Throughbred horses. The height, thoracic circumference and metacarpus circumference were also measured, Distal radial physeal closure time was sooner in females than males, and took 701 +/- 37 and 748 +/- 55 days respectively

  12. Intramuscular Neurotrophin-3 normalizes low threshold spinal reflexes, reduces spasms and improves mobility after bilateral corticospinal tract injury in rats

    Science.gov (United States)

    Kathe, Claudia; Hutson, Thomas Haynes; McMahon, Stephen Brendan; Moon, Lawrence David Falcon

    2016-01-01

    Brain and spinal injury reduce mobility and often impair sensorimotor processing in the spinal cord leading to spasticity. Here, we establish that complete transection of corticospinal pathways in the pyramids impairs locomotion and leads to increased spasms and excessive mono- and polysynaptic low threshold spinal reflexes in rats. Treatment of affected forelimb muscles with an adeno-associated viral vector (AAV) encoding human Neurotrophin-3 at a clinically-feasible time-point after injury reduced spasticity. Neurotrophin-3 normalized the short latency Hoffmann reflex to a treated hand muscle as well as low threshold polysynaptic spinal reflexes involving afferents from other treated muscles. Neurotrophin-3 also enhanced locomotor recovery. Furthermore, the balance of inhibitory and excitatory boutons in the spinal cord and the level of an ion co-transporter in motor neuron membranes required for normal reflexes were normalized. Our findings pave the way for Neurotrophin-3 as a therapy that treats the underlying causes of spasticity and not only its symptoms. DOI: http://dx.doi.org/10.7554/eLife.18146.001 PMID:27759565

  13. Immune-mediated steroid-responsive epileptic spasms and epileptic encephalopathy associated with VGKC-complex antibodies.

    Science.gov (United States)

    Suleiman, Jehan; Brenner, Tanja; Gill, Deepak; Troedson, Christopher; Sinclair, Adriane J; Brilot, Fabienne; Vincent, Angela; Lang, Bethan; Dale, Russell C

    2011-11-01

    Autoantibodies that bind to voltage-gated potassium-channel complex proteins (VGKC-complex antibodies) occur frequently in adults with limbic encephalitis presenting with cognitive impairment and seizures. Recently, VGKC-complex antibodies have been described in a few children with limbic encephalitis, and children with unexplained encephalitis presenting with status epilepticus. We report a case of infantile-onset epileptic spasms and developmental delay compatible with epileptic encephalopathy. Our patient was a female infant, aged 4 months at presentation. She had evidence of immune activation in the central nervous system with elevated cerebrospinal fluid neopterin and mirrored oligoclonal bands, which prompted testing for autoantibodies. VGKC-complex antibodies were elevated (201 pmol/L, normaltreatment, which was started late in the disease course. On review at 13 months of age, her development was consistent with an age of 5 to 6 months. These results suggest that VGKC-complex antibodies might represent a marker of immune therapy responsiveness in a subgroup of patients with infantile epileptic encephalopathy. © The Authors. Developmental Medicine & Child Neurology © 2011 Mac Keith Press.

  14. Asian over-representation among patients with hemifacial spasm compared to patients with cranial-cervical dystonia.

    Science.gov (United States)

    Wu, Yuncheng; Davidson, Anthony L; Pan, Tianhong; Jankovic, Joseph

    2010-11-15

    Hemifacial spasm (HFS) is a common movement disorder, but its prevalence in different populations has not been elucidated. We reviewed all patients with HFS currently followed at the Baylor College of Medicine Movement Disorders Clinic and compared their demographic and clinical data with a control group of patients with cranial-cervical dystonia (CD). In contrast to patients with CD (N=145, mean age 48.64±13.61 years), of whom 117 (80.69%) were Caucasians, 13 (8.97%) Hispanic, 10 (6.90%) African-American, and 5 (3.45%) were of Asian origin, there were 81 (61.36%) Caucasians, 24 (18.18%) Hispanic, 13 (9.85%) African-Americans, and 14 (10.61%) Asians in the HFS group (N=132, mean age 49.33±13.25). Although there was no statistical difference in the age and gender distribution between the two groups, the frequency of Asians in HFS group was 3.1 times higher than that in CD group (Pmovement disorder. Copyright © 2010 Elsevier B.V. All rights reserved.

  15. THE NIRSPEC ULTRACOOL DWARF RADIAL VELOCITY SURVEY

    International Nuclear Information System (INIS)

    Blake, Cullen H.; Charbonneau, David; White, Russel J.

    2010-01-01

    We report the results of an infrared Doppler survey designed to detect brown dwarf and giant planetary companions to a magnitude-limited sample of ultracool dwarfs. Using the NIRSPEC spectrograph on the Keck II telescope, we obtained approximately 600 radial velocity (RV) measurements over a period of six years of a sample of 59 late-M and L dwarfs spanning spectral types M8/L0 to L6. A subsample of 46 of our targets has been observed on three or more epochs. We rely on telluric CH 4 absorption features in Earth's atmosphere as a simultaneous wavelength reference and exploit the rich set of CO absorption features found in the K-band spectra of cool stars and brown dwarfs to measure RVs and projected rotational velocities. For a bright, slowly rotating M dwarf standard we demonstrate an RV precision of 50 m s -1 and for slowly rotating L dwarfs we achieve a typical RV precision of approximately 200 m s -1 . This precision is sufficient for the detection of close-in giant planetary companions to mid-L dwarfs as well as more equal mass spectroscopic binary systems with small separations (a +0.7 -0.6 Gyr, similar to that of nearby sun-like stars. We simulate the efficiency with which we detect spectroscopic binaries and find that the rate of tight (a +8.6 -1.6 %, consistent with recent estimates in the literature of a tight binary fraction of 3%-4%.

  16. Design of radial neutron spectrometer for ITER

    International Nuclear Information System (INIS)

    Nishitani, Takeo; Kasai, Satoshi; Iguchi, Tetsuo; Ebisawa, Katsuyuki; Kita, Yoshio.

    1996-09-01

    We designed the radial neutron spectrometer using a new type DT neutron spectrometer base on a recoil proton counter-telescope technique aiming ion temperature measurement for ITER. The neutron spectrometer will be installed on the well-collimated neutron beam line. A large-area recoil proton emitter is placed in parallel to the incident neutron beam and a micro-channel collimating plates are inserted between the radiator and the recoil proton detectors away from the neutron beam in order to limit the scattering angle of protons to the proton detectors. Here a very thin polyethylene film and a silicon surface barrier detector are employed as the radiator and proton detector, respectively. The energy resolution and detection efficiency are estimated to be 2.5% and 1x10 -5 counts/(n/cm 2 ), respectively for DT neutron through Monte Carlo calculations. Five units of the spectrometers will be installed just out side the bio-shield and consist a fun array using penetrations inside the bio-shield and a pre-collimator in the horizontal port. The life time of the proton detectors is estimated to be about one year in the Basic Performance Phase of ITER by neutron transport calculations using MCNP Monte Carlo code. The necessary R and D items and the design work were identified. (author)

  17. Elbow joint kinematics after excision of the radial head

    DEFF Research Database (Denmark)

    Jensen, Steen Lund; Olsen, Bo Sanderhoff; Søjbjerg, Jens Ole

    1999-01-01

    The contribution of the radial head to elbow joint kinematics was studied in 7 osteoligamentous elbow preparations. During unloaded flexion and extension, radial head excision induced a maximum varus displacement of 1.6 degrees with 20 degrees of joint flexion and a maximum external rotation of 3.......2 degrees at 110 degrees of flexion. With application of a 0.75-Nm load, radial head excision induced a maximum laxity of 3.3 degrees at 20 degrees of flexion in forced varus and a maximum laxity of 8.9 degrees at 10 degrees of flexion in forced external rotation. No laxity was observed in forced valgus...... or internal rotation. The results were independent of the rotation of the forearm. This study indicates that the radial head acts as stabilizer to the elbow joint in forced varus and in forced external rotation. The results suggest that fractures of the radial head cannot be treated by simple excision without...

  18. Safety and effectiveness of hormonal treatment versus hormonal treatment with vigabatrin for infantile spasms (ICISS): a randomised, multicentre, open-label trial.

    Science.gov (United States)

    O'Callaghan, Finbar J K; Edwards, Stuart W; Alber, Fabienne Dietrich; Hancock, Eleanor; Johnson, Anthony L; Kennedy, Colin R; Likeman, Marcus; Lux, Andrew L; Mackay, Mark; Mallick, Andrew A; Newton, Richard W; Nolan, Melinda; Pressler, Ronit; Rating, Dietz; Schmitt, Bernhard; Verity, Christopher M; Osborne, John P

    2017-01-01

    Infantile spasms constitutes a severe infantile epilepsy syndrome that is difficult to treat and has a high morbidity. Hormonal therapies or vigabatrin are the most commonly used treatments. We aimed to assess whether combining the treatments would be more effective than hormonal therapy alone. In this multicentre, open-label randomised trial, 102 hospitals (Australia [three], Germany [11], New Zealand [two], Switzerland [three], and the UK [83]) enrolled infants who had a clinical diagnosis of infantile spasms and a hypsarrhythmic (or similar) EEG no more than 7 days before enrolment. Participants were randomly assigned (1:1) by a secure website to receive hormonal therapy with vigabatrin or hormonal therapy alone. If parents consented, there was an additional randomisation (1:1) of type of hormonal therapy used (prednisolone or tetracosactide depot). Block randomisation was stratified for hormonal treatment and risk of developmental impairment. Parents and clinicians were not masked to therapy, but investigators assessing electro-clinical outcome were masked to treatment allocation. Minimum doses were prednisolone 10 mg four times a day or intramuscular tetracosactide depot 0·5 mg (40 IU) on alternate days with or without vigabatrin 100 mg/kg per day. The primary outcome was cessation of spasms, which was defined as no witnessed spasms on and between day 14 and day 42 from trial entry, as recorded by parents and carers in a seizure diary. Analysis was by intention to treat. The trial is registered with The International Standard Randomised Controlled Trial Number (ISRCTN), number 54363174, and the European Union Drug Regulating Authorities Clinical Trials (EUDRACT), number 2006-000788-27. Between March 7, 2007, and May 22, 2014, 766 infants were screened and, of those, 377 were randomly assigned to hormonal therapy with vigabatrin (186) or hormonal therapy alone (191). All 377 infants were assessed for the primary outcome. Between days 14 and 42 inclusive no

  19. High dose (4 mg/kg/day) versus usual dose (2 mg/kg/day) oral prednisolone for treatment of infantile spasms: an open-label, randomized controlled trial.

    Science.gov (United States)

    Chellamuthu, Prabaharan; Sharma, Suvasini; Jain, Puneet; Kaushik, Jaya Shankar; Seth, Anju; Aneja, Satinder

    2014-10-01

    This study aimed to test the hypothesis that high-dose prednisolone (4 mg/kg/day) may be more efficacious than usual-dose (2 mg/kg/day) prednisolone for spasm resolution at 14-days in children with infantile spasms. This was a randomized, open-label-trial conducted at a tertiary-level-hospital from February-2012 to March-2013. Children aged 3-months to 2-years presenting with infantile spasms in clusters (at least 1 cluster/day) with hypsarrhythmia or its variants on EEG were enrolled. The study participants were randomized to receive either high-dose prednisolone (4 mg/kg/day) or the usual-dose (2 mg/kg/day) prednisolone. The primary outcome measure was the proportion of children who achieved spasm freedom for 48-h at day-14 after treatment initiation as per parental reports in both the groups. The adverse effects were also monitored. The study was registered with the clinicaltrials.gov (ClinicalTrials.gov Identifier: NCT01575639). Sixty-three children were randomized into the two groups with comparable baseline characteristics. The proportion of children with spasm cessation on day-14 was significantly higher in the high-dose group as compared to the usual-dose group (51.6% vs. 25%, p=0.03). The absolute risk reduction was 26.6% (95% confidence interval 11.5-41.7%) with number needed to treat being 4. The adverse effects were comparable in both the groups. High-dose prednisolone (4 mg/kg/d) was more effective than low-dose prednisolone (2mg/kg/d) in achieving spasm cessation at 14-days (as per parental reports) in children with infantile spasms. Copyright © 2014 Elsevier B.V. All rights reserved.

  20. Radial notch labralization for proximal radioulnar joint dysplasia.

    Science.gov (United States)

    Bellato, Enrico; O'Driscoll, Shawn W

    2017-07-01

    Chronic posterior subluxation or dislocation of the radial head is uncommon and difficult to treat. To restore radiocapitellar alignment, procedures such as deepening of the notch using a high-speed burr have been described, but they can result in cartilage damage. We hypothesized that a radial notch labralization using soft tissue could improve radiocapitellar tracking without violating the joint surface. A radial notch labralization was performed in 3 patients with chronic posterior subluxation of the radial head and developmental dysplasia of the radial notch in the setting of complex recurrent instability of the elbow. A soft tissue graft (typically a portion of an allograft hamstring tendon) was used to create a meniscus-like bumper posteriorly, thereby deepening the radial notch and reducing its radius of curvature. A corrective anterior opening wedge ulnar osteotomy was also performed to realign the radial head with the capitellum. At a mean follow-up of 32 months, all 3 patients were pain free and had maintained a stable joint, with a functional range of motion. Each patient gave a rating of either "Greatly Improved" or "Almost Normal" on the Summary Outcome Determination scale. Radiographs performed during the last follow-up showed improved radiocapitellar alignment. Chronic posterior subluxation or dislocation of the radial head can occur subsequent to developmental joint changes. The radial notch labralization using a soft tissue graft associated with a corrective ulnar osteotomy was successful in restoring radial head stability and avoiding cartilage damage. Copyright © 2017 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.

  1. Scaling thermal effects in radial flow

    Science.gov (United States)

    Hudspeth, R. T.; Guenther, R. B.; Roley, K. L.; McDougal, W. G.

    To adequately evaluate the environmental impact of siting nuclear waste repositories in basalt aquicludes, it is essential to know the effects on parameter identification algorithms of thermal gradients that exist in these basaltic aquicludes. Temperatures of approximately 60°C and pressures of approximately 150 atm can be expected at potential repository sites located at depths of approximately 1000 m. The phenomenon of over-recovery has been observed in some pumping tests conducted at the Hanford Nuclear Reservation located in the Pasco Basin adjacent to the Columbia River in the state of Washington, USA. This over-recovery phenomenon may possibly be due to variations in the fluid density caused by thermal gradients. To assess the potential effects of these thermal gradients on indirect parameter identification algorithms, a systematic scaling of the governing field equations is required in order to obtain dimensionless equations based on the principle of similarity. The constitutive relationships for the specific weight of the fluid and for the porosity of the aquiclude are shown to be exponentially dependent on the pressure gradient. The dynamic pressure is converted to the piezometric head and the flow equation for the piezometric head is then scaled in radial coordinates. Order-of-magnitude estimates are made for all variables in unsteady flow for a typical well test in a basaltic aquiclude. Retaining all nonlinear terms, the parametric dependency of the flow equation on the classical dimensionless thermal and hydraulic parameters is demonstrated. These classical parameters include the Batchelor, Fourier, Froude, Grashof, and Reynolds Numbers associated with thermal flows. The flow equation is linearized from order-of-magnitude estimates based on these classical parameters for application in parameter identification algorithms.

  2. Radial Breathing Modes in Cosmochemistry and Meteoritics

    Science.gov (United States)

    Wilson, T.L.; Wilson, K.B.

    2009-01-01

    One area of continuing interest in cosmochemistry and meteoritics (C&M) is the identification of the nature of Q-phase, although some researchers in C&M are not reporting relevant portions of Raman spectral data. Q is the unidentified carrier of noble gases in carbonaceous chondrites (CCs). Being carbonaceous, the focus has been on any number of Q-candidates arising from the sp2 hybridization of carbon (C). These all derive from various forms of graphene, a monolayer of C atoms packed into a two-dimensional (2D) hexagonal honeycomb lattice that is the basic building block for graphitic materials of all other dimensions for sp2 allotropes of C. As a basic lattice, 2D graphene can be curled into fullerenes (0D), wrapped into carbon nanotubes or CNTs (1D), and stacked into graphite (3D). These take such additional forms as scroll-like carbon whiskers, carbon fibers, carbon onions, GPCs (graphite polyhedral crystals) [6], and GICs (graphite intercalation compounds). Although all of these have been observed in meteoritics, the issue is which can explain the Q-abundances. In brief, one or more of the 0D-3D sp2 hybridization forms of C is Q. For some Q-candidates, the radial breathing modes (RBMs) are the most important Raman active vibrational modes that exist, and bear a direct relevance to solving this puzzle. Typically in C&M they are ignored when present. Their importance is addressed here as smoking-gun signatures for certain Q-candidates and are very relevant to the ultimate identification of Q.

  3. Elongated nanostructures for radial junction solar cells.

    Science.gov (United States)

    Kuang, Yinghuan; Vece, Marcel Di; Rath, Jatindra K; Dijk, Lourens van; Schropp, Ruud E I

    2013-10-01

    In solar cell technology, the current trend is to thin down the active absorber layer. The main advantage of a thinner absorber is primarily the reduced consumption of material and energy during production. For thin film silicon (Si) technology, thinning down the absorber layer is of particular interest since both the device throughput of vacuum deposition systems and the stability of the devices are significantly enhanced. These features lead to lower cost per installed watt peak for solar cells, provided that the (stabilized) efficiency is the same as for thicker devices. However, merely thinning down inevitably leads to a reduced light absorption. Therefore, advanced light trapping schemes are crucial to increase the light path length. The use of elongated nanostructures is a promising method for advanced light trapping. The enhanced optical performance originates from orthogonalization of the light's travel path with respect to the direction of carrier collection due to the radial junction, an improved anti-reflection effect thanks to the three-dimensional geometric configuration and the multiple scattering between individual nanostructures. These advantages potentially allow for high efficiency at a significantly reduced quantity and even at a reduced material quality, of the semiconductor material. In this article, several types of elongated nanostructures with the high potential to improve the device performance are reviewed. First, we briefly introduce the conventional solar cells with emphasis on thin film technology, following the most commonly used fabrication techniques for creating nanostructures with a high aspect ratio. Subsequently, several representative applications of elongated nanostructures, such as Si nanowires in realistic photovoltaic (PV) devices, are reviewed. Finally, the scientific challenges and an outlook for nanostructured PV devices are presented.

  4. RADIAL VELOCITY MONITORING OF KEPLER HEARTBEAT STARS

    International Nuclear Information System (INIS)

    Shporer, Avi; Fuller, Jim; Isaacson, Howard; Hambleton, Kelly; Prša, Andrej; Thompson, Susan E.; Kurtz, Donald W.; Howard, Andrew W.; O’Leary, Ryan M.

    2016-01-01

    Heartbeat stars (HB stars) are a class of eccentric binary stars with close periastron passages. The characteristic photometric HB signal evident in their light curves is produced by a combination of tidal distortion, heating, and Doppler boosting near orbital periastron. Many HB stars continue to oscillate after periastron and along the entire orbit, indicative of the tidal excitation of oscillation modes within one or both stars. These systems are among the most eccentric binaries known, and they constitute astrophysical laboratories for the study of tidal effects. We have undertaken a radial velocity (RV) monitoring campaign of Kepler HB stars in order to measure their orbits. We present our first results here, including a sample of 22 Kepler HB systems, where for 19 of them we obtained the Keplerian orbit and for 3 other systems we did not detect a statistically significant RV variability. Results presented here are based on 218 spectra obtained with the Keck/HIRES spectrograph during the 2015 Kepler observing season, and they have allowed us to obtain the largest sample of HB stars with orbits measured using a single instrument, which roughly doubles the number of HB stars with an RV measured orbit. The 19 systems measured here have orbital periods from 7 to 90 days and eccentricities from 0.2 to 0.9. We show that HB stars draw the upper envelope of the eccentricity–period distribution. Therefore, HB stars likely represent a population of stars currently undergoing high eccentricity migration via tidal orbital circularization, and they will allow for new tests of high eccentricity migration theories.

  5. Radial head fracture associated with posterior interosseous nerve injury

    Directory of Open Access Journals (Sweden)

    Bernardo Barcellos Terra

    Full Text Available ABSTRACT Fractures of the radial head and radial neck correspond to 1.7-5.4% of all fractures and approximately 30% may present associated injuries. In the literature, there are few reports of radial head fracture with posterior interosseous nerve injury. This study aimed to report a case of radial head fracture associated with posterior interosseous nerve injury. CASE REPORT: A male patient, aged 42 years, sought medical care after falling from a skateboard. The patient related pain and limitation of movement in the right elbow and difficulty to extend the fingers of the right hand. During physical examination, thumb and fingers extension deficit was observed. The wrist extension showed a slight radial deviation. After imaging, it became evident that the patient had a fracture of the radial head that was classified as grade III in the Mason classification. The patient underwent fracture fixation; at the first postoperative day, thumb and fingers extension was observed. Although rare, posterior interosseous nerve branch injury may be associated with radial head fractures. In the present case, the authors believe that neuropraxia occurred as a result of the fracture hematoma and edema.

  6. Analytical design of an advanced radial turbine. [automobile engines

    Science.gov (United States)

    Large, G. D.; Finger, D. G.; Linder, C. G.

    1981-01-01

    The aerodynamic and mechanical potential of a single stage ceramic radial inflow turbine was evaluated for a high temperature single stage automotive engine. The aerodynamic analysis utilizes a turbine system optimization technique to evaluate both radial and nonradial rotor blading. Selected turbine rotor configurations were evaluated mechanically with three dimensional finite element techniques. Results indicate that exceptionally high rotor tip speeds (2300 ft/sec) and performance potential are feasible with radial bladed rotors if the projected ceramic material properties are realized. Nonradial rotors reduced tip speed requirements (at constant turbine efficiency) but resulted in a lower cumulative probability of success due to higher blade and disk stresses.

  7. Radial zoning of large LMFBRs. Part II. An analytical approach

    International Nuclear Information System (INIS)

    King, M.J.

    1975-08-01

    The optimum nuclear radial power peaking factor as a function of radial zoning is investigated for large carbide LMFBRs of the 5000 MW(t) class. Four different fuel volume fractions are considered. The two radial zones are characterized by different plutonium enrichments. The dependence of the optimum power peaking factor on the volume and enrichment ratios of outer to inner core is investigated for static conditions and for burn cycles corresponding to first core loading. A simple phenomenological model is used to determine the functional dependence of the optimum enrichment split on the ratio of inner to outer core volume

  8. Interaction of non-radially symmetric camphor particles

    Science.gov (United States)

    Ei, Shin-Ichiro; Kitahata, Hiroyuki; Koyano, Yuki; Nagayama, Masaharu

    2018-03-01

    In this study, the interaction between two non-radially symmetric camphor particles is theoretically investigated and the equation describing the motion is derived as an ordinary differential system for the locations and the rotations. In particular, slightly modified non-radially symmetric cases from radial symmetry are extensively investigated and explicit motions are obtained. For example, it is theoretically shown that elliptically deformed camphor particles interact so as to be parallel with major axes. Such predicted motions are also checked by real experiments and numerical simulations.

  9. Real-time reconstruction of sensitivity encoded radial magnetic resonance imaging using a graphics processing unit.

    Science.gov (United States)

    Sørensen, Thomas Sangild; Atkinson, David; Schaeffter, Tobias; Hansen, Michael Schacht

    2009-12-01

    A barrier to the adoption of non-Cartesian parallel magnetic resonance imaging for real-time applications has been the times required for the image reconstructions. These times have exceeded the underlying acquisition time thus preventing real-time display of the acquired images. We present a reconstruction algorithm for commodity graphics hardware (GPUs) to enable real time reconstruction of sensitivity encoded radial imaging (radial SENSE). We demonstrate that a radial profile order based on the golden ratio facilitates reconstruction from an arbitrary number of profiles. This allows the temporal resolution to be adjusted on the fly. A user adaptable regularization term is also included and, particularly for highly undersampled data, used to interactively improve the reconstruction quality. Each reconstruction is fully self-contained from the profile stream, i.e., the required coil sensitivity profiles, sampling density compensation weights, regularization terms, and noise estimates are computed in real-time from the acquisition data itself. The reconstruction implementation is verified using a steady state free precession (SSFP) pulse sequence and quantitatively evaluated. Three applications are demonstrated; real-time imaging with real-time SENSE 1) or k- t SENSE 2) reconstructions, and 3) offline reconstruction with interactive adjustment of reconstruction settings.

  10. How should children with West syndrome be efficiently and accurately investigated? Results from the National Infantile Spasms Consortium.

    Science.gov (United States)

    Wirrell, Elaine C; Shellhaas, Renée A; Joshi, Charuta; Keator, Cynthia; Kumar, Shilpi; Mitchell, Wendy G

    2015-04-01

    To prospectively evaluate the etiology of new-onset infantile spasms and evaluate the yield of genetic and metabolic investigations in those without obvious cause after initial clinical evaluation and magnetic resonance imaging (MRI). Twenty-one U.S. pediatric epilepsy centers prospectively enrolled infants with newly diagnosed West syndrome in a central database. Etiology and investigations performed within 3 months of diagnosis were documented. From June 2012 to June 2014, a total of 251 infants were enrolled (53% male). A cause was identified in 161 (64.4%) of 250 cases (genetic,14.4%; genetic-structural, 10.0%; structural-congenital, 10.8%; structural-acquired, 22.4%; metabolic, 4.8%; and infectious, 2.0%). An obvious cause was found after initial clinical assessment (history and physical examination) and/or MRI in 138 of 161, whereas further genetic and metabolic studies were revealing in another 23 cases. Of 112 subjects without an obvious cause after initial evaluation and MRI, 81 (72.3%) had undergone genetic testing, which showed a causal abnormality in 23.5% and a variant of unknown significance in 14.8%. Although metabolic studies were done in the majority (serum, 79.5%; urine, 69.6%; and cerebrospinal fluid [CSF], 38.4%), these revealed an etiology in only five cases (4.5%). No correlation was found between type of health insurance (public vs. private) and either genetic or metabolic testing. Clinical evaluation and MRI provide a specific diagnosis in 55% of children presenting with West syndrome. We propose that a cost-effective workup for those without obvious cause after initial clinical evaluation and MRI includes an array comparative genomic hybridization (aCGH) followed by an epilepsy gene panel if the microarray is not definitive, serum lactate, serum amino acids, and urine organic acids. Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.

  11. Radial furnace shows promise for growing straight boron carbide whiskers

    Science.gov (United States)

    Feingold, E.

    1967-01-01

    Radial furnace, with a long graphite vaporization tube, maintains a uniform thermal gradient, favoring the growth of straight boron carbide whiskers. This concept seems to offer potential for both the quality and yield of whiskers.

  12. Traumatic radial artery aneurysm at National Orthopaedic Hospital ...

    African Journals Online (AJOL)

    aneurysms within the period. All the three patients had excision of the aneurysms and reversed cephalic vein interposition graft. The outcomes were satisfactory. Keywords: radial artery aneurysm, vein graft. Nigerian Journal of Plastic Surgery Vol.

  13. Reinitiation of mRNA translation in a patient with X-linked infantile spasms with a protein-truncating variant in ARX.

    Science.gov (United States)

    Moey, Ching; Topper, Scott; Karn, Mary; Johnson, Amy Knight; Das, Soma; Vidaurre, Jorge; Shoubridge, Cheryl

    2016-05-01

    Mutations in the Aristaless-related homeobox gene (ARX) lead to a range of X-linked intellectual disability phenotypes, with truncating variants generally resulting in severe X-linked lissencephaly with ambiguous genitalia (XLAG), and polyalanine expansions and missense variants resulting in infantile spasms. We report two male patients with early-onset infantile spasms in whom a novel c.34G>T (p.(E12*)) variant was identified in the ARX gene. A similar variant c.81C>G (p.(Y27*)), has previously been described in two affected cousins with early-onset infantile spasms, leading to reinitiation of ARX mRNA translation resulting in an N-terminal truncated protein. We show that the novel c.34G>T (p.(E12*)) variant also reinitiated mRNA translation at the next AUG codon (c.121-123 (p.M41)), producing the same N-terminally truncated protein. The production of both of these truncated proteins was demonstrated to be at markedly reduced levels using in vitro cell assays. Using luciferase reporter assays, we demonstrate that transcriptional repression capacity of ARX was diminished by both the loss of the N-terminal corepressor octapeptide domain, as a consequence of truncation, and the marked reduction in mutant protein expression. Our study indicates that premature termination mutations very early in ARX lead to reinitiation of translation to produce N-terminally truncated protein at markedly reduced levels of expression. We conclude that even low levels of N-terminally truncated ARX is sufficient to improve the patient's phenotype compared with the severe phenotype of XLAG that includes malformations of the brain and genitalia normally seen in complete loss-of-function mutations in ARX.

  14. Radiocapitellar contact characteristics during prosthetic radial head subluxation.

    Science.gov (United States)

    Sahu, Dipit; Fitzsimmons, James S; Thoreson, Andrew R; An, Kai-Nan; O'Driscoll, Shawn W

    2017-01-01

    Metallic radial head prostheses are often used in the management of comminuted radial head fractures and elbow instability. We hypothesized that during radiocapitellar subluxation, the contact pressure characteristics of an anatomic radial head prosthesis will more closely mimic those of the native radial head compared with a monopolar circular or a bipolar circular radial head design. With use of 6 fresh frozen cadaver elbows, mean radiocapitellar contact pressures, contact areas, and peak pressures of the native radial head were assessed at 0, 2, 4, and 6 mm of posterior subluxation. These assessments were repeated after the native radial head was replaced with anatomic, monopolar circular and bipolar circular prostheses. The joint contact pressures increased with the native and the prosthetic radial head subluxation. The mean contact pressures for the native radial head and anatomic prosthesis increased progressively and significantly from 0 to 6 mm of subluxation (native, 0.6 ± 0.0 MPa to 1.9 ± 0.2 MPa; anatomic, 0.7 ± 0.0 MPa to 2.1 ± 0.3 MPa; P < .0001). The contact pressures with the monopolar and bipolar prostheses were significantly higher at baseline and did not change significantly further with subluxation (monopolar, 2.0 ± 0.1 MPa to 2.2 ± 0.2 MPa [P = .31]; bipolar, 1.7 ± 0.1 MPa to 1.9 ± 0.1 MPa [P = .12]). The pattern of increase in contact pressures with the anatomic prosthesis mimicked that of the native radial head. Conversely, the circular prostheses started out with higher contact pressures that stayed elevated. The articular surface design of a radial head prosthesis is an important determinant of joint contact pressures. Copyright © 2017 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.

  15. Radial, renal and craniofacial anomalies: Baller-Gerold syndrome

    Directory of Open Access Journals (Sweden)

    Murthy Jyotsna

    2008-01-01

    Full Text Available The Baller-Gerold syndrome is a rare syndrome with very few cases published in literature. Craniosynostosis and radial aplasia are striking features, easy to diagnose. However, there are many differential diagnoses. Often, the question raised is whether the Baller-Gerald syndrome is a distinct entity. We report a patient with findings of craniosynostosis and radial aplasia consistent with the diagnosis of the Baller-Gerold syndrome. Genotypic heterogeneity could possibly underlie the phenotypic variability exhibited by these cases.

  16. Anterior transposition of the radial nerve--a cadaveric study.

    Science.gov (United States)

    Yakkanti, Madhusudhan R; Roberts, Craig S; Murphy, Joshua; Acland, Robert D

    2008-01-01

    The radial nerve is at risk during the posterior plating of the humerus. The purpose of this anatomic study was to assess the extent of radial nerve dissection required for anterior transposition through the fracture site (transfracture anterior transposition). A cadaver study was conducted approaching the humerus by a posterior midline incision. The extent of dissection of the nerve necessary for plate fixation of the humerus fracture was measured. An osteotomy was created to model a humeral shaft fracture at the spiral groove (OTA classification 12-A2, 12-A3). The radial nerve was then transposed anterior to the humeral shaft through the fracture site. The additional dissection of the radial nerve and the extent of release of soft tissue from the humerus shaft to achieve the transposition were measured. Plating required a dissection of the radial nerve 1.78 cm proximal and 2.13 cm distal to the spiral groove. Transfracture anterior transposition of the radial nerve required an average dissection of 2.24 cm proximal and 2.68 cm distal to the spiral groove. The lateral intermuscular septum had to be released for 2.21 cm on the distal fragment to maintain laxity of the transposed nerve. Transfracture anterior transposition of the radial nerve before plating is feasible with dissection proximal and distal to the spiral groove and elevation of the lateral intermuscular septum. Potential clinical advantages of this technique include enhanced fracture site visualization, application of broader plates, and protection of the radial nerve during the internal fixation.

  17. Radial densities of nuclear matter and charge via moment methods

    International Nuclear Information System (INIS)

    Dalton, B.J.

    1980-01-01

    In this report I will discuss some initial efforts in our program to describe radial densities of nuclear matter and charge with the use of moment methods. A brief introduction to trace reduction formulas and computation problems along with proposed methods to overcome them will be given. This will be followed by a general discussion on computation of expectation values using moment methods with particular emphasis on formulation for the radial density applications

  18. Radial basis function neural networks applied to NASA SSME data

    Science.gov (United States)

    Wheeler, Kevin R.; Dhawan, Atam P.

    1993-01-01

    This paper presents a brief report on the application of Radial Basis Function Neural Networks (RBFNN) to the prediction of sensor values for fault detection and diagnosis of the Space Shuttle's Main Engines (SSME). The location of the Radial Basis Function (RBF) node centers was determined with a K-means clustering algorithm. A neighborhood operation about these center points was used to determine the variances of the individual processing notes.

  19. Diverse corrugation pattern in radially shrinking carbon nanotubes

    OpenAIRE

    Shima, Hiroyuki; Sato, Motohito; Iiboshi, Kohtaroh; Ghosh, Susanta; Arroyo, Marino

    2010-01-01

    Stable cross sections of multiwalled carbon nanotubes subjected to electron-beam irradiation are investigated in the realm of the continuum mechanics approximation. The self-healing nature of sp2 graphitic sheets implies that selective irradiation of the outermost walls causes their radial shrinkage with the remaining inner walls undamaged. The shrinking walls exert high pressure on the interior part of nanotubes, yielding a wide variety of radial-corrugation patterns (i.e. circumferential...

  20. Luxation of the radial carpal bone in a cat

    International Nuclear Information System (INIS)

    Pitcher, G.D.C.

    1996-01-01

    A case of radial carpal bone luxation in the cat and its management is described. Open reduction was performed and surgically maintained, in combination with repair of rupture of the short radial collateral ligament and joint capsule. The carpus was supported for one month following surgery by application of transarticular external fixation. Four months after treatment the cat was sound, despite evidence of degenerative joint disease. The mechanism of luxation appears to be analogous to that seen in the dog

  1. Nuclear reactor with self-orificing radial blanket

    International Nuclear Information System (INIS)

    Bishop, A.A.; Weiss, E.H.G.; Engel, F.C.

    1978-01-01

    The peripheral blanket of a breeder reactor requires a coolant flow rate which is a varying fraction of that of the central core region. A self-orificing blanket cooling structure which is characterized by a predominance of radial coolant flow, generated by the pressure difference across the blanket, is utilized to supply the necessary cooling. The blanket fuel assemblies are surrounded by perforated cans to allow for radial crossflow through the blanket region

  2. Construction of global Lyapunov functions using radial basis functions

    CERN Document Server

    Giesl, Peter

    2007-01-01

    The basin of attraction of an equilibrium of an ordinary differential equation can be determined using a Lyapunov function. A new method to construct such a Lyapunov function using radial basis functions is presented in this volume intended for researchers and advanced students from both dynamical systems and radial basis functions. Besides an introduction to both areas and a detailed description of the method, it contains error estimates and many examples.

  3. Introducing radiality constraints in capacitated location-routing problems

    Directory of Open Access Journals (Sweden)

    Eliana Mirledy Toro Ocampo

    2017-03-01

    Full Text Available In this paper, we introduce a unified mathematical formulation for the Capacitated Vehicle Routing Problem (CVRP and for the Capacitated Location Routing Problem (CLRP, adopting radiality constraints in order to guarantee valid routes and eliminate subtours. This idea is inspired by formulations already employed in electric power distribution networks, which requires a radial topology in its operation. The results show that the proposed formulation greatly improves the convergence of the solver.

  4. Control of radial propagation and polarity in a plasma jet in surrounding Ar

    Science.gov (United States)

    Gong, W.; Yue, Y.; Ma, F.; Yu, F.; Wan, J.; Nie, L.; Bazaka, K.; Xian, Y.; Lu, X.; Ostrikov, K.

    2018-01-01

    In recent years, the use of shielding gas to prevent the diffusion of the ambient air, particularly oxygen and nitrogen species, into the effluent of the atmospheric pressure plasma jet, and thus control the nature of chemical species used in the plasma treatment has increased. In this paper, the radial propagation of a plasma jet in ambient Ar is examined to find the key determinants of the polarity of plasma jets. The dynamics of the discharge reveal that the radial diffusion discharge is a special phenomenon observed only at the falling edge of the pulses. The radial transport of electrons, which is driven by the radial component of the applied electric field at the falling edge of the pulse, is shown to play an important role in increasing the seed electron density in the surrounding Ar. This result suggests a method to provide seed electrons at atmospheric pressure with a negative discharge. The polarity of the plasma jet is found to be determined by the pulse width rather than the polarity of the applied voltage, as it dictates the relative difference in the intensity of the two discharges in a single pulse, where the stronger discharge in a pulse dominates the behavior of the plasma jet. Accordingly, a method to control the polarity of a plasma jet through varying the pulse width is developed. Since plasma jets of different polarities differ remarkably in terms of their characteristics, the method to control the polarity reported in this paper will be of use for such applications as plasma-enhanced processing of materials and plasma biomedicine.

  5. Control of radial propagation and polarity in a plasma jet in surrounding Ar

    KAUST Repository

    Gong, W.

    2018-01-08

    In recent years, the use of shielding gas to prevent the diffusion of the ambient air, particularly oxygen and nitrogen species, into the effluent of the atmospheric pressure plasma jet, and thus control the nature of chemical species used in the plasma treatment has increased. In this paper, the radial propagation of a plasma jet in ambient Ar is examined to find the key determinants of the polarity of plasma jets. The dynamics of the discharge reveal that the radial diffusion discharge is a special phenomenon observed only at the falling edge of the pulses. The radial transport of electrons, which is driven by the radial component of the applied electric field at the falling edge of the pulse, is shown to play an important role in increasing the seed electron density in the surrounding Ar. This result suggests a method to provide seed electrons at atmospheric pressure with a negative discharge. The polarity of the plasma jet is found to be determined by the pulse width rather than the polarity of the applied voltage, as it dictates the relative difference in the intensity of the two discharges in a single pulse, where the stronger discharge in a pulse dominates the behavior of the plasma jet. Accordingly, a method to control the polarity of a plasma jet through varying the pulse width is developed. Since plasma jets of different polarities differ remarkably in terms of their characteristics, the method to control the polarity reported in this paper will be of use for such applications as plasma-enhanced processing of materials and plasma biomedicine.

  6. Channeling of protons through radial deformed carbon nanotubes

    Energy Technology Data Exchange (ETDEWEB)

    Borka Jovanović, V., E-mail: vborka@vinca.rs [Atomic Physics Laboratory (040), Vinča Institute of Nuclear Sciences, University of Belgrade, P.O. Box 522, 11001 Belgrade (Serbia); Borka, D. [Atomic Physics Laboratory (040), Vinča Institute of Nuclear Sciences, University of Belgrade, P.O. Box 522, 11001 Belgrade (Serbia); Galijaš, S.M.D. [Faculty of Physics, University of Belgrade, P.O. Box 368, 11001 Belgrade (Serbia)

    2017-05-18

    Highlights: • For the first time we presented theoretically obtained distributions of channeled protons with radially deformed SWNT. • Our findings indicate that influence of the radial deformation is very strong and it should not be omitted in simulations. • We show that the spatial and angular distributions depend strongly of level of radial deformation of nanotube. • Our obtained results can be compared with measured distributions to reveal the presence of various types of defects in SWNT. - Abstract: In this paper we have presented a theoretical investigation of the channeling of 1 GeV protons with the radial deformed (10, 0) single-wall carbon nanotubes (SWNTs). We have calculated channeling potential within the deformed nanotubes. For the first time we presented theoretically obtained spatial and angular distributions of channeled protons with radially deformed SWNT. We used a Monte Carlo (MC) simulation technique. We show that the spatial and angular distributions depend strongly of level of radial deformation of nanotube. These results may be useful for nanotube characterization and production and guiding of nanosized ion beams.

  7. Aneurisma idiopático de artéria radial: relato de caso Idiopathic radial artery aneurysm: case report

    Directory of Open Access Journals (Sweden)

    Luiz Ernani Meira Jr.

    2011-12-01

    Full Text Available Os aneurismas da artéria radial são extremamente raros. Em sua maioria, consistem de pseudoaneurismas pós-traumáticos. Os aneurismas da artéria radial verdadeiros podem ser idiopáticos, congênitos, pós-estenóticos ou associados a patologias, tais como vasculites e doenças do tecido conjuntivo. Foi relatado um caso de aneurisma idiopático de artéria radial em uma criança de três anos, que, após completa investigação diagnóstica complementar, foi submetida à ressecção cirúrgica.Radial artery aneurysms are extremely rare. Post-traumatic pseudoaneurysms are the vast majority. True radial artery aneurysms can be idiopathic, congenital, poststenotic, or associated with some pathologies, such as vasculitis and conjunctive tissue diseases. We report a case of an idiopathic aneurysm of the radial artery in a three-year-old child who was submitted to surgical resection after a complete diagnostic approach.

  8. Irreducible volar subluxation of the proximal interphalangeal joint due to radial collateral ligament interposition: case report and review of literature.

    Science.gov (United States)

    Cheung, Jason Pui Yin; Tse, Wing Lim; Ho, Pak Cheong

    2015-01-01

    Irreducible volar subluxation should be considered when assessing a patient with flexion deformity of the proximal interphalangeal finger joint (PIPJ). Primary assessment requires careful examination of the collateral ligaments and extensor tendon. Preoperative imaging such as ultrasound and MRI can help identify the interposed structures and plan the subsequent operation. Although rare, irreducible volar subluxation due to radial collateral ligament interposition is an important entity to be aware of. Prompt and appropriate management can prevent joint stiffness and loss of function.

  9. O-space with high resolution readouts outperforms radial imaging.

    Science.gov (United States)

    Wang, Haifeng; Tam, Leo; Kopanoglu, Emre; Peters, Dana C; Constable, R Todd; Galiana, Gigi

    2017-04-01

    While O-Space imaging is well known to accelerate image acquisition beyond traditional Cartesian sampling, its advantages compared to undersampled radial imaging, the linear trajectory most akin to O-Space imaging, have not been detailed. In addition, previous studies have focused on ultrafast imaging with very high acceleration factors and relatively low resolution. The purpose of this work is to directly compare O-Space and radial imaging in their potential to deliver highly undersampled images of high resolution and minimal artifacts, as needed for diagnostic applications. We report that the greatest advantages to O-Space imaging are observed with extended data acquisition readouts. A sampling strategy that uses high resolution readouts is presented and applied to compare the potential of radial and O-Space sequences to generate high resolution images at high undersampling factors. Simulations and phantom studies were performed to investigate whether use of extended readout windows in O-Space imaging would increase k-space sampling and improve image quality, compared to radial imaging. Experimental O-Space images acquired with high resolution readouts show fewer artifacts and greater sharpness than radial imaging with equivalent scan parameters. Radial images taken with longer readouts show stronger undersampling artifacts, which can cause small or subtle image features to disappear. These features are preserved in a comparable O-Space image. High resolution O-Space imaging yields highly undersampled images of high resolution and minimal artifacts. The additional nonlinear gradient field improves image quality beyond conventional radial imaging. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Comminuted fractures of the radial head: resection or prosthesis?

    Science.gov (United States)

    Lópiz, Yaiza; González, Ana; García-Fernández, Carlos; García-Coiradas, Javier; Marco, Fernando

    2016-09-01

    At present, surgical treatment of comminuted radial head fractures without associated instability continues to be controversial. When anatomical reconstruction is not possible, radial head excision is performed. However, the appearance of long-term complications with this technique, along with the development of new radial head implants situates arthroplasty as a promising surgical alternative. The purpose of the present study was to compare the mid-term functional outcomes of both techniques. A retrospective study was performed between 2002 and 2011 on 25 Mason type-III fractures, 11 patients treated with primary radial head resection and 14 who received treatment of the fracture with metal prosthesis. At the end of follow-up, patients were contacted and outcomes evaluated according to: Mayo Elbow Performance Score (MEPS), the Disabilities of the Arm, Shoulder and Hand score (DASH) and strength measurement. Radiographic assessment (proximal migration of the radius, osteoarthritic changes, and signs of prosthesis loosening) was also performed. The average age of the sample was 53.7 years in the resection group, and 54.4 years in the replacement group, with a mean follow-up of 60.3 and 42 months respectively. According to the MEPS scale, there were 6 excellent cases, 3 good and 2 acceptable in the resection group, and 6 excellent cases, 3 good, 3 acceptable, and 2 poor in the prosthesis group. The mean DASH score were 13.5, and 24.8 for the resection and the replacement group respectively. We found one postoperative complication in the resection group (stiffness and valgus instability) and 6 in the replacement group: 3 of joint stiffness, 1 case of prosthesis breakage, and 2 neurological injuries. Although this is a retrospective study, the high complication rate occurring after radial head replacement in comparison with radial head resection, as well as good functional results obtained with this last technique, leads us to recommend it for comminuted radial head

  11. Espasmo hemifacial: resultados do tratamento cirúrgico em 14 casos Hemifacial spasm: report of 14 cases submitted to surgical treatment

    Directory of Open Access Journals (Sweden)

    Rui R. D. Carvalho

    1973-06-01

    Full Text Available Quatorze pacientes com espasmo hemifacial foram submetidos a exploração cirúrgica e neurolise do 7º par craniano no ângulo ponto-cerebelar. Em 7 pacientes havia indubitável compressão do nervo facial por alça anômala da artéria cerebelar anterior e inferior sendo que um paciente também apresentava malformação de Arnold-Chiari. Em um paciente havia aracnoidite envolvendo o nervo. Em 6 outros, o nervo achava-se aparentemente livre. Houve alívio imediato e duradouro do espasmo em 10 pacientes, 2 permaneceram inalterados e um apresentou recidiva após 10 meses. Houve um óbito no pós-operatório imediato, conseqüente a traumatismo craniano por queda do paciente.Fourteen patients with hemifacial spasm were submitted to surgical exposure and neurolysis of the seventh nerve at the cerebellopontine angle. Seven patients had an anomalous anterior inferior cerebellar artery pulsating on the nerve, being present in one patient also Arnold-Chiari malformation. Another patient had chronic arachnoiditis involving the facial nerve. In 6 patients no abnormalities were found in the posterior fossa. There was complete relief of the spasm in 10 patients, 2 were unchanged, 1 had recurrence 10 months later and 1 died in the post-operative period due to head injury sustained during a fall.

  12. Presentation of 60 Cases of Infantile Spasms Based on Etiology, Clinical Manifestation EEG and Brain CT Scan in Mofid Children Hospital

    Directory of Open Access Journals (Sweden)

    Mohammad Mehdi Taghdiri

    2002-06-01

    Full Text Available Objective: Among different epileptic syndrome infantile spasm is one of the most malignant forms which cause irrepairable brain damage in the child. Consequently the longer this type of epilepsy lasts the more harmful results will follow. The majority of children with infantile spasm are younger than one year age and only 5 percent of affected children are in the age group above one year. Materials & Methods: This descriptive study was done on 60 (36 male and 24 female infants 2-24 months age with clinical examination, observation, interview and questionnaire  in pediatric neurology department of Mofid children hospital during two years. Results: From 60 patients (36 male and 24 female, 48 case (80% symptomatic and 12 case (20% cryptogenic and idiopathic. Based on clinical manifestation 35 case (58% were flexor type. 6 case (10% extensor and 19 cases (32% mixed. In EEG hypsarrhythmia in all patients was seen. Brain CT scan in 11 cases showed brain atrophy and in remainder was normal. Conclusion: In our study etiologically symptomatic and clinically flexor type was more common. Hysparrhythmia in all patients was seen and brain CT scan in 80% of patients was normal.

  13. Total corpus callosotomy for epileptic spasms after acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) in a case with tuberous sclerosis complex.

    Science.gov (United States)

    Okanishi, Tohru; Fujimoto, Ayataka; Motoi, Hirotaka; Kanai, Sotaro; Nishimura, Mitsuyo; Yamazoe, Tomohiro; Takagi, Atsushi; Yamamoto, Takamichi; Enoki, Hideo

    2017-05-01

    Corpus callosotomy is a palliative therapy for refractory epilepsy, including West syndrome, without a resectable epileptic focus. The surgical outcome of corpus callosotomy is relatively favorable in cryptogenic (non-lesional) West syndrome. Tuberous sclerosis complex (TSC) is a disorder that frequently leads to the development of refractory seizures by multiple cortical tubers. The multiple cortical tubers cause multiple or wide epileptic networks in these cases. Most of West syndrome cases in TSC with multiple tubers need additional resective surgery after corpus callosotomy. We describe a case of TSC in a boy aged 4years and 8months. He had multiple cortical tubers on his brain and developed epileptic spasms. The seizures were controlled with valproate. At the age of 1year and 4months, he presented with acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), and had relapsed epileptic spasms one month after the onset of the encephalopathy. The seizures were refractory to multiple antiepileptic drugs. A total corpus callosotomy was performed at the age of 3years and 8months. The patient did not show any seizures after the surgery. During 12months of the follow-up, the patient was free from any seizures. Even in cases of symptomatic WS with multiple lesions, total corpus callosotomy may be a good strategy if the patients have secondary diffuse brain insults. Copyright © 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  14. Radial voidage variation in fixed beds of fuel wood pellets

    International Nuclear Information System (INIS)

    Hamel, Stefan; Krumm, Wolfgang

    2012-01-01

    Fixed beds of fuel wood are commonly found in numerous processes: storage and transportation, drying and thermal conversion such as combustion or gasification. Pellets in particular are mostly used as fuel for domestic heating boilers. The characterization of spatial voidage distribution is of great importance for flow and reactor modelling. The present study focuses on the radial porosity variations of cylindrical beds of commercially available wood pellets. The experimental procedure is based on the classical technique of consolidating packed beds with a resin. The radial voidage distribution of three different cylindrical beds is determined by image analysis of sections of the solidified packing. The results are discussed and summarized in a mathematical expression correlating the radial voidage distribution depending on packing core porosity and dimensionless distance from the tube wall. -- Highlights: ► Packing characteristics for commercially available wood pellets were investigated. ► Radial porosity variations of cylindrical pellets beds were investigated. ► Epoxy resin consolidated packings were investigated by image analysis. ► Mathematical term for radial voidage distribution of pellet packing was derived.

  15. A user's evaluation of radial flow HEPA filters

    International Nuclear Information System (INIS)

    Purcell, J.A.

    1992-07-01

    High efficiency particulate air (HEPA) filters of rectangular cross section have been used to remove particulates and the associated radioactivity from air ventilation streams since the advent of nuclear materials processing. Use of round axial flow HEPA filters is also longstanding. The advantages of radial flow filters in a circular configuration have been well demonstrated in UKAEA during the last 5--7 years. An evaluation of radial flow filters for fissile process gloveboxes reveals several substantial benefits in addition to the advantages claimed in UKAEA Facilities. The radial flow filter may be provided in a favorable geometry resulting in improved criticality safety. The filter configuration lends to in-place testing at the glovebox to exhaust duct interface. This will achieve compliance with DOE Order 6430.1A, Section 99.0.2. Preliminary testing at SRS for radial flow filters manufactured by Flanders Filters, Inc. revealed compliance in all the usual specifications for filtration efficiency, pressure differential and materials of construction. An evaluation, further detailed in this report, indicates that the radial flow HEPA filter should be considered for inclusion in new ventilation system designs

  16. Early evolution of radial glial cells in Bilateria.

    Science.gov (United States)

    Helm, Conrad; Karl, Anett; Beckers, Patrick; Kaul-Strehlow, Sabrina; Ulbricht, Elke; Kourtesis, Ioannis; Kuhrt, Heidrun; Hausen, Harald; Bartolomaeus, Thomas; Reichenbach, Andreas; Bleidorn, Christoph

    2017-07-26

    Bilaterians usually possess a central nervous system, composed of neurons and supportive cells called glial cells. Whereas neuronal cells are highly comparable in all these animals, glial cells apparently differ, and in deuterostomes, radial glial cells are found. These particular secretory glial cells may represent the archetype of all (macro) glial cells and have not been reported from protostomes so far. This has caused controversial discussions of whether glial cells represent a homologous bilaterian characteristic or whether they (and thus, centralized nervous systems) evolved convergently in the two main clades of bilaterians. By using histology, transmission electron microscopy, immunolabelling and whole-mount in situ hybridization, we show here that protostomes also possess radial glia-like cells, which are very likely to be homologous to those of deuterostomes. Moreover, our antibody staining indicates that the secretory character of radial glial cells is maintained throughout their various evolutionary adaptations. This implies an early evolution of radial glial cells in the last common ancestor of Protostomia and Deuterostomia. Furthermore, it suggests that an intraepidermal nervous system-composed of sensory cells, neurons and radial glial cells-was probably the plesiomorphic condition in the bilaterian ancestor. © 2017 The Authors.

  17. Radial mode evolution in longitudinal bunched beam instability

    International Nuclear Information System (INIS)

    Zhang, S.Y.; Weng, W.T.

    1995-01-01

    An indispensable aspect of the bunched beam instability mechanism is the variation of the particle distribution with respect to the beam intensity. This density variation can be shown as the evolution of radial modes. The radial modes, which are determined by the stationary particle distribution and the impedance, represent the coherence of the particle density variation governed by the Vlasov equation. Using this coherence in the beam instability analysis gives rise to not only the computational efficiency but also the physical insight into the instability mechanism. The evolution of the radial modes displays several interesting properties for the cases without and with synchrotron frequency spread. If the azimuthal mode coupling cannot be neglected, then corresponding to each coherent frequency shift there exists an extended radial mode which includes the interactions from other azimuthal modes. In this article, the radial mode evolution and the related physical implications will be discussed, which are useful for the understanding of the beam instabilities, and also useful for the beam diagnostics

  18. Radial Structure Scaffolds Convolution Patterns of Developing Cerebral Cortex

    Directory of Open Access Journals (Sweden)

    Mir Jalil Razavi

    2017-08-01

    Full Text Available Commonly-preserved radial convolution is a prominent characteristic of the mammalian cerebral cortex. Endeavors from multiple disciplines have been devoted for decades to explore the causes for this enigmatic structure. However, the underlying mechanisms that lead to consistent cortical convolution patterns still remain poorly understood. In this work, inspired by prior studies, we propose and evaluate a plausible theory that radial convolution during the early development of the brain is sculptured by radial structures consisting of radial glial cells (RGCs and maturing axons. Specifically, the regionally heterogeneous development and distribution of RGCs controlled by Trnp1 regulate the convex and concave convolution patterns (gyri and sulci in the radial direction, while the interplay of RGCs' effects on convolution and axons regulates the convex (gyral convolution patterns. This theory is assessed by observations and measurements in literature from multiple disciplines such as neurobiology, genetics, biomechanics, etc., at multiple scales to date. Particularly, this theory is further validated by multimodal imaging data analysis and computational simulations in this study. We offer a versatile and descriptive study model that can provide reasonable explanations of observations, experiments, and simulations of the characteristic mammalian cortical folding.

  19. Effect of the radial electric field on turbulence

    International Nuclear Information System (INIS)

    Carreras, B.A.; Lynch, V.E.

    1990-01-01

    For many years, the neoclassical transport theory for three- dimensional magnetic configurations, such as magnetic mirrors, ELMO Bumpy Tori (EBTs), and stellarators, has recognized the critical role of the radial electric field in the confinement. It was in these confinement devices that the first experimental measurements of the radial electric field were made and correlated with confinement losses. In tokamaks, the axisymmetry implies that the neoclassical fluxes are ambipolar and, as a consequence, independent of the radial electric field. However, axisymmetry is not strict in a tokamak with turbulent fluctuations, and near the limiter ambipolarity clearly breaks down. Therefore, the question of the effect of the radial electric field on tokamak confinement has been raised in recent years. In particular, the radial electric field has been proposed to explain the transition from L-mode to H-mode confinement. There is some initial experimental evidence supporting this type of explanation, although there is not yet a self-consistent theory explaining the generation of the electric field and its effect on the transport. Here, a brief review of recent results is presented. 27 refs., 4 figs

  20. A survey of radial methods for information visualization.

    Science.gov (United States)

    Draper, Geoffrey M; Livnat, Yarden; Riesenfeld, Richard F

    2009-01-01

    Radial visualization, or the practice of displaying data in a circular or elliptical pattern, is an increasingly common technique in information visualization research. In spite of its prevalence, little work has been done to study this visualization paradigm as a methodology in its own right. We provide a historical review of radial visualization, tracing it to its roots in centuries-old statistical graphics. We then identify the types of problem domains to which modern radial visualization techniques have been applied. A taxonomy for radial visualization is proposed in the form of seven design patterns encompassing nearly all recent works in this area. From an analysis of these patterns, we distill a series of design considerations that system builders can use to create new visualizations that address aspects of the design space that have not yet been explored. It is hoped that our taxonomy will provide a framework for facilitating discourse among researchers and stimulate the development of additional theories and systems involving radial visualization as a distinct design metaphor.

  1. Comparison of Deterministic and Probabilistic Radial Distribution Systems Load Flow

    Science.gov (United States)

    Gupta, Atma Ram; Kumar, Ashwani

    2017-12-01

    Distribution system network today is facing the challenge of meeting increased load demands from the industrial, commercial and residential sectors. The pattern of load is highly dependent on consumer behavior and temporal factors such as season of the year, day of the week or time of the day. For deterministic radial distribution load flow studies load is taken as constant. But, load varies continually with a high degree of uncertainty. So, there is a need to model probable realistic load. Monte-Carlo Simulation is used to model the probable realistic load by generating random values of active and reactive power load from the mean and standard deviation of the load and for solving a Deterministic Radial Load Flow with these values. The probabilistic solution is reconstructed from deterministic data obtained for each simulation. The main contribution of the work is: Finding impact of probable realistic ZIP load modeling on balanced radial distribution load flow. Finding impact of probable realistic ZIP load modeling on unbalanced radial distribution load flow. Compare the voltage profile and losses with probable realistic ZIP load modeling for balanced and unbalanced radial distribution load flow.

  2. Influence of Radial Stress Gradient on Strainbursts: An Experimental Study

    Science.gov (United States)

    Su, Guoshao; Zhai, Shaobin; Jiang, Jianqing; Zhang, Gangliang; Yan, Liubin

    2017-10-01

    Strainbursts, which are violent disasters that are accompanied by the ejection failure of rocks, usually occur in hard brittle rocks around highly stressed underground openings. The release of the radial stress at excavation boundaries is one of the major inducing factors for strainbursts in tunnels. After excavation, the radial stress usually exhibits different but apparent gradient variations along the radial direction near the boundary within a certain depth under different in situ stress conditions. In this study, the influence of the radial stress gradient on strainbursts of granite was investigated using an improved true-triaxial rockburst testing system, which was equipped with an acoustic emission monitoring system. The stress state and boundary conditions (i.e., one face free, other faces loaded and increasing tangential stress) of the representative rock element in the vicinity of the excavation boundary were simulated. High-speed cameras were used to capture the ejection failure processes during strainbursts, and the kinetic energy of ejected fragments was quantitatively estimated by analyzing the recorded videos. The experimental results indicate that with an increasing radial stress gradient, the strength increases, the apparent yield platform prior to the peak stress on the stress-strain curves decreases, the failure mode changes from strainburst characterized by tensile splitting to strainburst characterized by shear rupture, and the kinetic energy of ejected fragments during strainbursts significantly increases.

  3. Punciones repetidas de la arteria radial para cateterismo cardíaco Repeated radial artery puncture for cardiac catheterization

    Directory of Open Access Journals (Sweden)

    Eduardo Magariños

    2007-06-01

    Full Text Available La punción percutánea de la arteria radial para la realización de procedimientos por cateterismo ha ganado aceptación debido a una eficacia similar a la obtenida con el acceso femoral. En el presente trabajo evaluamos los resultados obtenidos con la punción repetida de esta arteria. En un total de 182 accesos radiales se realizaron 17 punciones repetidas, y mediante ellas, 20 procedimientos (9 coronariografías y 11 angioplastias. Se obtuvo éxito del acceso en 15 punciones repetidas (88.2% y éxito del procedimiento en todos los casos cuando logramos éxito del acceso. Si bien el grupo es pequeño es suficiente para mostrar que la punción repetida de la arteria radial es factible y permite una alta tasa de éxito de los procedimientos con una disminución ostensible de las complicaciones locales.The radial artery approach for percutaneous cardiac interventions has gained worldwide acceptance due to the similar results obtained by the femoral artery access. In this paper, we report our experience with repeated puncture of the radial artery. One hundred and eighty two radial artery access procedures were performed, in 17 interventions the puncture was repeated once or twice, with a total of 20 therapeutic catheterizations (9 coronary angiographies, 11 angioplasties. There was no therapeutic failure through the radial approach but, we successfully gained access in 88.2% (15/17 of the re-interventions cases. Although an experience with a low number of cases, we had a very high successful therapeutic rate, and also a remarkable lowering of local complications, this shows the feasibility and potential of this technique.

  4. Radial Fingering in a Porous Medium Digitation radiale dans un milieu poreux

    Directory of Open Access Journals (Sweden)

    Ni W.

    2006-11-01

    Full Text Available The theory of immiscible radial displacement in a Hele-Shaw cell is extended to the case of a porous medium contained between two closely-spaced parallel plates, and experiments are described for the displacement of glycerine by paraffin oil in such a system. Data are presented for the number of fingers, the breakthrough time, and the glycerine recovery, for a range of flowrates varying through three orders of magnitude. Good agreement between theory and experiment is observed. La théorie s'appliquant aux déplacements radiaux dans les cellules Hele-Shaw a été étendue à un système qui consiste en une couche mince de milieux poreux encapsulée entre deux plaques en verre. Dans cet article, on examine les déplacements de la glycérine par de l'huile de paraffine. En faisant varier le débit de l'huile de paraffine dans un intervalle de trois ordres de grandeur, on a étudié les variables telles que le nombre de digitations, le temps de percée et le taux de récupération de la glycérine. On a observé un bon accord entre la théorie et les résultats expérimentaux.

  5. Magnetic resonance imaging of radial sclerosing lesions (radial scars) of the breast

    Energy Technology Data Exchange (ETDEWEB)

    Linda, Anna, E-mail: annalinda33@gmail.com [Institute of Diagnostic Radiology, Azienda Ospedaliero-Universitaria Santa Maria della Misericordia, Udine (Italy); Zuiani, Chiara; Londero, Viviana [Institute of Diagnostic Radiology, Azienda Ospedaliero-Universitaria Santa Maria della Misericordia, Udine (Italy); Cedolini, Carla [Department of Surgery, Azienda Ospedaliero-Universitaria Santa Maria della Misericordia, Udine (Italy); Girometti, Rossano; Bazzocchi, Massimo [Institute of Diagnostic Radiology, Azienda Ospedaliero-Universitaria Santa Maria della Misericordia, Udine (Italy)

    2012-11-15

    Purpose: To identify magnetic resonance (MR) imaging (MRI) features of radial sclerosing lesions (RSLs) of the breast. Methods and materials: The radiologic and pathologic records for 4629 consecutive patients undergoing MR examinations of the breast were retrospectively reviewed. Patients who received a pathologic diagnosis of RSL without atypia or carcinoma at surgical excision were identified. The MR images were evaluated according to the BI-RADS-MRI lexicon by two experienced breast radiologists. The frequency of morphologic and kinetic patterns and of BI-RADS-MRI assessment categories was calculated. Results: Twenty-nine patients with 29 surgically excised RSL were identified. Nine (31%) RSL were MR-occult; the remaining 20 (69%) RSL presented as masses (10/20, 50%), architectural distortions (5/20, 25%), non-mass lesions (4/20, 20%), and focus (1/20, 5%). Kinetic analysis was performed in 18 RSL: enhancement features were benign in 9 (50%) cases, suspicious in 7 (39%) cases and indeterminate in 2 (11%) cases. Twelve (41%) MR examinations were assessed as suspicious (BI-RADS-MRI 4 and 5), and 17 (59%) as negative (BI-RADS-MRI 1) or benign (BI-RADS-MRI 2 and 3). Conclusion: RSLs are often visualized on MR imaging. Just as in mammography and sonography, RSL can have variable morphologic and kinetic features, and not infrequently they can mimic invasive carcinoma of the breast.

  6. Choking Prevention

    Science.gov (United States)

    ... Healthy Living Healthy Living Healthy Living Nutrition Fitness Sports Oral Health Emotional Wellness Growing Healthy Sleep Safety & Prevention Safety & Prevention Safety and Prevention Immunizations At Home ...

  7. Rayleigh-Taylor instability of cylindrical jets with radial motion

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Xiang M. [GE Nuclear, Wilmington, NC (United States); Schrock, V.E.; Peterson, P.F. [Univ. of California, Berkeley, CA (United States)

    1995-09-01

    Rayleigh-Taylor instability of an interface between fluids with different densities subjected to accelleration normal to itself has interested researchers for almost a century. The classic analyses of a flat interface by Rayleigh and Taylor have shown that this type of instability depends on the direction of acceleration and the density differences of the two fluids. Plesset later analyzed the stability of a spherically symmetric flows (and a spherical interface) and concluded that the instability also depends on the velocity of the interface as well as the direction and magnitude of radial acceleration. The instability induced by radial motion in cylindrical systems seems to have been neglected by previous researchers. This paper analyzes the Rayleigh-Taylor type of the spherical case, the radial velocity also plays an important role. As an application, the example of a liquid jet surface in an Inertial Confinement Fusion (ICF) reactor design is analyzed.

  8. Point Set Denoising Using Bootstrap-Based Radial Basis Function.

    Science.gov (United States)

    Liew, Khang Jie; Ramli, Ahmad; Abd Majid, Ahmad

    2016-01-01

    This paper examines the application of a bootstrap test error estimation of radial basis functions, specifically thin-plate spline fitting, in surface smoothing. The presence of noisy data is a common issue of the point set model that is generated from 3D scanning devices, and hence, point set denoising is one of the main concerns in point set modelling. Bootstrap test error estimation, which is applied when searching for the smoothing parameters of radial basis functions, is revisited. The main contribution of this paper is a smoothing algorithm that relies on a bootstrap-based radial basis function. The proposed method incorporates a k-nearest neighbour search and then projects the point set to the approximated thin-plate spline surface. Therefore, the denoising process is achieved, and the features are well preserved. A comparison of the proposed method with other smoothing methods is also carried out in this study.

  9. Optimal design of radial Bragg cavities and lasers.

    Science.gov (United States)

    Ben-Bassat, Eyal; Scheuer, Jacob

    2015-07-01

    We present a new and optimal design approach for obtaining maximal confinement of the field in radial Bragg cavities and lasers for TM polarization. The presented approach outperforms substantially the previously employed periodic and semi-periodic design schemes of such lasers. We show that in order to obtain maximal confinement, it is essential to consider the complete reflection properties (amplitude and phase) of the propagating radial waves at the interfaces between Bragg layers. When these properties are taken into account, we find that it is necessary to introduce a wider ("half-wavelength") layer at a specific radius in the "quarter-wavelength" radial Bragg stack. It is shown that this radius corresponds to the cylindrical equivalent of Brewster's angle. The confinement and field profile are calculated numerically by means of transfer matrix method.

  10. Interactions between Radial Electric Field, Transport and Structure in Helical Plasmas

    International Nuclear Information System (INIS)

    Ida, Katsumi and others

    2006-01-01

    Control of the radial electric field is considered to be important in helical plasmas, because the radial electric field and its shear are expected to reduce neoclassical and anomalous transport, respectively. Particle and heat transport, that determines the radial structure of density and electron profiles, sensitive to the structure of radial electric field. On the other hand, the radial electric field itself is determined by the plasma parameters. In general, the sign of the radial electric field is determined by the plasma collisionality, while the magnitude of the radial electric field is determined by the temperature and/or density gradients. Therefore the structure of radial electric field and temperature and density are strongly coupled through the particle and heat transport and formation mechanism of radial electric field. Interactions between radial electric field, transport and structure in helical plasmas is discussed based on the experiments on Large Helical Device

  11. Event-Specific Quantification of Radiation Belt Radial Diffusion

    Science.gov (United States)

    Tu, W.; Sarris, T. E.; Ozeke, L.

    2016-12-01

    Recently, there has been a great emphasis on developing event-specific inputs for radiation belt models, since they are proven critical for reproducing the observed radiation belt dynamics during strong events. For example, our DREAM3D simulation of the 8-9 October 2012 storm demonstrates that event-specific chorus wave model and seed population are critical to reproduce the strong enhancement of MeV electrons in this event. However, the observed fast electron dropout preceding the enhancement was not captured by the simulation, which could be due to the combined effects of fast outward radial diffusion of radiation belt electrons with magnetopause shadowing and enhanced electron precipitation. Without an event-specific quantification of radial diffusion, we cannot resolve the relative contribution of outward radial diffusion and precipitation to the observed electron dropout or realistically reproduce the dynamics during the event. In this work, we provide physical quantification of radial diffusion specific to the October 2012 event by including both real-time and global distributions of ULF waves from a constellation of wave measurements and event-specific estimation of ULF wave mode structure. The global maps of ULF waves during the event are constructed by combining the real-time measurements from the Van Allen Probes, THEMIS, and GOES satellites in space and a large array of ground magnetometers. The real-time ULF wave mode structure is then estimated using the new Cross-Wavelet Transform technique, applied to various azimuthally aligned pairs of ULF wave measurements that are located at the same L shells. The cross power and phase differences between the time series are calculated using the technique, based on which the wave power per mode number is estimated. Finally, the physically estimated radial diffusion coefficients specific to the event are applied to the DREAM3D model to quantify the relative contribution of radial diffusion to the electron dynamics

  12. Radial shortening following a fracture of the proximal radius.

    Science.gov (United States)

    Duckworth, Andrew D; Watson, Bruce S; Will, Elizabeth M; Petrisor, Brad A; Walmsley, Phillip J; Court-Brown, Charles M; McQueen, Margaret M

    2011-06-01

    The Essex-Lopresti lesion is thought to be rare, with a varying degree of disruption to forearm stability probable. We describe the range of radial shortening that occurs following a fracture of the proximal radius, as well as the short-term outcome in these patients. Over an 18-month period, we prospectively assessed all patients with a radiographically confirmed proximal radial fracture. Patients noted to have ipsilateral wrist pain at initial presentation underwent bilateral radiography to determine whether there was disruption of the distal radio-ulnar joint suggestive of an Essex-Lopresti lesion. Outcome was assessed after a mean of 6 (1.5-12) months using clinical and radiographic results, including the Mayo elbow score (MES) and the short musculoskeletal function assessment (SMFA) questionnaire. One patient with a Mason type-I fracture was lost to follow-up after initial presentation. 60 patients had ipsilateral wrist pain at the initial assessment of 237 proximal radial fractures. Radial shortening of ≥ 2mm (range: 2-4mm) was seen in 22 patients (mean age 48 (19-79) years, 16 females). The most frequent mechanism of injury was a fall from standing height (10/22). 21 fractures were classified as being Mason type-I or type-II, all of which were managed nonoperatively. One Mason type-III fracture underwent acute radial head replacement. Functional outcome was assessed in 21 patients. We found an excellent or good MES in 18 of the 20 patients with a Mason type-I or type-II injury. The incidence of the Essex-Lopresti lesion type is possibly under-reported as there is a spectrum of injuries, and subtle disruptions often go unidentified. A full assessment of all patients with a proximal radial fracture is required in order to identify these injuries, and the index of suspicion is raised as the complexity of the fracture increases.

  13. Radial multipliers on amalgamated free products of II-factors

    DEFF Research Database (Denmark)

    Möller, Sören

    2014-01-01

    Let ℳi be a family of II1-factors, containing a common II1-subfactor 풩, such that [ℳi : 풩] ∈ ℕ0 for all i. Furthermore, let ϕ: ℕ0 → ℂ. We show that if a Hankel matrix related to ϕ is trace-class, then there exists a unique completely bounded map Mϕ on the amalgamated free product of the ℳi with a...... with amalgamation over 풩, which acts as a radial multiplier. Hereby, we extend a result of Haagerup and the author for radial multipliers on reduced free products of unital C*- and von Neumann algebras....

  14. Studies of radial distortions of the ATLAS Inner Detector

    CERN Document Server

    The ATLAS collaboration

    2018-01-01

    The measurement of the absolute momentum scale of charged particles provided by the ATLAS inner detector is affected by biases related to geometrical deformations which are not well constrained by the track-based alignment procedure. The focus of this note is on momentum biases related to radial distortions of the inner detector. The $J/\\psi$, $\\Upsilon$, and $Z$-boson resonances decaying into pairs of muons are used to study and quantify such radial distortions in the barrel region of the inner detector. The analysis is performed on data collected in 2016 during Run 2 of the Large Hadron Collider, and corresponding to 33 fb$^{-1}$ of integrated luminosity.

  15. THE RADIAL VELOCITY EXPERIMENT (RAVE): THIRD DATA RELEASE

    International Nuclear Information System (INIS)

    Siebert, A.; Williams, M. E. K.; Siviero, A.; Boeche, C.; Steinmetz, M.; De Jong, R. S.; Enke, H.; Anguiano, B.; Reid, W.; Ritter, A.; Fulbright, J.; Wyse, R. F. G.; Munari, U.; Zwitter, T.; Watson, F. G.; Burton, D.; Cass, C. J. P.; Fiegert, K.; Hartley, M.; Russel, K. S.

    2011-01-01

    We present the third data release of the RAdial Velocity Experiment (RAVE) which is the first milestone of the RAVE project, releasing the full pilot survey. The catalog contains 83,072 radial velocity measurements for 77,461 stars in the southern celestial hemisphere, as well as stellar parameters for 39,833 stars. This paper describes the content of the new release, the new processing pipeline, as well as an updated calibration for the metallicity based upon the observation of additional standard stars. Spectra will be made available in a future release. The data release can be accessed via the RAVE Web site.

  16. Modeling the Radial Color Profile of M31

    Directory of Open Access Journals (Sweden)

    Semionov D.

    2003-12-01

    Full Text Available We present a preliminary study of a fragment of the radial color profile of the spiral galaxy M 31 in terms of 2-D model accounting for internal extinction in the disk. The two stellar population disk model was assumed. The old dust-free disk population is represented by the double exponential law, and the young disk population, well mixed with the dust, resides in spiral arms of various scale-heights. We find a good agreement among the radial color B-R profiles produced by this simple model and the profile measured around the spiral arm S4 of M 31.

  17. Three Cases of Radial Nerve Palsy with Bee Venom Therapy

    Directory of Open Access Journals (Sweden)

    Kim Hyo-Soo

    2004-06-01

    Full Text Available Objectives : The purpose of this study is to report the efficiency of Bee Venom Therapy by managering of radial nerve palsy patients. Methods : Three patients were treatmented by Bee Venom therapy and acupucture therapy. And We took pictures of someone's wrist and checked the power of muscles by the improving phase. Results : Almost cases shows the improvement in the movement of wrist and the numbness of hand. By using acuputure and Bee Venom therapy, the symptoms of radial nerv palsy was more fastly recovered.

  18. Matching problems in pulse power radial transmission lines

    International Nuclear Information System (INIS)

    Mittag, K.; Brandelik, A.

    1984-12-01

    In this report we study the power transfer from a generator along a coaxial transmission line followed by a radial transmission line into a load, which in our application is a pseudo-spark plasma of about one millimeter diameter and about 15 cm in length. First the theoretical background based on transmission line theory is described. Then numerical results are presented. The main conclusion is that when matching the pulse power generator to the pseudo-spark plasma, the effect of the impedance transformation caused by the radial transmission line has to be taken into account. The conditions to obtain an optimal match are described. (orig.) [de

  19. Bilateral radial neck fractures – A Case Report

    Directory of Open Access Journals (Sweden)

    ABY Ng

    2007-11-01

    Full Text Available Radial head and neck fractures are the most frequently seen elbow fractures. The usual cause of this injury is a fall onto an outstretched hand with a partly flexed elbow. We report here an unusual case of bilateral non-displaced radial neck fractures in a patient who presented with complaints of pain in both elbows following a simple fall. This case highlights the need for a high index of suspicion in the diagnosis of multiple injuries, no matter how `trivial` the mechanism of injury.

  20. Imaging of radial wrist pain. I. Imaging modalities and anatomy

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ryan Ka Lok; Griffith, James F.; Ng, Alex Wing Hung [The Chinese University of Hong Kong, Department of Imaging and Interventional Radiology, Prince of Wales Hospital, Hong Kong, Shatin (China); Wong, Clara Wing Yee [The Chinese University of Hong Kong, Department of Orthopedics and Traumatology, Shatin (China)

    2014-06-15

    Radial wrist pain is a common clinical complaint. The relatively complex anatomy in this region, combined with the small size of the anatomical structures and occasionally subtle imaging findings, can pose problems when trying to localize the exact cause of pain. To fully comprehend the underlying pathology, one needs a good understanding of both radial-sided wrist anatomy and the relative merits of the different imaging techniques used to assess these structures. In part I of this review, these aspects will be discussed. (orig.)