Persistent hemifacial spasm after microvascular decompression: a risk assessment model.

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Shah, Aalap; Horowitz, Michael

2017-06-01

Microvascular decompression (MVD) for hemifacial spasm (HFS) provides resolution of disabling symptoms such as eyelid twitching and muscle contractions of the entire hemiface. The primary aim of this study was to evaluate the predictive value of patient demographics and spasm characteristics on long-term outcomes, with or without intraoperative lateral spread response (LSR) as an additional variable in a risk assessment model. A retrospective study was undertaken to evaluate the associations of pre-operative patient characteristics, as well as intraoperative LSR and need for a staged procedure on the presence of persistent or recurrent HFS at the time of hospital discharge and at follow-up. A risk assessment model was constructed with the inclusion of six clinically or statistically significant variables from the univariate analyses. A receiving operator characteristic curve was generated, and area under the curve was calculated to determine the strength of the predictive model. A risk assessment model was first created consisting of significant pre-operative variables (Model 1) (age >50, female gender, history of botulinum toxin use, platysma muscle involvement). This model demonstrated borderline predictive value for persistent spasm at discharge (AUC .60; p=.045) and fair predictive value at follow-up (AUC .75; p=.001). Intraoperative variables (e.g. LSR persistence) demonstrated little additive value (Model 2) (AUC .67). Patients with a higher risk score (three or greater) demonstrated greater odds of persistent HFS at the time of discharge (OR 1.5 [95%CI 1.16-1.97]; p=.035), as well as greater odds of persistent or recurrent spasm at the time of follow-up (OR 3.0 [95%CI 1.52-5.95]; p=.002) Conclusions: A risk assessment model consisting of pre-operative clinical characteristics is useful in prognosticating HFS persistence at follow-up.

Scientific skepticism and new discoveries: an analysis of a report of zinc/phytase supplementation and the efficacy of botulinum toxins in treating cosmetic facial rhytides, hemifacial spasm and benign essential blepharospasm.

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Cohen, Joel L

2014-10-01

A recent paper in the Journal of Drugs in Dermatology by Koshy and colleagues (2012, 11( 4 ):507-512) report on "Effect of Dietary Zinc and Phytase Supplementation on Botulinum Toxin Treatments" and conclude by claiming the discovery of "a potentially meaningful role for zinc and/or phytase supplementation in increasing the degree and duration of botulinum toxin effect in the treatment of cosmetic facial rhytids, benign essential blepharospasm, and hemifacial spasm". The purpose of this paper is to examine these published claims for possible methodological and design errors and potential sources of bias. The authors evaluated the published results in comparison to the published literature on zinc deficiency, the role of phytase, prior reports of an effect of zinc on activity of botulinum toxin, issues of study design and execution and if the reported results of the study supported the study's conclusions. Multiple issues are present in the reported study, which appear to invalidate its conclusions. These areas include lack of direct evidence for the presence of clinical or subclinical zinc deficiency in the study population or for the level of phytate in the study population sufficient to interfere with zinc absorption in these subjects. Additionally, there is ambiguity as to the actual dose of zinc used as well as in the study design itself. Also there is a failure of the study through the "unmasking" of the crossover design. There is potential financial conflict of interest in the study execution that may have biased the reported results. Finally there is inadequate data presented to evaluate the claims made of a "new discovery" as to the three disease entities reported on and the various botulinum toxins used in each of the three treatment arms of the study. Based on this evaluation, it appears that a high level of clinical and scientific skepticism is warranted concerning any claim of a beneficial effect of zinc and phytase supplementation on the efficacy or

Efficacy of levetiracetam in primary hemifacial spasm.

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Kuroda, Takeshi; Saito, Yu; Fujita, Kazuhisa; Yano, Satoshi; Ishigaki, Seiichiro; Kato, Hirotaka; Murakami, Hidetomo; Ono, Kenjiro

2016-12-01

Hemifacial spasm (HFS) is a peripherally-induced movement disorder characterized by the involuntary, unilateral, intermittent, irregular, tonic or clonic contractions of muscles innervated by the ipsilateral facial nerve. Kindling-like hyperactivity of the facial nucleus induced by constant stimulation of compressing artery is considered as the predominant mechanism underlying the pathogenesis of HFS. As a treatment for HFS, microsurgical decompression and botulinum toxin injection have been shown to be highly successful. Anticonvulsant drugs relieve HFS in some patients; however, the use of such drugs is limited owing to their side effects, predominantly in elderly patients. We experienced two elderly HFS patients who exhibited a marked response to levetiracetam (LEV) without side effects. Although the exact underlying pharmacological mechanism remains unknown, we assume anti-kindling effect as one of the important pharmacological mechanism underlying the effect of LEV against HFS. Moreover, LEV is considered to be suitable for use in elderly patients because of its good tolerability. In addition, the lack of hepatic induction or inhibition makes it an easy and safe drug when used in addition to other anticonvulsants. Although the long-term benefit remains unknown, LEV may represent an alternative treatment for elderly HFS patients who are unable to undergo or decline surgical intervention and/or botulinum toxin injections or are intolerant to other anticonvulsants. Copyright © 2016 Elsevier Ltd. All rights reserved.

Effect of Hemifacial Spasm on Intraocular Pressure Measurement.

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Cicik, Erdogan; Yildirim, Rengin; Arici, Ceyhun; Dikkaya, Funda; Arslan, Osman Sevki

2018-01-01

To evaluate the effect of hemifacial spasm (HFS) on intraocular pressure (IOP) measurement. Twenty-four consecutive patients with HFS and 25 age- and gender-matched randomly selected eyes of healthy volunteers underwent corneal pachymetry and IOP measurements using Goldmann applanation tonometer (GAT) and noncontact tonometer (NCT). IOP measurements were performed before (during HFS) and 2 weeks after Botox injections in HFS patients and in healthy volunteers without Botox injections. There was no statistical difference between involved eye side and uninvolved eye side of HFS patients in measured central corneal thickness. Similarly, no difference was found between involved eye side of HFS patients and controls. There were no statistically significant differences comparing IOP values before treatment and levels measured at 2 weeks of Botox injections, either with GAT ( p = 0.33, 0.11) or NCT ( p = 0.80, 0.43) devices in the involved eyes and uninvolved eyes of patients with HFS, respectively. There were also no significant differences in these parameters (GAT ( p = 0.63) and NCT ( p = 0.54)) in controls. Contractions in facial muscles may not lead to significant increase in IOP in HFS patients. This result may help clinical decision making in the treatment of glaucoma patients with HFS. This trial is registered with NCT03390803.

Blepharospasm in children and adolescents.

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Kinard, Krista; Miller, Neil R; Digre, Kathleen B; Katz, Bradley J; Crum, Alison V; Warner, Judith E A

2016-02-01

Benign essential blepharospasm (BEB) generally is considered a disorder of adults; however, it rarely can present in childhood or adolescence. The main purpose of this study was to determine the prevalence of BEB in children and adolescents. Our research question was whether blepharospasm is seen in children or adolescents as well as in the adult population. We conducted a retrospective chart review at the University of Utah and Johns Hopkins University. We reviewed our databases for diagnoses of blepharospasm and tic disorder over the past 10 years in patients of all ages. Charts then were reviewed to confirm the diagnosis, and a questionnaire was sent to subjects whose blepharospasm had apparently begun before age 20 years. We identified 26 patients diagnosed with eyelid spasms that had begun while under the age of 20. We confirmed BEB in four of these cases. Of these individuals, all had developed symptoms in adolescence or before and all were still symptomatic but had noted improvement in the severity and frequency of their symptoms. Although rare, BEB can develop in the first decade of life, producing symptoms and signs that are similar to adults, with persistence into adulthood.

Altered spontaneous brain activity in patients with hemifacial spasm: a resting-state functional MRI study.

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Ye Tu

Full Text Available Resting-state functional magnetic resonance imaging (fMRI has been used to detect the alterations of spontaneous neuronal activity in various neurological and neuropsychiatric diseases, but rarely in hemifacial spasm (HFS, a nervous system disorder. We used resting-state fMRI with regional homogeneity (ReHo analysis to investigate changes in spontaneous brain activity of patients with HFS and to determine the relationship of these functional changes with clinical features. Thirty patients with HFS and 33 age-, sex-, and education-matched healthy controls were included in this study. Compared with controls, HFS patients had significantly decreased ReHo values in left middle frontal gyrus (MFG, left medial cingulate cortex (MCC, left lingual gyrus, right superior temporal gyrus (STG and right precuneus; and increased ReHo values in left precentral gyrus, anterior cingulate cortex (ACC, right brainstem, and right cerebellum. Furthermore, the mean ReHo value in brainstem showed a positive correlation with the spasm severity (r = 0.404, p = 0.027, and the mean ReHo value in MFG was inversely related with spasm severity in HFS group (r = -0.398, p = 0.028. This study reveals that HFS is associated with abnormal spontaneous brain activity in brain regions most involved in motor control and blinking movement. The disturbances of spontaneous brain activity reflected by ReHo measurements may provide insights into the neurological pathophysiology of HFS.

Prevalence of Bruxism in Hemifacial-Spasm Patients.

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Ella, Bruno; Guillaud, Etienne; Langbour, Nicolas; Guehl, Dominique; Burbaud, Pierre

2017-06-01

A previous study reported an increased prevalence of bruxism (25%) in patients with cranio-cervical dystonia (CCD) compared to normal controls (13%). CCD can affect the muscles of the head and neck. Besides the CCD affecting these muscles, hemifacial spasm (HFS) is a form of peripheral myoclonus due to a neurovascular conflict affecting the muscles of the face. The fact that they affect the same muscle regions could lead to other links in clinical manifestations such as bruxism, which is more common in patients with CCD than in the normal population. The aim was to study the prevalence of bruxism in patients with HFS. Patients with HFS were enrolled in the department of clinical neurophysiology (Bordeaux University Hospital) over a 6-month period. They were paired regarding age, the absence of neurological pathology or neuroleptics intake. To be included in the study, patients needed to have had unilateral involuntary facial muscle contractions affecting one hemiface. A hetero-questionnaire and a clinicial study were performed. The diagnostic criteria of bruxism included parafunction items such as grinding and clenching and at least one of the following clinical signs: abnormal tooth wear, temporomandibular joint (TMJ) pain, TMJ clicking, muscle hypertonia (masseter or temporal muscles). Additional epidemiological data were collected including age, sex, disease duration, stress, and sleep disorders. Stress symptoms inventory included symptoms like depression, strong heartbeat, dry mouth, anger, inability to concentrate, weakness, fatigability, insomnia, headache, and excessive sweating. The sleep disorder diagnosis included at least two of the symptoms described in the ICSD-3. All these criteria were recorded as either present (scored "1") or absent (scored "0"). The prevalence of bruxism in the two groups (normal and HFS) was not significantly different (p = 0.37). The rate was not significantly different between sleep and awake bruxism (p = 0.15) in both groups

MICROVASCULAR DECOMPRESSION FOR HEMIFACIAL SPASM--TECHNICAL NOTES AND COMPLICATION PRE VENTION: EXPERIENCE OF 338 CASES

Institute of Scientific and Technical Information of China (English)

赵卫国; 沈建康; 成侃; 胡锦清; 濮春华; 卞留贯; 孙青芳; 朱军

2003-01-01

Objective To summarize our clinical experience of microvascular decompression (MVD) for medically intractable hemifacial spasm(HFS) patients with emphasis on microsurgical manipulation and to improve cure rate and avoid surgical complications.MethodsThree hundred and thirty eight patients with HFS underwent MVD under general anesthesia. With the help of "zero retraction" technique, prosthesis can be properly inserted between offending vessel loop and affected facial nerve REZ in a "rolling ball" fashion under operative microscope.ResultsSurgical intervention achieved high relief rate of 91.4% and no major complications, with low recurrence rate of only 3.2% after averaging more than two years follow up (M=32 months).ConclusionIt is possible to approach to the facial nerve REZ with "zero retraction", which is fundamentally important to clear from cranial nerve and cerebellar injury. Skilled microsurgical technique along with correct recognition and mobilization of offending vessels are a must to assure MVD a highly efficacious and low risk treatment of choice for HFS patients.

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

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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.

A Case with Improvement of Blepharospasm by Zolpidem

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Munkyung Sunwoo

2011-05-01

Full Text Available Zolpidem is usually used for the treatment of insomnia as a hypnotic drug. It was also suggested to be effective in the treatment of dystonia in some studies. A 74-year-old woman had been suffering from frequent and intense bilateral spasms of the eyelids for 20 years. She has been treated with botulinum toxin injection and taken some medications. But, she experienced a little effect and was not satisfied with those treatments. Her symptom was improved after taking Zolpidem which had been prescribed for insomnia by her primary physician. She did not show any improvement after placebo injection and neostigmine test. This is the first report which shows improvement of isolated blepharospasm by Zolpidem in Korea. Zolpidem can be one of useful alternative pharmacological treatments for blepharospasm. Further randomized, blinded, placebo-controlled studies are needed to validate this finding.

Treatment of hemifacial spasm with botulinum toxin type a: effective, long lasting and well tolerated

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Jean Pierre Mette Batisti

Full Text Available ABSTRACT Hemifacial spasm (HFS is a common movement disorder characterized by involuntary tonic or clonic contractions of the muscles innervated by the facial nerve. Objective To evaluate the long-term effect of botulinum toxin type A (BTX-A in the treatment of HFS. Methods A retrospective analysis of patients treated at the Movement Disorders Outpatient Clinic in the Neurology Service, Hospital de Clínicas, Federal University of Paraná, Curitiba, from 2009 to 2013 was carried out. A total of 550 BTX-A injections were administered to 100 HFS patients. Results Mean duration of improvement following each injection session was 3.1 months, mean latency to detection of improvement was 7.1 days and mean success rate was 94.7%. Patients were evaluated at an interval of 5.8 months after each application. Adverse effects, which were mostly minor, were observed in 37% of the patients at least once during follow-up. The most frequent was ptosis (35.1%. Conclusion Treatment of HFS with BTX-A was effective, sustainable and safe and had minimal, well-tolerated side effects.

Asian over-representation among patients with hemifacial spasm compared to patients with cranial-cervical dystonia.

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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.

Efficacy of Botulinum Toxin Injections in the Treatment of Various Types of Facial Region Disorders

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Arzu Çoban

2012-12-01

Full Text Available OBJECTIVE: Local injection of botulinum toxin is a highly effective treatment option for a wide range of movement disorders and there are reliable sources of information on its indications, effects and safety in clinical practice. In this study, we report our experience with botulinum toxin in the treatment of facial region disorders. METHODS: Patients who had been followed in the Botulinum Toxin Outpatient Clinic of the Neurology Department were retrospectively evaluated. Two preparations of botulinum toxin type A (BT-A were used. The efficacy of BT-A injections was rated according to the improvement in symptoms as follows: marked - 75-100% improvement, good - 50-74%, moderate - 25-49%, and insufficient - less than 25% symptom relief. RESULTS: One hundred eighty-two patients (73 male, 109 female with various facial region disorders were included. The efficacy rates for patients who had very good and good improvement were high in the treatment of blepharospasm, hemifacial spasm, facial synkinesis, and Meige syndrome and moderate for oromandibular dystonia and hypersalivation. Ptosis was the most common side effect. CONCLUSION: According to our results, botulinum toxin was very effective treatment for blepharospasm, Meige syndrome, hemifacial spasm and facial synkinesis, whereas it demonstrated good efficacy in oromandibular dystonia and hypersalivation

Botulinum toxin A is effective to treat tension-type headache caused by hemifacial spasm.

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Mizuma, Atsushi; Nagata, Eiichiro; Yasuda, Takashi; Kouchi, Maiko; Nakayama, Taira; Honma, Kazunari; Tokuoka, Kentaro; Kitagawa, Yasuhisa; Nogawa, Shigeru; Takizawa, Shunya

2017-10-01

We examined the relationship between hemifacial spasm (HFS; a form of cranio-cervical dystonia) and chronic primary headache, including tension-type headache (TTH). We also examined whether botulinum toxin A (BoNT/A) therapy for HFS ameliorates concomitant TTH. Fifty-one HFS patients receiving BoNT/A therapy were recruited. Patients' characteristics (including age, gender, chronic headache history, exercise habits, stiff neck, cervical spondylolysis history), stress factors, worsening/new onset of headache associated with HFS, and dose of BoNT/A were examined. We diagnosed headache types according to The International Classification of Headache Disorders, 3rd edition, beta. Numerical Rating Scale (NRS) and Headache Impact Test-6 (HIT-6) scores for headache severity were compared between the 6-week baseline before BoNT/A therapy and 6-week follow-up after BoNT/A therapy. Of 51 patients with HFS, 17 (33.3%) reported worsening or new onset of headache (especially TTH) associated with HFS (Group-S), and 34 were not aware of headache (Group-N). Twelve patients (70.6%) in group-S reported improvement of headache after BoNT/A therapy. NRS (from 7 [5-9] to 0 [0-5], pheadache (odds ratio 28.53: 2.96-275.10, pheadache, especially TTH, is associated with HFS. BoNT/A therapy for HFS may also be indirectly effective for treatment of TTH. Copyright © 2017 Elsevier Ltd. All rights reserved.

Hemifacial spasm in a patient with neurofibromatosis and Arnold-Chiari malformation: a unique case association Espasmo hemifacial em paciente com neurofibromatose e malformação de Arnold-Chiari: uma associação rara

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Andre Carvalho Felício

2007-09-01

Full Text Available BACKGROUND: The association of hemifacial spasm (HFS, Chiari type I malformation (CIM and neurofibromatosis type 1 (NF1 has not been described yet. CASE REPORT: We report the case of a 31-year-old woman with NF1 who developed a right-sided HFS. On magnetic resonance imaging (MRI a CIM was seen without syringomyelia. The patient has been successfully treated with botulinum toxin type A injections for 5 years without major side effects. CONCLUSION:Clinical features of HFS, CMI and NF1 are highlighted together with their possible relationship. Also, therapeutic strategies are also discussed.INTRODUÇÃO: A associação entre espasmo hemifacial (EHF, malformação de Chiari tipo I (MCI e neurofibromatose tipo I (NFI ainda não foi descrita. RELATO DO CASO: Relatamos o caso de mulher com 31 anos com NFI que desenvolveu EHF à direita. Na ressonância magnética (RM foi observada MCI sem seringomielia associada. A paciente foi tratada com sucesso com toxina botulínica tipo A por 5 anos sem efeitos colaterais. CONCLUSÃO: Ressaltamos as características clínicas do EHF, MCI e NFI assim como uma possível relação entre elas. Além disto, discutimos também estratégias terapêuticas.

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)

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

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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.

Long-term depression-like plasticity of the blink reflex for the treatment of blepharospasm.

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Kranz, Gottfried; Shamim, Ejaz A; Lin, Peter T; Kranz, George S; Hallett, Mark

2013-04-01

Our previous work showed a beneficial therapeutic effect on blepharospasm using slow repetitive transcranial magnetic stimulation, which produces a long-term depression (LTD)-like effect. High-frequency supraorbital electrical stimulation, asynchronous with the R2 component of the blink reflex, can also induce LTD-like effects on the blink reflex circuit in healthy subjects. Patients with blepharospasm have reduced inhibition of their blink recovery curves; therefore, a LTD-like intervention might normalize the blink reflex recovery (BRR) and have a favorable therapeutic effect. This is a randomized, sham-controlled, observer-blinded prospective study. In 14 blepharospasm patients, we evaluated the effects of high-frequency supraorbital stimulation on three separate treatment days. We applied 28 trains of nine stimuli, 400 Hz, either before or after the R2 or used sham stimulation. The primary outcome was the blink rate, number of spasms rated by a blinded physician and patient rating before, immediately after and 1 hour after stimulation while resting, reading, and talking; secondary outcome was the BRR. Stimulation "before" and "after" the R2 both showed a similar improvement as sham stimulation in physician rating, but patients felt significantly better with the before condition. Improvement in recovery of the blink reflex was noted only in the before condition. Clinical symptoms differed in the three baseline conditions (resting, reading, and talking). Stimulation before R2 increased inhibition in trigeminal blink reflex circuits in blepharospasm toward normal values and produced subjective, but not objective, improvement. Inhibition of the blink reflex pathway by itself appeared to be insufficient for a useful therapeutic effect. Copyright © 2013 Movement Disorder Society.

Clinical outcomes of individualized botulinum neurotoxin type A injection techniques in patients with essential blepharospasm.

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Sung, Youngje; Nam, Sang Min; Lew, Helen

2015-04-01

To assess the clinical outcomes following botulinum neurotoxin type A (BoNT-A) treatment with an individualized injection technique based on the types of spasms and to compare the results of the individualized injection technique with those of the conventional injection technique in the same patients. From November 2011 to July 2013, 77 BoNT-A injections were performed in 38 patients. Eighteen patients were treated with conventional BoNT-A injections before 2011, and 20 patients were referred to our hospital for unsatisfactory results after a conventional injection technique. We classified the patients by spasm-dominant sites: the lateral orbital area, representing the orbital orbicularis-dominant group (ODG); the glabella, representing the corrugator-dominant group (CDG); and the ptosis, representing the palpebral part of the orbicularis-dominant group (PDG). We increased the injection dose into the spasm-dominant sites of the blepharospasm groups. We assessed subjective symptom scores (functional disability score, FDS) after treatment. This study included 38 patients (26 women, 12 men; mean age, 60.6 ± 10.9 years). There were 21 patients in the ODG, 10 patients in the CDG, and 7 patients in the PDG. Mean ages were 59.7 ± 12.6, 59.8 ± 8.5, and 66.8 ± 9.0 years, and mean BoNT-A injection dose was 38.8 ± 11.2, 38.8 ± 11.2, and 38.8 ± 10.8 U in each group, respectively (p = 0.44, 0.82 Kruskal-Wallis test). Mean FDS after injection was 1.7 ± 0.7 in the ODG, 1.4 ± 0.8 in the CDG, and 1.2 ± 0.3 in the PDG. There were significant differences in reading and job scale among the three groups. In a comparison between the conventional and individualized injection techniques, there was a significant improvement in mean FDS and in the reading scale in the PDG with the individualized injection technique. The success rate was 92.1% in the conventional injection group and 94.1% in the individualized injection group. The individualized injection technique of Bo

The blepharospasm disability scale: an instrument for the assessment of functional health in blepharospasm

NARCIS (Netherlands)

Lindeboom, R.; de Haan, R.; Aramideh, M.; Speelman, J. D.

1995-01-01

Assessment of the functional status in patients with blepharospasm is of major importance for clinical practice and outcome studies. The Blepharospasm Disability Scale (BDS) is specifically directed to measure the disability in these patients. The metric properties of this instrument were evaluated.

Identification of offending vessele in trigeminal neuralgia and hemifacial spasm using SPGR-MRI and 3D-TOF-MRA

International Nuclear Information System (INIS)

Niwa, Yoshikazu; Shiotani, Masahiro; Karasawa, Hidetake; Ohseto, Kiyoshige; Naganuma, Yoshikazu

1996-01-01

We investigated 100 consecutive patients with trigeminal neuralgia (TN) and 53 patients with hemifacial spasm (HFS) concerning the anatomical relationship between the root entry (exit) zone (REZ) of cranial nerve and the offending artery, using spoiled GRASS MRI (SPGR-MRI) and three dimensional-time of fly-MRA (MRA). In 67 of 100 (67%) patients with TN, this new radiological method, SPGR-MRI and MRA demonstrated the relationship between the fifth cranial nerve root and offending artery causing neurovascular compression (NVC), and in 46 of 53 (87%) with HFS, demonstrated the similar relationship between seventh and eighth nerve complex and offending artery. Microvascular decompression (MVD) was performed in 10 with HFS, and NVC of the REZ of the facial nerve caused by the offending artery was exactly predicted by SPGR-MRI and MRA in 9 (90%). The combination of SPGR-MRI and MRA is very useful for demonstrating NVC as the cause of TN and HFS. On the other hand, we investigated asymptomatic 206 trigemimal and 253 facial nerves about the relationship between their REZ and the surrounding structures using the similar method. The contact of REZ of cranial nerve with surrounding artery is demonstrated in 31.6% of trigeminal nerves and in 22.5% of facial nerves. These results indicate that the contact of REZ of cranial nerve with surrounding artery is not rare in healthy subjects, though causing TN and HFS in particular patients. In this context, we discussed the difference between the contact which is asymptomatic and the compression which is symptomatic. (author)

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

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

2018-05-01

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 < 0.001). The 7.0-T scans have a clearer view of vessels in the cerebellopontine angle, which may have significant 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

Benign Essential Blepharospasm

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... the same for many years; and, in rare cases, improve spontaneously. Clinical Trials Throughout the U.S. and Worldwide NINDS Clinical Trials Related ... Definition Benign essential blepharospasm (BEB) is a progressive neurological ...

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

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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.

Simultaneous display of MRA and MPR in detecting vascular compression for trigeminal neuralgia or hemifacial spasm: comparison with oblique sagittal views of MRI

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Arbab, A.S.; Aoki, S.; Yoshikawa, T.; Kumagai, H.; Araki, T.; Nishiyama, Y.; Nagaseki, Y.; Nukui, H.

2000-01-01

A new technique, simultaneous display of magnetic resonance angiography (MRA) and multiplanar reconstruction (MPR), was performed by a workstation to identify the involved vessels in patients with trigeminal neuralgia (TN) or hemifacial spasm (HFS), and the results were compared with those of oblique sagittal MRI technique. Twelve patients with either HFS or TN were prospectively assessed by simultaneous display of MRA and MPR, and oblique sagittal techniques, to point out the neurovascular compression and to identify the involved vessels. Three-dimensional (3D) time-of-flight (TOF) spoiled gradient-echo (SPGR) images were acquired to create MRA and MPR. Oblique sagittal views were also created and displayed on films. A total of 15 vessels in 12 patients were identified as compressing vessels during surgery. Simultaneous display of MRA and MPR technique pointed out the presence of vessels at and/or around root entry/exit zone (REZ) in all 12 patients, but proper identification by the name of the individual vessel was correct in 13 of 15 cases. However, oblique sagittal technique indicated the presence of vessels at and/or around REZ in 11 patients, but only 8 of 14 vessels were correctly identified. Our new method, simultaneous display of MRA-MPR, facilitated correct identification of the involved vessels compared with the oblique sagittal view method. (orig.)

Preoperative assessment of trigeminal neuralgia and hemifacial spasm using constructive interference in steady state-three-dimensional fourier transformation magnetic resonance imaging

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Yamakami, Iwao; Kobayashi, Eiichi; Hirai, Shinji; Yamaura, Akira [Chiba Univ. (Japan). School of Medicine

2000-11-01

Results of microvascular decompression (MVD) for trigeminal neuralgia (TN) and hemifacial spasm (HFS) may be improved by accurate preoperative assessment of neurovascular relationships at the root entry/exit zone (REZ). Constructive interference in steady state (CISS)-three-dimensional Fourier transformation (3DFT) magnetic resonance (MR) imaging was evaluated for visualizing the neurovascular relationships at the REZ. Fourteen patients with TN and eight patients with HFS underwent MR imaging using CISS-3DFT and 3D fast inflow with steady-state precession (FISP) sequences. Axial images of the cerebellopontine angle (CPA) obtained by the two sequences were reviewed to assess the neurovascular relationships at the REZ of the trigeminal and facial nerves. Eleven patients subsequently underwent MVD. Preoperative MR imaging findings were related to surgical observations and results. CISS MR imaging provided excellent contrast between the cranial nerves, small vessels, and cerebrospinal fluid (CSF) in the CPA. CISS was significantly better than FISP for delineating anatomic detail in the CPA (trigeminal and facial nerves, petrosal vein) and abnormal neurovascular relationships responsible for TN and HFS (vascular contact and deformity at the REZ). Preoperative CISS MR imaging demonstrated precisely the neurovascular relationships at the REZ and identified the offending artery in all seven patients with TN undergoing MVD. CISS MR imaging has high resolution and excellent contrast between cranial nerves, small vessels, and CSF, so can precisely and accurately delineate normal and abnormal neurovascular relationships at the REZ in the CPA, and is a valuable preoperative examination for MVD. (author)

Preoperative assessment of trigeminal neuralgia and hemifacial spasm using constructive interference in steady state-three-dimensional fourier transformation magnetic resonance imaging

International Nuclear Information System (INIS)

Yamakami, Iwao; Kobayashi, Eiichi; Hirai, Shinji; Yamaura, Akira

2000-01-01

Results of microvascular decompression (MVD) for trigeminal neuralgia (TN) and hemifacial spasm (HFS) may be improved by accurate preoperative assessment of neurovascular relationships at the root entry/exit zone (REZ). Constructive interference in steady state (CISS)-three-dimensional Fourier transformation (3DFT) magnetic resonance (MR) imaging was evaluated for visualizing the neurovascular relationships at the REZ. Fourteen patients with TN and eight patients with HFS underwent MR imaging using CISS-3DFT and 3D fast inflow with steady-state precession (FISP) sequences. Axial images of the cerebellopontine angle (CPA) obtained by the two sequences were reviewed to assess the neurovascular relationships at the REZ of the trigeminal and facial nerves. Eleven patients subsequently underwent MVD. Preoperative MR imaging findings were related to surgical observations and results. CISS MR imaging provided excellent contrast between the cranial nerves, small vessels, and cerebrospinal fluid (CSF) in the CPA. CISS was significantly better than FISP for delineating anatomic detail in the CPA (trigeminal and facial nerves, petrosal vein) and abnormal neurovascular relationships responsible for TN and HFS (vascular contact and deformity at the REZ). Preoperative CISS MR imaging demonstrated precisely the neurovascular relationships at the REZ and identified the offending artery in all seven patients with TN undergoing MVD. CISS MR imaging has high resolution and excellent contrast between cranial nerves, small vessels, and CSF, so can precisely and accurately delineate normal and abnormal neurovascular relationships at the REZ in the CPA, and is a valuable preoperative examination for MVD. (author)

Screening for Cognitive Impairments in Primary Blepharospasm.

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Yang, Jing; Song, Wei; Wei, Qianqian; Ou, Ruwei; Cao, Bei; Liu, Wanglin; Shao, Na; Shang, Hui-Fang

2016-01-01

Studies have reported that non-motor symptoms are an important component of primary dystonia. However, evidence supporting cognitive impairment in primary dystonia is limited and contradictory. We applied the Chinese version of the Addenbrooke's Cognitive Examination-Revised and the Mini-Mental State Examination (MMSE) to screen for cognitive impairment in patients with primary blepharospasm. In addition, we investigated the relationship between performance on the Addenbrooke's Cognitive Examination-Revised and quality of life as measured by the Medical Outcomes Study 36-item Short-Form (SF36). The study included 68 primary blepharospasm patients and 68 controls matched by age, sex and education. The prevalence of cognitive deficits was 22.0% and 32.3% in primary blepharospasm patients group, as measured by the MMSE and the Addenbrooke's Cognitive Examination-Revised, respectively. Primary blepharospasm patents had a broad range of cognitive deficits, with the most frequently affected domains being visuospatial function (30.9%) and language (30.9%), followed by memory (27.9%), orientation/attention (26.4%) and verbal fluency (22.0%). Patients with cognitive deficits had lower total SF36 scores, especially in the subdomains of physical functioning, role-physical and social functioning, compared to those without cognitive deficits. Scores on the Addenbrooke's Cognitive Examination-Revised were significantly correlated with both the SF36 scores and the scores on the subdomains of physical functioning and social functioning. Some patients with primary blepharospasm have cognitive deficits. Poor performance on the Addenbrooke's Cognitive Examination-Revised is related to poorer quality of life.

Three-dimensional short-range MR angiography and multiplanar reconstruction images in the evaluation of neurovascular compression in hemifacial spasm

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Choi, Woo Suk; Kim, Eui Jong; Lee, Jae Gue; Rhee, Bong Arm [Kyunghee Univ. Hospital, Seoul (Korea, Republic of)

1998-08-01

To evaluate the diagnostic efficacy of three-dimensional(3D) short-range MR angiography(MRA) and multiplanar reconstruction(MPR) imaging in hemifacial spasm(HS). Materials and Methods : Two hundreds patients with HS were studied using a 1.5T MRI system with a 3D time-of-flight(TOF) MRA sequence. To reconstruct short-range MRA, 6-10 source images near the 7-8th cranial nerve complex were processed using a maximum-intensity projection technique. In addition, an MPR technique was used to investigate neurovascular compression. We observed the relationship between the root-exit zone(REZ) of the 7th cranial nerve and compressive vessel, and identified the compressive vessels on symptomatic sides. To investigate neurovascular contact, asymptomatic contralateral sides were also evaluated. Results : MRI showed that in 197 of 200 patients there was vascular compression or contact with the facial nerve REZ on symptomatic sides. One of the three remaining patients was suffering from acoustic neurinoma on the symptomatic side, while in two patients there were no definite abnormal findings.Compressive vessels were demonstrated in all 197 patients; 80 cases involved the anterior inferior cerebellar artery(AICA), 74 the posterior cerebellar artery(PICA), 13 the vertebral artery(VA), 16 the VA and AICA, eight the VA and PICA, and six the AICA and PICA. In all 197 patients, compressive vessels were reconstructed on one 3D short-range MRA image without discontinuation from vertebral or basilar arteries. 3D MPR studies provided additional information such as the direction of compression and course of the compressive vessel. In 31 patients there was neurovascular contact on the contralateral side at the 7-8th cranial nerve complex. Conclusion : Inpatients with HS, 3D short-range MRA and MPR images are excellent and very helpful for the investigation of neurovascular compression and the identification of compressive vessels.

Three-dimensional short-range MR angiography and multiplanar reconstruction images in the evaluation of neurovascular compression in hemifacial spasm

International Nuclear Information System (INIS)

Choi, Woo Suk; Kim, Eui Jong; Lee, Jae Gue; Rhee, Bong Arm

1998-01-01

To evaluate the diagnostic efficacy of three-dimensional(3D) short-range MR angiography(MRA) and multiplanar reconstruction(MPR) imaging in hemifacial spasm(HS). Materials and Methods : Two hundreds patients with HS were studied using a 1.5T MRI system with a 3D time-of-flight(TOF) MRA sequence. To reconstruct short-range MRA, 6-10 source images near the 7-8th cranial nerve complex were processed using a maximum-intensity projection technique. In addition, an MPR technique was used to investigate neurovascular compression. We observed the relationship between the root-exit zone(REZ) of the 7th cranial nerve and compressive vessel, and identified the compressive vessels on symptomatic sides. To investigate neurovascular contact, asymptomatic contralateral sides were also evaluated. Results : MRI showed that in 197 of 200 patients there was vascular compression or contact with the facial nerve REZ on symptomatic sides. One of the three remaining patients was suffering from acoustic neurinoma on the symptomatic side, while in two patients there were no definite abnormal findings.Compressive vessels were demonstrated in all 197 patients; 80 cases involved the anterior inferior cerebellar artery(AICA), 74 the posterior cerebellar artery(PICA), 13 the vertebral artery(VA), 16 the VA and AICA, eight the VA and PICA, and six the AICA and PICA. In all 197 patients, compressive vessels were reconstructed on one 3D short-range MRA image without discontinuation from vertebral or basilar arteries. 3D MPR studies provided additional information such as the direction of compression and course of the compressive vessel. In 31 patients there was neurovascular contact on the contralateral side at the 7-8th cranial nerve complex. Conclusion : Inpatients with HS, 3D short-range MRA and MPR images are excellent and very helpful for the investigation of neurovascular compression and the identification of compressive vessels

Hemilingual spasm: defining a new entity, its electrophysiological correlates and surgical treatment through microvascular decompression.

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Osburn, Leisha L; Møller, Aage R; Bhatt, Jay R; Cohen-Gadol, Aaron A

2010-07-01

We report on vascular compression syndrome of the 12th cranial nerve (hypoglossal), an occurrence not previously reported, and demonstrate, through corresponding objective electrophysiological evidence, that microvascular decompression of the hypoglossal nerve root can cure hemilingual spasm. A 52-year-old man had lower face muscle twitching and tongue spasms, which worsened with talking, chewing, or emotional stress. Carbamazepine offered only temporary relief, and relief from injections of botulinum toxin was insignificant. He was referred for surgical treatment. High-resolution magnetic resonance imaging of his posterior fossa contents revealed no obvious evidence of any compressive vessel along the facial nerve, but a compressive vessel along the hypoglossal nerve was apparent. The presence of preoperative tongue spasms encouraged interoperative monitoring of tongue motor responses. The facial nerve exit zone was explored, but microsurgical inspection of the seventh/eighth cranial nerve complex did not reveal any compressive vessel. However, at the anterolateral aspect of the medulla oblongata, the hypoglossal nerve was clearly compressed and distorted laterally by a large tortuous vertebral artery. When the artery was mobilized away from the nerve, the abnormal late electromyographic response to transcranial electrical stimulation disappeared; immediately after shredded Teflon was interpositioned between the artery and the nerve, the abnormal spontaneous tongue fasciculation also disappeared. The patient has remained spasm free 6 months after surgery. Hemilingual spasm may be caused by vascular contact/compression along cranial nerve XII at the lower brainstem and belong to the same family of cranial nerve hyperactivity disorders as hemifacial spasm.

Glucose hypermetabolism in the thalamus of patients with drug-induced blepharospasm.

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Suzuki, Y; Kiyosawa, M; Wakakura, M; Mochizuki, M; Ishiwata, K; Oda, K; Ishii, K

2014-03-28

We examined the difference in cerebral function alterations between drug-induced blepharospasm patients and essential blepharospasm (EB) patients by using positron emission tomography with (18)F-fluorodeoxyglucose. Cerebral glucose metabolism was examined in 21 patients with drug-induced blepharospasm (5 men and 16 women; mean age, 53.1 [range, 29-78] years), 21 essential EB patients (5 men and 16 women; mean age, 53.0 [range, 33-72] years) and 24 healthy subjects (6 men and 18 women; mean age, 57.9 [range, 22-78] years) with long-term history of benzodiazepines use (drug healthy subjects). Drug-induced blepharospasm patients developed symptoms while taking benzodiazepines or thienodiazepines. Sixty-three normal volunteers (15 men and 48 women; mean age, 53.6 [range, 20-70] years) were examined as controls. Differences between the patient groups and control group were examined by statistical parametric mapping. Additionally, we defined regions of interests on both sides of the thalamus, caudate nucleus, anterior putamen, posterior putamen and primary somatosensory area. The differences between groups were tested using two-sample t-tests with Bonferroni correction for multiple comparisons. Cerebral glucose hypermetabolism on both side of the thalamus was detected in drug-induced blepharospasm, EB patients and drug healthy subjects by statistical parametric mapping. In the analysis of regions of interest, glucose metabolism in both sides of the thalamus in the drug-induced blepharospasm group was significantly lower than that in the EB group. Moreover, we observed glucose hypermetabolism in the anterior and posterior putamen bilaterally in EB group but not in drug-induced blepharospasm group and drug healthy subjects. Long-term regimens of benzodiazepines or thienodiazepines may cause down-regulation of benzodiazepine receptors in the brain. We suggest that the functional brain alteration in drug-induced blepharospasm patients is similar to that in EB patients, and

Hemifacial Spasm

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... the face (facial). The disorder occurs in both men and women, although it more frequently affects middle-aged or elderly women. It is much more ... the face (facial). The disorder occurs in both men and women, although it more frequently affects middle-aged or elderly women. It is much more ...

Hemifacial microsomia: A case report

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Kapur R

2008-05-01

Full Text Available Hemifacial microsomia is a congenital malformation in which there is a deficiency in the amount of hard and soft tissue on one side of the face. It is primarily a syndrome of the first branchial arch, involving underdevelopment of the temporomandibular joint, mandibular ramus, masticatory muscles and the ear. The affected ear may have an external soft-tissue malformation in addition to being lower set than on the contra lateral side. Hearing loss may result from underdevelopment of the osseous components of the auditory system and a diminished or absent external auditory meatus. Occasionally, second branchial arch defects involving the facial nerve and facial muscles coexist with Hemifacial microsomia. Radiographic examination in case of Hemifacial microsomia is of limited value because of superimposition of normal and abnormal bony structures. The skeletal and soft-tissue findings of a patient with Hemifacial microsomia who underwent three-dimensional computerized tomography is presented here to improve our knowledge and diagnostic skill of this uncommon entity.

Genetics Home Reference: benign essential blepharospasm

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... eyes, and eye irritation that is aggravated by wind, air pollution, sunlight, and other irritants. These symptoms ... find a genetics professional in my area? Other Names for This Condition essential blepharospasm eyelid twitching primary ...

The use of botulinum toxin in head and face medicine: An interdisciplinary field

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Laskawi Rainer

2008-03-01

Full Text Available Abstract Background In this review article different interdisciplinary relevant applications of botulinum toxin type A (BTA in the head and face region are demonstrated. Patients with head and face disorders of different etiology often suffer from disorders concerning their musculature (example: synkinesis in mimic muscles or gland-secretion. This leads to many problems and reduces their quality of life. The application of BTA can improve movement disorders like blepharospasm, hemifacial spasm, synkinesis following defective healing of the facial nerve, palatal tremor, severe bruxism, oromandibular dystonias hypertrophy of the masseter muscle and disorders of the autonomous nerve system like hypersalivation, hyperlacrimation, pathological sweating and intrinsic rhinitis. Conclusion The application of botulinum toxin type A is a helpful and minimally invasive treatment option to improve the quality of life in patients with head and face disorders of different quality and etiology. Side effects are rare.

Hemifacial Microsomia: A Case Report and Overview

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Medhini Madi

2014-06-01

Full Text Available Hemifacial Microsomia is a developmental craniofacial anomaly typically displaying reduced growth and development of half of the face as a result of abnormal development of first and second branchial arches. The patients with Hemifacial Microsomia usually exhibit unilateral involvement of the face but occasionally might involve both the sides. Cases which show vertebral anomalies and epibulbar dermoids have been considered to form a separate category within this condition . The condition is now known to be extremely complex and heterogenous. Here we present a case of Hemifacial Microsomia with its characteristic clinical and radiographic features that will help us in diagnosing and differentiating this rare entity from other closely related syndromes [Cukurova Med J 2014; 39(3.000: 625-635

Lipomatosis: a diverse form of hemifacial hyperplasia

International Nuclear Information System (INIS)

Arora, Preeti Chawla; Umarji, Hemant R.; Arora, Aman; Ramaswami, Easwaran

2012-01-01

A case of hemifacial hyperplasia that presented with muscular, skeletal, and dental hyperplasia along with lipomatous infiltration was described. Advanced imaging was useful in identifying the lipomatous infiltration present in the lesion, which raises the possibility of lipomatosis having a diverse presentation in hemifacial hyperplasia. As there was a scarcity of related literature in the field of dentomaxillofacial radiology, this report would make us familiar with its computed tomographic and magnetic resonance image findings.

Acute hemifacial dystonia possibly induced by clebopride.

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Bosco, Domenico; Plastino, Massimiliano; Marcello, Maria Giovanna; Mungari, Pasquale; Fava, Antonietta

2009-01-01

Dystonic reactions produce twisting and repetitive movements or abnormal posturing. Severe dystonic reactions have been shown to occur in concert with numerous medications. This report details the case of a patient who developed hemifacial dystonia as acute side reaction from administration of clebopride for dyspeptic prophylaxis. When the drug was immediately stopped, the dystonic posture disappeared completely within 2 weeks. The use of clebopride may be associated with not only a reversible or persistent parkinsonism syndrome but also hemifacial dystonia; therefore, attention must be drawn to this possible side effect.

Crotoxin in humans: analysis of the effects on extraocular and facial muscles

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Geraldo de Barros Ribeiro

2012-12-01

Full Text Available PURPOSE: Crotoxin is the main neurotoxin of South American rattlesnake Crotalus durissus terrificus. The neurotoxic action is characterized by a presynaptic blockade. The purpose of this research is to assess the ability of crotoxin to induce temporary paralysis of extraocular and facial muscles in humans. METHODS: Doses of crotoxin used ranged from 2 to 5 units (U, each unit corresponding to one LD50. We first applied 2U of crotoxin in one of the extraocular muscles of 3 amaurotic individuals to be submitted to ocular evisceration. In the second stage, we applied crotoxin in 12 extraocular muscles of 9 patients with strabismic amblyopia. In the last stage, crotoxin was used in the treatment of blepharospasm in another 3 patients. RESULTS: No patient showed any systemic side effect or change in vision or any eye structure problem after the procedure. The only local side effects observed were slight conjunctival hyperemia, which recovered spontaneously. In 2 patients there was no change in ocular deviation after 2U crotoxin application. Limitation of the muscle action was observed in 8 of the 12 applications. The change in ocular deviation after application of 2U of crotoxin (9 injections was in average 15.7 prism diopters (PD. When the dose was 4U (2 applications the change was in average 37.5 PD and a single application of 5U produced a change of 16 PD in ocular deviation. This effect lasted from 1 to 3 months. Two of the 3 patients with blepharospasm had the hemifacial spasm improved with crotoxin, which returned after 2 months. CONCLUSIONS: This study provides data suggesting that crotoxin may be a useful new therapeutic option for the treatment of strabismus and blepharospasm. We expect that with further studies crotoxin could be an option for many other medical areas.

Crotoxin in humans: analysis of the effects on extraocular and facial muscles.

Science.gov (United States)

Ribeiro, Geraldo de Barros; Almeida, Henderson Celestino de; Velarde, David Toledo

2012-01-01

Crotoxin is the main neurotoxin of South American rattlesnake Crotalus durissus terrificus. The neurotoxic action is characterized by a presynaptic blockade. The purpose of this research is to assess the ability of crotoxin to induce temporary paralysis of extraocular and facial muscles in humans. Doses of crotoxin used ranged from 2 to 5 units (U), each unit corresponding to one LD50. We first applied 2U of crotoxin in one of the extraocular muscles of 3 amaurotic individuals to be submitted to ocular evisceration. In the second stage, we applied crotoxin in 12 extraocular muscles of 9 patients with strabismic amblyopia. In the last stage, crotoxin was used in the treatment of blepharospasm in another 3 patients. No patient showed any systemic side effect or change in vision or any eye structure problem after the procedure. The only local side effects observed were slight conjunctival hyperemia, which recovered spontaneously. In 2 patients there was no change in ocular deviation after 2U crotoxin application. Limitation of the muscle action was observed in 8 of the 12 applications. The change in ocular deviation after application of 2U of crotoxin (9 injections) was in average 15.7 prism diopters (PD). When the dose was 4U (2 applications) the change was in average 37.5 PD and a single application of 5U produced a change of 16 PD in ocular deviation. This effect lasted from 1 to 3 months. Two of the 3 patients with blepharospasm had the hemifacial spasm improved with crotoxin, which returned after 2 months. This study provides data suggesting that crotoxin may be a useful new therapeutic option for the treatment of strabismus and blepharospasm. We expect that with further studies crotoxin could be an option for many other medical areas.

Profile of Xeomin® (incobotulinumtoxinA for the treatment of blepharospasm

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Park J

2011-06-01

Full Text Available Juwan Park1, Michael S Lee2, Andrew R Harrison31Department of Ophthalmology, The Catholic University of Korea, Seoul, Korea; 2Department of Ophthalmology, Neurology and Neurosurgery, 3Department of Ophthalmology and Otolaryngology, University of Minnesota, MN, USAAbstract: Even though conventional botulinum neurotoxin (BoNT products have shown successful treatment results in patients with benign blepharospasm (BEB, the main, potential long-term side effect of BoNT use is the development of immunologic resistance due to the production of neutralizing antibody to the neurotoxin after repeated injections. Xeomin® (incobotulinumtoxinA, a unique botulinum neurotoxin type A (BoNT/A drug free of complexing proteins otherwise contained in all conventional BoNT/A drugs, was recently approved by US Food and Drug Administration for the treatment of cervical dystonia or blepharospasm in adults. The newly approved BoNT/A drug may overcome this limitation of previous conventional products, since it contains pure neurotoxin (150 kDa through a manufacturing process that separates it from complexing proteins such as hemagglutinins produced by fermentation of Clostridium botulinum. Many studies have also shown that Xeomin® has the same efficacy and safety profile as complexing protein-containing products such as Botox® and is exchangeable with Botox® using a simple 1:1 conversion ratio. Xeomin® represents a new treatment option for the repeated treatment of patients with blepharospasm in that it may reduce antibody-induced therapy failure. But, long-term comparative trials in naïve patients between Xeomin® and conventional BoNT/A drugs are required to confirm the low immunogenicity of Xeomin®.Keywords: blepharospasm, botulinum neurotoxin type A, Xeomin®, incobotulinumtoxinA, complexing proteins, neutralizing antibodies

Gamma knife radiosurgery for cerebellopontine angle epidermoid tumors.

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El-Shehaby, Amr M N; Reda, Wael A; Abdel Karim, Khaled M; Emad Eldin, Reem M; Nabeel, Ahmed M

2017-01-01

Intracranial epidermoid tumors are commonly found in the cerebellopontine angle where they usually present with either trigeminal neuralgia or hemifacial spasm. Radiosurgery for these tumors has rarely been reported. The purpose of this study is to assess the safety and clinical outcome of the treatment of cerebellopontine epidermoid tumors with gamma knife radiosurgery. This is a retrospective study involving 12 patients harboring cerebellopontine angle epidermoid tumors who underwent 15 sessions of gamma knife radiosurgery. Trigeminal pain was present in 8 patients and hemifacial spasm in 3 patients. All cases with trigeminal pain were receiving medication and still uncontrolled. One patient with hemifacial spasm was medically controlled before gamma knife and the other two were not. Two patients had undergone surgical resection prior to gamma knife treatment. The median prescription dose was 11 Gy (10-11 Gy). The tumor volumes ranged from 3.7 to 23.9 cc (median 10.5 cc). The median radiological follow up was 2 years (1-5 years). All tumors were controlled and one tumor shrank. The median clinical follow-up was 5 years. The trigeminal pain improved or disappeared in 5 patients, and of these, 4 cases stopped their medication and one decreased it. The hemifacial spasm resolved in 2 patients who were able to stop their medication. Facial palsy developed in 1 patient and improved with conservative treatment. Transient diplopia was also reported in 2 cases. Gamma knife radiosurgery provides good clinical control for cerebellopontine angle epidermoid tumors.

Observational Study of IncobotulinumtoxinA for Cervical Dystonia or Blepharospasm (XCiDaBLE: Interim Results for the First 170 Subjects with Blepharospasm

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Hubert H. Fernandez

2014-07-01

Full Text Available Background: XCiDaBLE is a large, prospective, observational “naturalistic” study evaluating Xeomin® for Cervical Dystonia or BLEpharospasm in the United States. We report the interim results from the blepharospasm cohort of XCiDaBLE.Methods: Subjects (≥18 years old with blepharospasm were followed for two treatment cycles of incobotulinumtoxinA and monitored for 4 weeks after injection via interactive voice/web response system (IVRS/IWRS. The investigator‐reported scale includes the Clinical Global Impression Scale‐Severity subscale (CGI‐S. Patient‐reported outcome measures include the Patient Global Impression Scale‐Severity (PGI‐S and ‐Improvement (PGI‐I subscales, Jankovic Rating Scale (JRS, SF‐12v2® health survey, and Work Productivity and Activity Impairment questionnaire. Subjects are seen by the investigator at baseline (including the first injection, during the second injection, and at a final study visit (12 weeks after the second injection.Results: One hundred seventy subjects were included in this interim analysis. The majority of subjects were female (77.1% and white (91.8%, and had previously been treated with botulinum toxins (96.5%. The mean total dose (both eyes was 71.5 U of incobotulinumtoxinA for the first injection. PGI‐S, PGI‐I, and JRS scores were significantly improved 4 weeks after treatment (all p<0.0001. No differences were noted in either quality of life (QoL or work productivity in this short assessment period. No unexpected adverse events occurred.Discussion: This is an interim study and assessment method based on an IVRS/IWRS. In this predominantly toxin‐experienced cohort, significant benefits in specific and global measures of disease severity were seen in the immediate post‐incobotulinumtoxinA injection period. It will be interesting to see if there are improvements in QoL with consistent individualized injections over a longer period.

Teflon granulomas mimicking cerebellopontine angle tumors following microvascular decompression.

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Deep, Nicholas L; Graffeo, Christopher S; Copeland, William R; Link, Michael J; Atkinson, John L; Neff, Brian A; Raghunathan, Aditya; Carlson, Matthew L

2017-03-01

To report two patients with a history of microvascular decompression (MVD) for hemifacial spasm who presented with Teflon granulomas (TG) mimicking cerebellopontine angle (CPA) tumors and to perform a systematic review of the English-language literature. Case series at a single tertiary academic referral center and systematic review. Retrospective chart review with analysis of clinical, radiological, and histopathological findings. Systematic review using PubMed, Embase, MEDLINE, and Web of Science databases. Two patients with large skull base TGs mimicking CPA tumors clinically and radiographically were managed at the authors' institution. The first presented 4 years after MVD with asymmetrical sensorineural hearing loss, multiple progressive cranial neuropathies, and brainstem edema due to a growing TG. Reoperation with resection of the granuloma confirmed a foreign-body reaction consisting of multinucleated giant cells containing intracytoplasmic Teflon particles. The second patient presented 11 years after MVD with asymmetrical sensorineural hearing loss and recurrent hemifacial spasm. No growth was noted over 2 years, and the patient has been managed expectantly. Only one prior case of TG after MVD for hemifacial spasm has been reported in the English literature. TG is a rare complication of MVD for hemifacial spasm. The diagnosis should be suspected in patients presenting with a new-onset enhancing mass of the CPA after MVD, even when performed decades earlier. A thorough clinical and surgical history is critical toward establishing an accurate diagnosis to guide management and prevent unnecessary morbidity. Surgical intervention is not required unless progressive neurologic complications ensue. 4 Laryngoscope, 127:715-719, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

Hemifacial atrophy treated with autologous fat transplantation

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Gandhi Vijay

2005-01-01

Full Text Available A 23-year-old male developed right hemifacial atrophy following marphea profunda. Facial asymmetry due to residual atrophy was treated with autologous fat harvested from buttocks with marked cosmetic improvement.

[A girl with congenital hemifacial hypertrophy

NARCIS (Netherlands)

Broeke, S.M. van den; Wolvius, E.B.; Adrichem, L.N. van; Baat, C. de

2006-01-01

A girl with congenital hemifacial hypertrophy had been observed and treated by a multidisciplinary team for craniofacial disorders in an academic medical centre since birth. At the age of 8 she was treated on account of considerable facial asymmetry and multiple intraoral problems. The two-step

Persistent accommodative spasm nine years after head trauma.

Science.gov (United States)

Bohlmann, B J; France, T D

1987-09-01

Spasm of the near reflex is most often seen on a functional basis in young adults with underlying emotional problems. In particular, when convergence spasm is associated with miosis on attempted lateral gaze, a functional basis for the disorder should be suspected. Patients who experience spasm of the near reflex following trauma commonly follow a benign course with spontaneous resolution of their ocular complaints within 1-2 years. Accommodative spasm, manifested by pseudomyopia, or spasm of convergence, alone, or in combination with miosis, may be found as isolated signs of spasm of the near reflex. We report a patient who continues to demonstrate accommodative spasm 9 years after a motor vehicle accident.

Nonsurgical treatment of hemifacial microsomia by therapeutic ultrasound and hybrid functional appliance

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Tarek El-Bialy

2010-03-01

Full Text Available Tarek El-Bialy1, Ali Hasan2, Ahmad Janadas3, Tarik Albaghdadi41Division of Orthodontics, Department of Dentistry, University of Alberta, Edmonton, Alberta, Canada; 2Division of Orthodontics, Department of Preventive Dental Sciences, Faculty of Dentistry; 3Division of Oral and Maxillofacial Surgery, Department of Oral and Maxillofacial Surgery, Faculty of Dentistry; 4Division of Radiology, Faculty of Medicine, King Abdul Aziz University, Jeddah, Saudi ArabiaAim: Conventional treatment of patients with hemifacial microsomia involves orthognathic surgery and/or distraction osteogenesis of the mandible. Previous reports showed that low-intensity pulsed ultrasound (LIPUS enhances mandibular growth in growing rabbits and monkeys. In monkeys, LIPUS enhanced mandibular growth when combined with functional jaw orthopedic appliances. The purpose of this pilot study was to investigate if LIPUS could enhance mandibular growth in children with hemifacial microsomia.Methods: Five children (age range 3–11 years with hemifacial microsomia were treated with hybrid jaw orthopedic functional appliances and treatment of the affected mandibular condyle by LIPUS for 20 minutes per day.Results: The results showed that after one year of treatment, significant improvement of the underdeveloped side of patients’ faces and mandibles was recognized both clinically and radiographically.Discussion: Although improvement took a longer time than did a surgical approach, optimizing this technique may achieve better results in a shorter treatment time. A randomized controlled clinical trial to investigate the effect of optimized LIPUS application or functional appliances in the treatment of hemifacial microsomia is warranted.Keywords: hemifacial microsomia, LIPUS, non-surgical treatment, children

Accommodative spasm in siblings: A unique finding

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Rutstein Robert

2010-01-01

Full Text Available Accommodative spasm is a rare condition occurring in children, adolescents, and young adults. A familial tendency for this binocular vision disorder has not been reported. I describe accommodative spasm occurring in a brother and sister. Both children presented on the same day with complaints of headaches and blurred vision. Treatment included cycloplegia drops and bifocals. Siblings of patients having accommodative spasm should receive a detailed eye exam with emphasis on recognition of accommodative spasm.

Accommodative spasm in siblings: A unique finding

Science.gov (United States)

Rutstein, Robert P

2010-01-01

Accommodative spasm is a rare condition occurring in children, adolescents, and young adults. A familial tendency for this binocular vision disorder has not been reported. I describe accommodative spasm occurring in a brother and sister. Both children presented on the same day with complaints of headaches and blurred vision. Treatment included cycloplegia drops and bifocals. Siblings of patients having accommodative spasm should receive a detailed eye exam with emphasis on recognition of accommodative spasm. PMID:20534925

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...

Nonsurgical Treatment of Hemifacial Microsomia: A Case Report.

Science.gov (United States)

Nouri, Mahtab; Farzan, Arash

2015-11-01

Hemifacial microsomia (HFM) is a birth defect involving craniofacial structures derived from the first and second branchial arches. Although it is a relatively uncommon malformation, it is the second most common craniofacial birth defect after cleft lip and palate (CL/P). This is a case report about the successful orthodontic treatment of a patient with mild hemifacial microsomia (HFM), using a non-surgical orthopedic and orthodontic treatment approach. The aim of this approach was to make the best noninvasive modality to treat HFM. A 7-year-old boy with a mild HFM presented with a convex profile and slight chin deviation. Orthopedic treatment performed using a hybrid functional and high pulls headgear. Treatment continued by fixed orthodontic straight wire appliance to achieve perfect occlusion. Excellent esthetic and functional results achieved; total treatment duration was about 72 months.

Progressive Hemifacial Atrophy with Morphea of Cheek

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Ajit Auluck

2006-01-01

Full Text Available Scleroderma is a rare collagen disorder in which fibrosis of skin, subcutaneous tissues and muscles can occur with occasional involvement of bones. Localized scleroderma is a benign condition but can cause significant deformity when it affects the face. We report a case of localized scleroderma of the face causing progressive hemifacial atrophy.

CLINICAL APPLICATION OF BOTULINUM TOXIN TYPE B IN MOVEMENT DISORDERS AND AUTONOMIC SYMPTOMS

Institute of Scientific and Technical Information of China (English)

Xin-hua Wan; Kevin Dat Vuong; Joseph Jankovic

2005-01-01

Objective To evaluate efficacy and safety of botulinum toxin type B (BTX-B) in treatment of movement disorders including blepharospasm, oromandibular dystonia, hemifacial spasm, tremor, tics, and hypersecretory disorders such as sialorrhea and hyperhidrosis.Methods A retrospective study of BTX-B injections in treatment of 58 patients with various neurological disorders was performed. The mean follow-up time was 0.9 ± 0.8 years. Results of the first and last treatment of patients with at least 3injection sessions were compared.Results The response of 58 patients to a total of 157 BTX-B treatment sessions was analyzed. Of the 157 treatment sessions, 120 sessions (76.4%) resulted in moderate or marked improvement while 17 sessions (10.8%) had no response.The clinical benefits after BTX-B treatment lasted an average of 14 weeks. Of the 41 patients with at least 3 injection sessions (mean 10 ± 8.6), most patients needed increased dosage upon the last session compared to the first session. Nineteen patients (32.8%) with 27 sessions (17.2%) reported adverse effects with BTX-B treatment.Conclusios Though most patients require increased dosage to maintain effective response after repeated injections,BTX-B is an effective and safe treatment drug for a variety of movement disorders, as well as drooling and hyperhidrosis.

Conversion Ratio between Botox®, Dysport®, and Xeomin® in Clinical Practice

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Francesco Scaglione

2016-03-01

Full Text Available Botulinum neurotoxin has revolutionized the treatment of spasticity and is now administered worldwide. There are currently three leading botulinum neurotoxin type A products available in the Western Hemisphere: onabotulinum toxin-A (ONA Botox®, abobotulinum toxin-A (ABO, Dysport®, and incobotulinum toxin A (INCO, Xeomin®. Although the efficacies are similar, there is an intense debate regarding the comparability of various preparations. Here we will address the clinical issues of potency and conversion ratios, as well as safety issues such as toxin spread and immunogenicity, to provide guidance for BoNT-A use in clinical practice. INCO was shown to be as effective as ONA with a comparable adverse event profile when a clinical conversion ratio of 1:1 was used. The available clinical and preclinical data suggest that a conversion ratio ABO:ONA of 3:1—or even lower—could be appropriate for treating spasticity, cervical dystonia, and blepharospasm or hemifacial spasm. A higher conversion ratio may lead to an overdosing of ABO. While uncommon, distant spread may occur; however, several factors other than the pharmaceutical preparation are thought to affect spread. Finally, whereas the three products have similar efficacy when properly dosed, ABO has a better cost-efficacy profile.

Addison's disease presenting with muscle spasm.

Science.gov (United States)

Bhattacharjee, Rana; Sharma, A; Rays, A; Thakur, I; Sarkar, D; Mandal, B; Mookerjee, S K; Chatterjee, S K; Chowdhury, Pradip Roy

2013-09-01

Primary hypoadrenalism has various causes and protean manifestation. We report a young female patient who presented with severe muscle spasm as her primary complaint. On evaluation she was found to be a case of Addison's disease secondary to adrenal tuberculosis. Her muscle spasm disappeared rapidly with replacement dose of glucocorticoid.

Spasm induced by protection balloon during carotid artery stenting

International Nuclear Information System (INIS)

Tsutsumi, Masanori; Kazekawa, Kiyoshi; Onizuka, Masanari

2007-01-01

The PercuSurge system is a distal balloon embolic protection device used for carotid artery stenting (CAS). We performed a retrospective study on the prognosis and clinical effects of spasms induced by the PercuSurge GuardWire system (PercuSurge-induced spasm). We performed CAS in 118 carotid stenoses using the PercuSurge system. Of the 118 procedures, 31 (26.3%) of the patients experienced PercuSurge-induced spasm, and all underwent postoperative follow-up studies by cerebral angiography and antiplatelet treatment. On follow-up angiograms obtained a mean of 5.2 months (range 3-10 months) after CAS, all 31 PercuSurge-induced spasms had disappeared, and no delayed stenosis was found at the sites where the spasms had occurred. No ischemic events due to the spasms occurred during a mean follow-up of 13 months (range 3-32 months). In the hands of physicians experienced in endovascular surgery, CAS using the PercuSurge system is a safe method with which to treat patients with carotid stenosis. Our study demonstrated that PercuSurge-induced spasms had no morphological or clinical adverse effects. (author)

Neurología funcional del blefaroespasmo Functional neurology of blepharospasm

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Fidias E. León-Sarmiento

2008-08-01

Full Text Available El blefaroespasmo esencial benigno cursa con movimientos repetitivos anormales del cierre de los párpados y espasmo del músculo orbicular de los ojos. Modernas teorías postulan que este trastorno del movimiento se origina por alteraciones en el procesamiento de la información aferente, con posterior desintegración de dicha información a nivel del programa neural sensorimotor que existe en el sistema nervioso central, que se manifiesta luego como movimiento anormal en los individuos genéticamente susceptibles. Diferentes investigaciones que incluyen estudios imagenológicos, genéticos y neurofisiológicos han proporcionado nuevos hallazgos acerca de las áreas neurales involucradas en esta patología y la forma como se genera este trastorno. Dentro de estas investigaciones sobresale el estudio del reflejo del parpadeo obtenido eléctricamente, el cual consta de tres respuestas llamadas no-nociceptiva (R1, nociceptiva (R2 y ultranociceptiva (R3. Dicho reflejo y, principalmente, la respuesta refleja ultranociceptiva (R3 parece ser muy útil para entender más profundamente la fisiopatología de esta distonía focal y realizar la endofenotipificación funcional y seguimiento en la neuro rehabilitación correspondiente de este complejo problema neurológico.Benign essential blepharospasm is characterized by abnormal repetitive movements of lid closure and spasm of the orbiculari oculi muscles. Modern theories postulate that this movement disorder originates by abnormal processing of afferent information with further disintegration of the sensorimotor neural program at central levels of the nervous system all of which is seen as dystonic movements in genetically susceptible people. Different investigations including neuroimagin, genetic and neurophysiological studies have discovered new findings on what structures are involved and how this abnormal movement is generated. Among these research is noteworthy the study of electrically elicited

Recognizing Uncommon Presentations of Psychogenic (Functional Movement Disorders

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José Fidel Baizabal-Carvallo

2015-01-01

Full Text Available Background: Psychogenic or functional movement disorders (PMDs pose a challenge in clinical diagnosis. There are several clues, including sudden onset, incongruous symptoms, distractibility, suggestibility, entrainment of symptoms, and lack of response to otherwise effective pharmacological therapies, that help identify the most common psychogenic movements such as tremor, dystonia, and myoclonus.Methods: In this manuscript, we review the frequency, distinct clinical features, functional imaging, and neurophysiological tests that can help in the diagnosis of uncommon presentations of PMDs, such as psychogenic parkinsonism, tics, and chorea; facial, palatal, and ocular movements are also reviewed. In addition, we discuss PMDs at the extremes of age and mass psychogenic illness.Results: Psychogenic parkinsonism (PP is observed in less than 10% of the case series about PMDs, with a female–male ratio of roughly 1:1. Lack of amplitude decrement in repetitive movements and of cogwheel rigidity help to differentiate PP from true parkinsonism. Dopamine transporter imaging with photon emission tomography can also help in the diagnostic process. Psychogenic movements resembling tics are reported in about 5% of PMD patients. Lack of transient suppressibility of abnormal movements helps to differentiate them from organic tics. Psychogenic facial movements can present with hemifacial spasm, blepharospasm, and other movements. Some patients with essential palatal tremor have been shown to be psychogenic. Convergence ocular spasm has demonstrated a high specificity for psychogenic movements. PMDs can also present in the context of mass psychogenic illness or at the extremes of age.Discussion: Clinical features and ancillary studies are helpful in the diagnosis of patients with uncommon presentations of psychogenic movement disorders.

Neonatal hypoglycemic brain injury is a cause of infantile spasms

OpenAIRE

YANG, GUANG; ZOU, LI-PING; WANG, JING; SHI, XIUYU; TIAN, SHUPING; YANG, XIAOFAN; JU, JUN; YAO, HONGXIANG; LIU, YUJIE

2016-01-01

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 who...

Espasmo hemifacial e impressão basilar associados a malformação de arnold-chiari relato de caso

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Manoel Baldoino Leal Filho

1992-09-01

Full Text Available Os autores relatam o caso de uma paciente com espasmo hemifacial e impressão basilar associados a malformação de Arnold-Chiari. Com a descompressão cirúrgica da fossa posterior, empregada no tratamento da impressão basilar, houve melhora do quadro clínico e o espasmo hemifacial se reduziu quanto à frequência, duração e intensidade. É enfatizada a necessidade do tratamento etiológico do espasmo hemifacial, antes de se recorrer à toxina botulínica.

Hemifacial microsomia: clinicoradiological insight and report of a case

African Journals Online (AJOL)

Background: Hemifacial microsomia is a congenital malformation characterized by deficiency in the amount of hard and soft tissue on one side of the face. It is primarily a syndrome of the first branchial arch, involving underdevelopment of the temporomandibular joint, masticatory muscles, mandibular ramus, ear and, ...

Spasm of the near reflex associated with head injury.

Science.gov (United States)

Knapp, Christopher; Sachdev, Arun; Gottlob, Irene

2002-03-01

Spasm of the near reflex is characterized by intermittent miosis, convergence spasm and pseudomyopia with blurred vision at distance. Usually, it is a functional disorder in young patients with underlying emotional problems. Only rarely is it caused by organic disorder. We report a patient who developed convergent spasm associated with miosis after head trauma at the age of 84 years.

The angiographic findings of coronary arterial spasm (a report of 37 cases)

International Nuclear Information System (INIS)

Kuang Jianyi; Chen Jianhun; Du Zhimin; Li Xiangmin; Hu Chengheng

2002-01-01

Objective: The arterial spasm which often seen in the coronary angiography was considered as a result of the pathological spasm of the coronary artery or a complication of catheterization. The coronary arterial spasm is a considerable situation because it increases the danger of stenotic lesions in coronary artery, which should be managed immediately. Methods: In total 37 patients presented with coronary arterial spasm in the coronary angiography (Judkins method): 23 male and 14 female, aged 43-60 years (the 47 years in average). Spasms were relieved in all patients after the administration of glonoin. Results: Solitary spasm were the most common, the angiographic findings were long section (>10 mm) with stenosis of 50%-65% in left anterior descending artery (12 cases), or in the middle and near portion of the right coronary artery (5 cases), where the orifice of the branches were involved; severe stenosis of the orifice of the right coronary artery (3 cases); irregular, mild and local spasm in left anterior descending artery or circumflex (8 cases). And multiple spasm was observed in left anterior descending artery (4 cases) or in the right coronary artery (5 cases). In 19 cases, stenosis accompanying with spasm were revealed and local spasm were observed at the site of arterial sclerosis. Conclusion: The spasm owing to the catheterization doesn't result in chest discomfort and changes in EKG, while pathological spasm occurs in the population with high risk of coronary heart disease, which indicates the potential myocardial infraction. The angiographic findings helps differentiating the two situation

Spasm of accommodation associated with closed head trauma.

Science.gov (United States)

Chan, R V Paul; Trobe, Jonathan D

2002-03-01

Spasm of accommodation, creating pseudomyopia, is generally associated with miosis and excess convergence as part of spasm of the near reflex. It may also exist as an isolated entity, usually attributed to psychogenic causes. We present six cases of accommodative spasm associated with closed head injury. All patients were male, ranging in age between 16 and 37 years. The degree of pseudomyopia, defined as the difference between manifest and cycloplegic refraction, was 1.5 to 2 diopters. A 3-year trial of pharmacologically induced cycloplegia in one patient did not lead to reversal of the spasm when the cycloplegia was stopped. All patients required the manifest refraction to see clearly at distance. The pseudomyopia endured for at least 7 years following head trauma. This phenomenon may represent traumatic activation or disinhibition of putative brain stem accommodation centers in young individuals.

Infantile spasms: A prognostic evaluation

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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.

Microvascular decompression of the cochleovestibular nerve for treatment of tinnitus and vertigo : a systematic review and meta-analysis of individual patient data

NARCIS (Netherlands)

van den Berge, Minke J C; van Dijk, J. Marc C.; Posthumus, Iris A; Smidt, Nynke; van Dijk, Pim; Free, Rolien H

OBJECTIVE Microvascular decompression (MVD) is regarded as a valid treatment modality in neurovascular conflicts (NVCs) causing, for example, trigeminal neuralgia and hemifacial spasms. An NVC of the cochleovestibular nerve might cause tinnitus and/or vertigo; however, general acceptance of MVD for

[Spasm of the near reflex. Treatment with botulinum toxin].

Science.gov (United States)

Merino, P; Rojas, P; Gómez de Liaño, P; Franco Iglesias, G

2015-05-01

A 38-year old female with diplopia and esotropia, with limitation of ocular abduction in both eyes, with full abduction after doll's head rotation also being observed. She was diagnosed with spasm of the near reflex. Treatment with injections of botulinum toxin in both medial rectus has temporally resolved the convergence spasm. Near reflex spasm is characterized as miosis, pseudomyopia, and convergent strabismus that lead to diplopia, blurred vision, headache, and variable, progressive, and intermittent esotropia. As the spasm worsens there will be limited ocular versions and ductions simulating a sixth nerve palsy. Botulinum toxin may be effective in some cases. Copyright © 2013 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

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

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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.

Brainstem evoked potentials in infantile spasms

International Nuclear Information System (INIS)

Miyazaki, Masahito; Hashimoto, Toshiaki; Murakawa, Kazuyoshi; Tayama, Masanobu; Kuroda, Yasuhiro

1992-01-01

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)

Relation between flexor spasms, uninhibited detrusor contractions and anal sphincter activity

DEFF Research Database (Denmark)

Pedersen, E; Petersen, T; Schrøder, H D

1986-01-01

The time relation between flexor spasms, detrusor contractions and anal sphincter activity was recorded in a consecutive series of 111 patients with hyperreflexic bladder and flexor spasms. In 76 of the patients flexor spasms were preceded by detrusor contractions. The opposite pattern, namely de...

Acute Heart Failure Triggered by Coronary Spasm With Transient Left Ventricular Dysfunction.

Science.gov (United States)

Adachi, Yusuke; Sakakura, Kenichi; Ibe, Tatsuro; Yoshida, Nanae; Wada, Hiroshi; Fujita, Hideo; Momomura, Shin-Ichi

2017-04-06

Coronary spasm is abnormal contraction of an epicardial coronary artery resulting in myocardial ischemia. Coronary spasm induces not only depressed myocardial contractility, but also incomplete myocardial relaxation, which leads to elevated ventricular filling pressure. We herein report the case of a 55-year-old woman who had repeated acute heart failure caused by coronary spasm. Acetylcholine provocation test with simultaneous right heart catheterization was useful for the diagnosis of elevated ventricular filling pressure as well as coronary artery spasm. We should add coronary spasm to a differential diagnosis for repeated acute heart failure.

Microvascular decompression for the patient with painful tic convulsif after Bell palsy.

Science.gov (United States)

Jiao, Wei; Zhong, Jun; Sun, Hui; Zhu, Jin; Zhou, Qiu-Meng; Yang, Xiao-Sheng; Li, Shi-Ting

2013-05-01

Painful tic convulsif is referred to as the concurrent trigeminal neuralgia and hemifacial spasm. However, painful tic convulsif after ipsilateral Bell palsy has never been reported before. We report a case of a 77-year-old woman with coexistent trigeminal neuralgia and hemifacial spasm who had experienced Bell palsy half a year ago. The patient underwent microvascular decompression. Intraoperatively, the vertebrobasilar artery was found to deviate to the symptomatic side and a severe adhesion was observed in the cerebellopontine angle. Meanwhile, an ectatic anterior inferior cerebellar artery and 2 branches of the superior cerebellar artery were identified to compress the caudal root entry zone (REZ) of the VII nerve and the rostroventral cisternal portion of the V nerve, respectively. Postoperatively, the symptoms of spasm ceased immediately and the pain disappeared within 3 months. In this article, the pathogenesis of the patient's illness was discussed and it was assumed that the adhesions developed from inflammatory reactions after Bell palsy and the anatomic features of the patient were the factors that generated the disorder. Microvascular decompression surgery is the suggested treatment of the disease, and the dissection should be started from the caudal cranial nerves while performing the operation.

Theoretical Analyses of the Functional Regions of the Heavy Chain of Botulinum Neurotoxin

Science.gov (United States)

1994-01-01

SE. Schulz GM. liallctt M. Effects of hb)tulinum toxin injections on speech in adductor spasmodic dysphonia . Neurology 1988,38:1220-1225. 3. Jankovic...hemifacial spasm. Mov Disord 1987:4:237-254. 5. Brin MF. Blitzer A, Fahn S, Gould W. Lovelace RE. Adductor laryngeal dystonia (spastic dysphonia ): treatment

Hemifacial Display of Emotion in the Resting State

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M. K. Mandal

1992-01-01

Full Text Available The human face at rest displays distinguishable asymmetries with some lateralization of emotion or expression. The asymmetrical nature of the resting face was examined by preparing hemifacial composites, left–left, right–right, along with normal facial orientation. The left side and right side composites were constructed by using the lateral half of one side of the face and its mirror-reversal. The left side facial composites were found to be more emotional than the right side or normal facial orientations of neutral expressions.

Precise positioning of an intraoral distractor using augmented reality in patients with hemifacial microsomia.

Science.gov (United States)

Qu, Miao; Hou, Yikang; Xu, Yourong; Shen, Congcong; Zhu, Ming; Xie, Le; Wang, Hao; Zhang, Yan; Chai, Gang

2015-01-01

Through three-dimensional real time imaging, augmented reality (AR) can provide an overlay of the anatomical structure, or visual cues for specific landmarks. In this study, an AR Toolkit was used for distraction osteogenesis with hemifacial microsomia to define the mandibular osteotomy line and assist with intraoral distractor placement. 20 patients with hemifacial microsomia were studied and were randomly assigned to experimental and control groups. Pre-operative computed tomography was used in both groups, whereas AR was used in the experimental group. Afterwards, pre- and post-operative computed tomographic scans of both groups were superimposed, and several measurements were made and analysed. Both the conventional method and AR technique achieved proper positioning of the osteotomy planes, although the AR was more accurate. The difference in average vertical distance from the coronoid and condyle process to the pre- and post-operative cutting planes was significant (p 0.05) was observed in the average angle between the two planes. The difference in deviations between the intersection points of the overlaid mandible across two cutting planes was also significant (p Augmented reality tools such as the AR Toolkit may be helpful for precise positioning of intraoral distractors in patients with hemifacial microsomia in craniofacial surgery. Copyright © 2014 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Three-dimensional photogrammetry for surgical planning of tissue expansion in hemifacial microsomia.

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Jayaratne, Yasas S N; Lo, John; Zwahlen, Roger A; Cheung, Lim K

2010-12-01

We aim to illustrate the applications of 3-dimensional (3-D) photogrammetry for surgical planning and longitudinal assessment of the volumetric changes in hemifacial microsomia. A 3-D photogrammetric system was employed for planning soft tissue expansion and transplantation of a vascularized scapular flap for a patient with hemifacial microsomia. The facial deficiency was calculated by superimposing a mirror of the normal side on the preoperative image. Postsurgical volumetric changes were monitored by serial superimposition of 3-D images. A total of 31 cm(3) of tissue expansion was achieved within a period of 4 weeks. A scapular free flap measuring 8 cm × 5 cm was transplanted to augment the facial deficiency. Postsurgical shrinkage of the flap was observed mainly in the first 3 months and it was minimal thereafter. 3-D photogrammetry can be used as a noninvasive objective tool for assessing facial deformity, planning, and postoperative follow-up of surgical correction of facial asymmetry.

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

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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.

Avaliação do filme lacrimal de pacientes com distonia facial durante tratamento com toxina botulínica tipo A Lacrimal film evaluation of patients with facial dystonia during botulinum toxin type A treatment

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Patricia Grativol Costa

2006-06-01

Full Text Available OBJETIVO: Determinar o efeito da toxina botulínica no filme lacrimal em pacientes com distonia facial. MÉTODOS: Foram incluídos 24 pacientes portadores de blefaroespasmo essencial e espasmo hemifacial que receberam aplicação de toxina botulínica tipo A que foram submetidos à propedêutica do filme lacrimal previamente à aplicação e após, com 7 e 30 dias. RESULTADOS: Houve diminuição das queixas de olho seco trinta dias após a aplicação, entretanto, o tempo de ruptura do filme lacrimal e o teste de Schirmer não demonstraram variação significativa entre os períodos pré-tratamento e 1 mês da aplicação. Em relação ao teste de coloração com rosa bengala, todos os olhos que coraram no pré-tratamento, melhoraram na última avaliação. CONCLUSÃO: A injeção de toxina botulínica pode aliviar as queixas de olho seco nos pacientes com distonia facial pela provável ação de inibição do orbicular na sua função de bomba lacrimal.PURPOSE: To determine the effect of botulinum toxin injection in the eyelid on lacrimal film in patients with facial dystonia. METHODS: Twenty-four patients with essential blepharospasm and hemifacial spasm were submitted to botulinum toxin injection and lacrimal film tests were performed before the application and after seven and thirty days. RESULTS: There was improvement in symptoms of dry eye and rose bengal test, however, the breakup time and Schirmer's test did not show significant variation between pretreatment and after 1 month of follow-up. CONCLUSION: The dry eye symptoms in patients with facial dystonia may be attenuated by botulinum toxin due to its possible inhibitory effect on the orbicular muscle leading to a decrease in lacrimal pump.

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

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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.

The therapeutic usage of botulinum toxin (Botox in non-cosmetic head and neck conditions – An evidence based review

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Kamran Habib Awan

2017-01-01

Full Text Available Botulinum toxin (Botox is an exotoxin produced from Clostridium botulinum. It blocks the release of acetylcholine from the cholinergic nerve end plates resulting in inactivity of the muscles or glands innervated. The efficacy of Botox in facial aesthetics is well established; however, recent literature has highlighted its utilization in multiple non-cosmetic medical and surgical conditions. The present article reviews the current evidence pertaining to Botox use in the non-cosmetic head and neck conditions. A literature search was conducted using MEDLINE, EMBASE, ISI Web of Science and the Cochrane databases limited to English Language articles published from January 1980 to December 2014. The findings showed that there is level 1 evidence supporting the efficacy of Botox in the treatment of laryngeal dystonia, headache, cervical dystonia, masticatory myalgia, sialorrhoea, temporomandibular joint disorders, bruxism, blepharospasm, hemifacial spasm and rhinitis. For chronic neck pain there is level 1 evidence to show that Botox is ineffective. Level 2 evidence exists for vocal tics and trigeminal. For stuttering, facial nerve paresis, Frey’s syndrome and oromandibular dystonia the evidence is level 4. Thus, there is compelling evidence in the published literature to demonstrate the beneficial role of Botox in a wide range of non-cosmetic conditions pertaining to the head and neck (mainly level 1 evidence. With more and more research, the range of clinical applications and number of individuals getting Botox will doubtlessly increase. Botox appears to justify its title as ‘the poison that heals’.

Pelvic floor spasm as a cause of voiding dysfunction.

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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.

[Mandibular-driven simultaneous maxillo-mandibular distraction for hemifacial microsomia with rapid prototyping technology].

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Gao, Quan-Wen; Song, Hui-Feng; Xu, Ming-Huo; Liu, Chun-Ming; Chai, Jia-Ke

2013-11-01

To explore the clinical application of mandibular-driven simultaneous maxillo-mandihular distraction to correct hemifacial microsomia with rapid prototyping technology. The patient' s skull resin model was manufactured with rapid prototyping technology. The osteotomy was designed on skull resin model. According to the preoperative design, the patients underwent Le Fort I osteotomy and mandibular ramus osteotomy. The internal mandible distractor was embedded onto the osteotomy position. The occlusal titanium pin was implanted. Distraction were carried out by mandibular-driven simultaneous maxillo-mandihular distraction 5 days after operation. The distraction in five patients was complete as designed. No infection and dysosteogenesis happened. The longest distance of distraction was 28 mm, and the shortest distance was 16 mm. The facial asymmetry deformity was significantly improved at the end of distraction. The ocelusal plane of patients obviously improved. Rapid prototyping technology is helpful to design precisely osteotomy before operation. Mandibular-driven simultaneous maxillo-mandibular distraction can correct hemifacial microsomia. It is worth to clinical application.

Hemifacial microsomia: management of the vertical ramus compartment

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Maurice Yves Mommaerts

2015-06-01

Full Text Available Hemifacial microsomia and Goldenhar syndrome pose unique challenges to the craniofacial surgeon. The O.M.E.N.S. classification provides a description of the craniofacial features. For the "M" of O.M.E.N.S. (the mandible, the Pruzansky-Kaban classification provides therapeutic guidelines for joint and face reconstruction. A sequence of standard procedures, including temporomandibular joint reconstruction, facial rotation surgery, gluteal fat grafting, and patient-specific titanium implantation, each have their intricacies. The author provides his expert opinion, acquired over thirty years of experience, with an emphasis on descriptions of and solutions for ten problematic issues.

Esophageal epiphrenic diverticulum associated with diffuse esophageal spasm.

Science.gov (United States)

Matsumoto, Hideo; Kubota, Hisako; Higashida, Masaharu; Manabe, Noriaki; Haruma, Ken; Hirai, Toshihiro

2015-01-01

Esophageal diverticulum, a relatively rare condition, has been considered to be associated with motor abnormalities such as conditions that cause a lack of coordination between the distal esophagus and lower esophageal sphincter. We herein report a case of esophageal epiphrenic diverticulum associated with diffuse esophageal spasm. A 73-year-old woman presented with dysphagia and regurgitation. Imaging examinations revealed a right-sided esophageal diverticulum located about 10cm above the esophagogastric junction. High-resolution manometry revealed normal esophageal motility. However, 24-h pH monitoring revealed continuous acidity due to pooling of residue in the diverticulum. An esophageal epiphrenic diverticulum was diagnosed and resected thoracoscopically. Her dysphagia recurred 2 years later. High-resolution manometry revealed diffuse esophageal spasm. The diverticulum in the present case was considered to have been associated with diffuse esophageal spasm. The motility disorder was likely not identified at the first evaluation. In this case, the patient's symptoms spontaneously resolved without any treatment; however, longer-term follow-up is needed. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

Non-invasive method for recognition of coronary artery spasm

International Nuclear Information System (INIS)

Mathey, D.; Montz, R.; Hanrath, P.; Kuck, K. H.; Bleifeld, W.; Hamburg Univ.

1980-01-01

For evaluation of coronary artery spasm 201 thallium sequential scintigraphy of the myocardium after ergotamine provocation was performed in 10 patients with recurrent angina pectoris at rest and normal exercise ECG. In 9 out of the 10 patients ergotamine administration in the same dosage was repeated during the coronary angiography. A reversible defect in the 201 thallium scintigram representative of regional myocardial ischaemia developed in 9 patients after ergotamine. Only in 4 out of the 9 patients angina pectoris and ST elevation were present at the same time. In all cases coronary spasm after ergotamine was demonstrable in the coronary angiogram; in the 4 patients with ergotamine-induced Prinzmetal angina pectoris it consisted of complete vascular occlusion, in the asymptomatic patients of incomplete vascular narrowing of a higher degree. In all cases the spasm could be relieved by ergotamine antidotes within a few minutes. (orig.) [de

Ramus marginalis mandibulae nervus facialis palsy in hemifacial microsomia.

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Silvestri, A; Mariani, G; Vernucci, R A

2008-12-01

The paralysis of the ramus marginalis mandibulae nervus facialis may occur in Hemifacial Microsomia (HM); the combination of both HM and palsy contributes to an elongation of the mandibular body. This study explores a possible correlation between neurological deficit, muscular atony, and structural deficiency. Of 58 patients with HM who had come to the University of Rome (Sapienza) Pre-surgical Orthodontics Unit, 4 patients were afflicted with Hemifacial Microsomia and ramus marginalis mandibulae nervus palsy; these patients underwent physical, neurological, opthamologic and systemic examinations. The results were then analysed in order to determine a possible correlation between neuro-muscular and structural deficit. Electroneurographic and electromyographic examinations were performed to estimate facial nerve and muscles involvement. Neuroelectrographic exam showed a damage of the nervous motor fibres of the facial nerve ipsilateral to HM, with an associated damage of the muscular function, while neuro-muscular functions on the healthy side were normal. The peripheral nervous and muscular deficits affect the function of facial soft tissues and the growth of mandibular body with an asymmetry characterised by a hypodevelopment of the ramus (due to the HM) and by an elongation of the mandibular body (due to ramus marginalis mandibulae nerve palsy), so that the chin deviation is contralateral to HM. In these forms, a neurological examination is necessary to assess the neurological damage on the HM side. Neuromuscular deficiency can also contribute to a relapse tendency after a surgical-orthodontic treatment.

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

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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.

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

Linear Nevus Sebaceum Syndrome and Infantile Spasms

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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.

Swallowing disorders in patients with blepharospasm Trastornos deglutorios en pacientes con blefaroespasmo

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María G. Cersósimo

2005-04-01

Full Text Available Blepharospasm is a focal dystonia characterized by involuntary eye closure due to abnormal contraction of orbicular eyelid muscles. When blepharospasm is associated to the presence of involuntary oromandibular movements, it is termed Meige syndrome. The aim of this study was to investigate the presence of deglutition alterations in patients with concurrent blepharospasm and Meige syndrome. Twenty consecutive patients were studied by video fluoroscopy using a barium technique. The 4 stages of deglutition were investigated. Ninety percent of patients (18 cases presented deglutition disorders. The more commonly found alterations were premature food drop, 15 cases (83% and vallecuale residuals, 14 cases (78%. Sixty seven percent of abnormal findings occurred in the third stage of deglutition. Eighty-nine percent of patients (16 presented more than one swallowing alteration. There was a positive and significant correlation between the number of alterations and patient's age or disease duration. Prevalence of swallowing disorders in the healthy elderly population is reported to be 44%. In our series it reached 90%, suggesting that our findings might be related not only with age but also with a more widespread dystonia exceeding the orofacial muscles.El blefaroespasmo es una distonía focal caracterizada por el cierre involuntario de los ojos debido a la contracción anormal de los músculos orbiculares de los párpados. Cuando el blefaroespasmo se asocia a la presencia de movimientos involuntarios oromandibulares se denomina síndrome de Meige. El objetivo de este estudio fue investigar la presencia de alteraciones deglutorias en pacientes con blefaroespasmo y síndrome de Meige. Se incluyeron 20 pacientes consecutivos que fueron estudiados mediante vídeo fluoroscopia con técnica de bario. Se investigaron las 4 etapas de la deglución. El 90% de los pacientes (18 casos presentó trastornos en la deglución. Las alteraciones más comúnmente halladas

Very late coronary spasm inducing acute myocardial infarction in a heart transplant recipient.

Science.gov (United States)

Santoro, Francesco; Lopizzo, Agostino; Centola, Antonio; Cuculo, Andrea; Ruggiero, Antonio; Di Biase, Matteo; Brunetti, Natale Daniele

2016-12-01

: We report coronary angio findings of very late (10-year) coronary spasm inducing acute myocardial infarction with typical chest pain in a heart transplant recipient. Coronary spasm was promptly relieved by intra-coronary infusion of nitrates.

[Spasm of accommodation].

Science.gov (United States)

Lindberg, Laura

2014-01-01

Spasm of accommodation refers to prolonged contraction of the ciliary muscle, most commonly causing pseudomyopia to varying degrees in both eyes by keeping the lens in a state of short sightedness. It may also be manifested as inability to allow the adaptation spasticity prevailing in the ciliary muscle relax without measurable myopia. As a rule, this is a functional ailment triggered by prolonged near work and stress. The most common symptoms include blurring of distance vision, varying visual acuity as well as pains in the orbital region and the head, progressing into a chronic state. Cycloplegic eye drops are used as the treatment.

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

Endothelial dysfunction and the occurrence of radial artery spasm during transradial coronary procedures: The ACRA-Spasm study

NARCIS (Netherlands)

Van Der Heijden, D.J. (Dirk J.); M.A.H. van Leeuwen (Maarten); G.N. Janssens (Gladys N.); Hermie, J. (Jailen); M.J. Lenzen (Mattie); M.J.P.F. Ritt; P.M. van de Ven (Peter); F. Kiemeneij (Ferdinand); N. van Royen (Niels)

2016-01-01

textabstractAims: The aim of this study was to analyse the relation between endothelial dysfunction (ED) and the occurrence of radial artery spasm (RAS) during transradial coronary procedures. Methods and results: From May 2014 to June 2015, endothelial function was assessed by EndoPAT and FMD

Arterial compression of nerve is the primary cause of trigeminal neuralgia.

Science.gov (United States)

Chen, Guo-Qiang; Wang, Xiao-Song; Wang, Lin; Zheng, Jia-Ping

2014-01-01

Whether arterial or venous compression or arachnoid adhesions are primarily responsible for compression of the trigeminal nerve in patients with trigeminal neuralgia is unclear. The aim of this study was to determine the causes of trigeminal nerve compression in patients with trigeminal neuralgia. The surgical findings in patients with trigeminal neuralgia who were treated by micro vascular decompression were compared to those in patients with hemifacial spasm without any signs or symptoms of trigeminal neuralgia who were treated with microvascular decompression. The study included 99 patients with trigeminal neuralgia (median age, 57 years) and 101 patients with hemifacial spasm (median age, 47 years). There were significant differences between the groups in the relationship of artery to nerve (p relationship of vein to nerve. After adjustment for age, gender, and other factors, patients with vein compression of nerve or with artery compression of nerve were more likely to have trigeminal neuralgia (OR = 5.21 and 42.54, p = 0.026 and p compression of the trigeminal nerve is the primary cause of trigeminal neuralgia and therefore, decompression of veins need not be a priority when performing microvascular dissection in patients with trigeminal neuralgia.

Diffuse esophageal spasm

International Nuclear Information System (INIS)

Ott, D.J.; Chen, Y.M.; Hewson, E.G.; Richter, J.E.; Wu, W.C.; Gelfand, D.W.; Castell, D.O.

1988-01-01

Radiologic and manometric findings were correlated in 17 patients with diffuse esophageal spasm (DES). All patients initially had chest pain and/or dysphagia and had a manometric diagnosis of DES. Mean percentage of normal peristalsis manometrically was 46% (range, 20%-80%). Based on radiologic examination, an esophageal motor disorder consistent with DES was diagnosed in 12 of 17 patients, and there was one misinterpretation of achalasia. Radiologic detection was not related significantly to the percentage of peristalsis seen on manometric examination. Mean esophageal wall thickness as measured radiographically in patients with DES was 2.6 mm, compared with 2.5 mm in 17 individuals with normal results of manometry

An evidence-based review of botulinum toxin (Botox) applications in non-cosmetic head and neck conditions

Science.gov (United States)

Persaud, Ricardo; Garas, George; Silva, Sanjeev; Stamatoglou, Constantine; Chatrath, Paul; Patel, Kalpesh

2013-01-01

Botulinum toxin (Botox) is an exotoxin produced from Clostridium botulinum. It works by blocking the release of acetylcholine from the cholinergic nerve end plates leading to inactivity of the muscles or glands innervated. Botox is best known for its beneficial role in facial aesthetics but recent literature has highlighted its usage in multiple non-cosmetic medical and surgical conditions. This article reviews the current evidence pertaining to Botox use in the head and neck. A literature review was conducted using The Cochrane Controlled Trials Register, Medline and EMBASE databases limited to English Language articles published from 1980 to 2012. The findings suggest that there is level 1 evidence supporting the efficacy of Botox in the treatment of spasmodic dysphonia, essential voice tremor, headache, cervical dystonia, masticatory myalgia, sialorrhoea, temporomandibular joint disorders, bruxism, blepharospasm, hemifacial spasm and rhinitis. For chronic neck pain there is level 1 evidence to show that Botox is ineffective. Level 2 evidence exists for vocal tics, trigeminal neuralgia, dysphagia and post-laryngectomy oesophageal speech. For stuttering, ‘first bite syndrome’, facial nerve paresis, Frey's syndrome, oromandibular dystonia and palatal/stapedial myoclonus the evidence is level 4. Thus, the literature highlights a therapeutic role for Botox in a wide range of non-cosmetic conditions pertaining to the head and neck (mainly level 1 evidence). With ongoing research, the spectrum of clinical applications and number of people receiving Botox will no doubt increase. Botox appears to justify its title as ‘the poison that heals’. PMID:23476731

The application of Fasudil in treating vascular spasm occurred in interventional treatment for hepatocellular carcinomas

International Nuclear Information System (INIS)

Fan Xiaoqiang; Shen Jie; Zhang Xuena; Liu Qiuru; Ma Aiying

2011-01-01

Objective: To explore an effective way to treat the vascular spasm occurred during TACE for hepatocellular carcinomas. Methods: During interventional chemoembolization for hepatocellular carcinomas, Fasudil of 2.5 mg was injected via the catheter if vessel spasm occurred, which was followed by DSA to determine the dilatation of the arteries. Adverse effect was observed and recorded. Results: After the injection of Fasudil the vascular spasm was completely relieved in all the 30 cases. The interventional procedure for hepatocellular carcinomas was successfully accomplished in all patients. No obvious side effect occurred. Conclusion: The injection of Fasudil via the catheter is an effective and safe method to eliminate vessel spasm occurred during TACE for hepatocellular carcinomas. (authors)

Cranial computed tomography in infantile spasms

International Nuclear Information System (INIS)

Howitz, P.; Neergaard, K.; Pedersen, H.

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)

[Usefullness of transesophageal echocardiography in early detection of coronary spasm].

Science.gov (United States)

Sagara, M; Haraguchi, M; Hamu, Y; Isowaki, S; Yoshimura, N

1996-04-01

Intraoperative transesophageal echocardiography (TEE) was performed on a 62-year-old man who underwent abdominal aortic replacement for abdominal aortic aneurysm under general anesthesia combined with epidural anesthesia. Coronary artery spasm occurred after unexpected massive hemorrhage, and TEE showed hypokinesis in the posterior-inferior left ventricular wall. The changes in TEE preceded the ST elevation in the ECG. Bolus infusion of isosorbide dinitrate and continuous infusion of nitroglycerin alleviated these changes. TEE enabled us to detect and evaluate coronary spasm before the appearance of ST changes in ECG.

Motor unit firing rates during spasms in thenar muscles of spinal cord injured subjects

NARCIS (Netherlands)

Zijdewind, Inge; Bakels, Robert; Thomas, Christine K.

2014-01-01

Involuntary contractions of paralyzed muscles (spasms) commonly disrupt daily activities and rehabilitation after human spinal cord injury (SCI). Our aim was to examine the recruitment, firing rate modulation, and derecruitment of motor units that underlie spasms of thenar muscles after cervical

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

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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.

[Analysis of gene mutation of early onset epileptic spasm with unknown reason].

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Yang, X; Pan, G; Li, W H; Zhang, L M; Wu, B B; Wang, H J; Zhang, P; Zhou, S Z

2017-11-02

Objective: To summarize the gene mutation of early onset epileptic spasm with unknown reason. Method: In this prospective study, data of patients with early onset epileptic spasm with unknown reason were collected from neurological department of Children's Hospital of Fudan University between March 2016 and December 2016. Patients with known disorders such as infection, metabolic, structural, immunological problems and known genetic mutations were excluded. Patients with genetic disease that can be diagnosed by clinical manifestations and phenotypic characteristics were also excluded. Genetic research methods included nervous system panel containing 1 427 epilepsy genes, whole exome sequencing (WES), analysis of copy number variation (CNV) and karyotype analysis of chromosome. The basic information, phenotypes, genetic results and the antiepileptic treatment of patients were analyzed. Result: Nine of the 17 cases with early onset epileptic spasm were boys and eight were girls. Patients' age at first seizure onset ranged from 1 day after birth to 8 months (median age of 3 months). The first hospital visit age ranged from 1 month to 2 years (median age of 4.5 months). The time of following-up ranged from 8 months to 3 years and 10 months. All the 17 patients had early onset epileptic spasm. Video electroencephalogram was used to monitor the spasm seizure. Five patients had Ohtahara syndrome, 10 had West syndrome, two had unclear classification. In 17 cases, 10 of them had detected pathogenic genes. Nine cases had point mutations, involving SCN2A, ARX, UNC80, KCNQ2, and GABRB3. Except one case of mutations in GABRB3 gene have been reported, all the other cases had new mutations. One patient had deletion mutation in CDKL5 gene. One CNV case had 6q 22.31 5.5MB repeats. Ten cases out of 17 were using 2-3 antiepileptic drugs (AEDs) and the drugs had no effect. Seven cases used adrenocorticotropic hormone (ACTH) and prednisone besides AEDs (a total course for 8 weeks

A novel medical protocol to treat uterotubal spasm during Essure hysteroscopic sterilization: a pilot study

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Chapa HO

2012-06-01

Full Text Available Hector O Chapa,1 Gonzalo Venegas21Women's Specialty Center, Dallas, TX, USA; 2Clinical Faculty, Department ObGyn Methodist Medical Center, Dallas, TX, USAObjective: To evaluate sublingual hyoscyamine (0.125 mg as a uterotubal spasm reliever to increase successful bilateral, first-attempt Essure microinsert placement.Study design: Prospective cohort study as pilot clinical investigation of study medicine.Setting: An inner-city Dallas, TX obstetrics and gynecology office practice.Materials and methods: The study period was January 1, 2008 to July 1, 2010. Patients requesting sterilization were offered Essure under local anesthesia (office setting. Those declining were referred for operative laparoscopy. Patients accepting office sterilization were offered study participation. Study patients noted to have intraoperative uni/bilateral uterotubal spasms preventing cannulation were given one sublingual hyoscyamine (0.125 mg tablet. Primary endpoint: spasm alleviation and successful tubal cannulation (bilaterally. Secondary endpoint: percentage of bilateral tubal occlusions documented by follow up hysterosalpingogram (12 weeks, and adverse events possibly related to medication.Results: Within the study period, 316 patients underwent office sterilization (local anesthesia; 21 had unilateral tubal spasm preventing cannulation (6%. Spasm was relieved in 17/21 (80% after hyoscyamine, allowing for microinsert placement. The mean time from attempted cannulation to medication was 9 minutes (8.3–10.5 minutes, and the mean time for spasm resolution was 53 seconds (49–72 seconds. At follow-up confirmation testing, all 17 who were hyoscynamine responders were found to have bilateral tubal occlusion as well as proper microinsert location. The 4/21 hyoscyamine nonresponders underwent a second unilateral attempt 48–72 hours later. Fifty percent (n = 2 had successful placement, leaving two for alternative care. Rapid pulse was reported by 1/21 (4.7% 5 minutes

A Case of Devic’s Syndrome Presenting with Tonic Spasm: Response to Levetiracetam Treatment

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Alev Leventoğlu

2011-03-01

Full Text Available Neuromyelitis optica or Devic’s syndrome is a rare autoimmune disorder which is characterized by inflammatory demyelination of the optic nerves and the spinal cord. Clinically, it causes visual loss in one or both eyes, and numbness or paralysis of the arms and legs. Although tonic spasm is the most frequent movement disorder occuring in MS, it has not been definetely described clinical entity for Devic’s syndrome. We hereby describe a case of Devic’s syndrome with tonic spasms treated with levetiracetam as a new approach and discussed the results of the treatment. A 52-year-old woman with Devic’s syndrome with the complaint of painful tonic spasms primarily affecting the abdomen was given levetiracetam therapy. Levetiracetam therapy resulted in a good response in our patient. Levetiracetam can be a new choice for the treatment of painful tonic spasm with Devic’s syndrome. However, more detailed studies are necessary to investigate efficacy of levetiracetam.

'Variant' angina: Evidence for small vessel coronary artery spasm

International Nuclear Information System (INIS)

Pfisterer, M.; Mueller-Brand, J.; Cueni, T.; Luetold, B.; Burkart, F.; Basel Univ.

1980-01-01

A unique case of 'variant' angina pectoris has been observed in a patient with normal coronary arteries and typical chest pain appearing spontaneously at rest, and repeatedly provoked by ergonovine maleate (0.1 mg iv) associated with large transmural perfusion defects on 201 TI-imaging (after ergonovine) and a marked increase in T wave voltage despite no demonstrable spasm of a major coronary artery after the same doses of ergonovine. While saline solution could not provoke chest pain and treatment with a beta-blocking agent increased the frequency of ischemic attacks, a calcium antangonist therapy reduced and eventually eliminated the attacks. Thus, this case provides evidence for yet another aspect of a 'variant' form of angina pectoris: small vessel coronary artery spasm. (orig.) [de

Convergence spasm due to aquaporin-positive neuromyelitis optica spectrum disorder

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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.

MOTOR UNIT FIRING RATES DURING SPASMS IN THENAR MUSCLES OF SPINAL CORD INJURED SUBJECTS

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Inge eZijdewind

2014-11-01

Full Text Available Abstract Involuntary contractions of paralyzed muscles (spasms commonly disrupt daily activities and rehabilitation after human spinal cord injury. Our aim was to examine the recruitment, firing rate modulation, and derecruitment of motor units that underlie spasms of thenar muscles after cervical spinal cord injury. Intramuscular electromyographic activity (EMG, surface EMG, and force were recorded during thenar muscle spasms that occurred spontaneously or that were triggered by movement of a shoulder or leg. Most spasms were submaximal (mean: 39%, SD: 33 of the force evoked by median nerve stimulation at 50 Hz with strong relationships between EMG and force (R2>0.69. Unit recruitment occurred over a wide force range (0.2-103% of 50 Hz force. Significant unit rate modulation occurred during spasms (frequency at 25% maximal force: 8.8 Hz, 3.3 SD; at maximal force: 16.1 Hz, 4.1 SD. Mean recruitment frequency (7.1 Hz, 3.2 SD was significantly higher than derecruitment frequency (5.4 Hz, 2.4 SD. Coactive unit pairs that fired for more than 4 s showed high (R2>0.7, n=4 or low (R2:0.3-0.7, n=12 rate-rate correlations, and derecruitment reversals (21 pairs, 29%. Later recruited units had higher or lower maximal firing rates than lower threshold units. These discrepant data show that coactive motoneurons are driven by both common inputs and by synaptic inputs from different sources during muscle spasms. Further, thenar motoneurons can still fire at high rates in response to various peripheral inputs after spinal cord injury, supporting the idea that low maximal voluntary firing rates and forces in thenar muscles result from reduced descending drive.

Preoperative evaluation of neurovascular relationships for microvascular decompression in the cerebellopontine angle in a virtual reality environment.

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Du, Zhuo-Ying; Gao, Xiang; Zhang, Xiao-Luo; Wang, Zhi-Qiu; Tang, Wei-Jun

2010-09-01

In this paper the authors' goal was to evaluate the feasibility and efficacy of a virtual reality (VR) system in preoperative planning for microvascular decompression (MVD) procedures treating idiopathic trigeminal neuralgia and hemifacial spasm. The system's role in surgical simulation and training was also assessed. Between May 2008 and April 2009, the authors used the Dextroscope system to visualize the neurovascular complex and simulate MVD in the cerebellopontine angle in a VR environment in 16 patients (6 patients had trigeminal neuralgia and 10 had hemifacial spasm). Reconstructions were carried out 2-3 days before MVD. Images were printed in a red-blue stereoscopic format for teaching and discussion and were brought into the operating room to be compared with real-time intraoperative findings. The VR environment was a powerful aid for spatial understanding of the neurovascular relationship in MVD for operating surgeons and trainees. Through an initial series of comparison/confirmation experiences, the senior neurosurgeon became accustomed to the system. He could predict intraoperative problems and simulate surgical maneuvering, which increased his confidence in performing the procedure. The Dextroscope system is an easy and rapid method to create a stereoscopic neurovascular model for MVD that is highly concordant with intraoperative findings. It effectively shortens the learning curve and adds to the surgeon's confidence.

Accommodative spasm with bilateral vision loss due to untreated intermittent exotropia in an adult.

Science.gov (United States)

Shanker, V; Ganesh, S; Sethi, S

2012-01-01

Intermittent exotropia (IXT) is an exodeviation intermittently controlled by fusional mechanisms. Patients with IXT may present with asthenopic symptoms, blurred vision, headaches, diplopia or visual confusion and reading difficulties; especially after prolonged periods of near work. To report the presentation and management of a young adult with intractable accommodative spasm secondary to long standing intermittent exotropia. The patient was found to have bilateral accommodative spasm with high pseudomyopia and severe impairment of vision. There was a tendency for recurrence with discontinuation of cycloplegics. A total relief of symptoms was noticed after strabismus surgery was undertaken for the exotropia. A detailed orthoptic evaluation with emphasis on recognizing accommodative spasm as an unusual presentation of IXT, could aid in appropriate diagnosis and treatment of such cases. © NEPjOPH.

Progressive hemifacial atrophy with ciliary body atrophy and ocular hypotony

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T Ashwini Kini

2015-01-01

Full Text Available Progressive hemifacial atrophy (PHA is a disease of unknown etiology affecting one-half of the face. Ocular involvement is uncommon. Atrophy of iris is rare, with only a few cases of partial atrophy being reported in the literature. We report a case of total atrophy of iris and ciliary body with associated ocular hypotony in a 16-year-old girl with PHA. We believe this is the first reported case of complete atrophy of iris and ciliary body in PHA. Ocular hypotony in PHA was thought to be due to intra-ocular inflammation. However in our case it appears to be secondary to severe atrophy of the ciliary body.

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

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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.

Qualidade de vida e custos diretos em pacientes com blefaroespasmo essencial e espasmo hemifacial, tratados com toxina botulínica-A

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Osaki Midori Hentona

2004-01-01

Full Text Available OBJETIVO: Avaliar os custos e a qualidade de vida dos pacientes com blefaroespasmo essencial e espasmo hemifacial, submetidos à terapia com a toxina botulínica-A. MÉTODOS: Vinte e oito pacientes, sendo 16 com blefaroespasmo essencial e 12 com espasmo hemifacial, tratados com toxina botulínica-A, foram avaliados por meio de dois instrumentos: o SF-36 e a Escala de Disfunção de Blefaroespasmo. Os custos foram calculados segundo o número de unidades da toxina utilizadas por cada paciente no período de um ano. RESULTADOS: O custo total direto foi estimado em 1081,62 reais por ano (±89,39 para blefaroespasmo essencial e 618,06 reais por ano (±60,06 para espasmo hemifacial, para tratamento ambulatorial (p<0,001. Antes do tratamento, todos os domínios do SF-36 foram significantemente piores em ambas as doenças. Sob tratamento efetivo com toxina botulínica-A, os aumentos nos índices do SF-36 evidenciaram mudanças nas taxas de saúde geral da população em estudo, com diferença estatisticamente significante entre as respostas pré e pós-tratamento (p<0,05. Relacionando-se o SF-36 com Escala de Disfunção de Blefaroespasmo, foram observadas nos casos de blefaroespasmo essencial, relações significantes positivas (capacidade funcional, estado geral, aspecto emocional e aspectos sociais, ao passo que nos pacientes com espasmo hemifacial não foi encontrada nenhuma correlação. CONCLUSÃO: Este estudo demonstra notável impacto negativo das doenças na qualidade de vida desses pacientes, e uma significante melhora em ambas as doenças após tratamento com toxina botulínica-A. Além disso, os dados fornecem subsídios para justificar o alto custo da aquisição da toxina botulínica-A, devido a seus consideráveis benefícios para a qualidade de vida.

Role of ARX Gene in Infantile Spasms and Dystonia

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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.

Spastic paretic hemifacial contracture as a presenting feature of multiple sclerosis.

Science.gov (United States)

Koutsis, Georgios; Breza, Marianthi; Evangelopoulos, Maria-Eleftheria; Anagnostouli, Maria; Andreadou, Elisavet; Karagiorgis, Georgios; Kokotis, Panagiotis; Kilidireas, Costas; Karandreas, Nikolaos

2017-04-01

Spastic paretic hemifacial contracture (SPHC) is characterized by sustained unilateral contraction of the facial muscles associated with mild ipsilateral facial paresis. Rarely described in the context of multiple sclerosis (MS), it has never been reported as presenting symptom of MS. Two patients developed SPHC within the context of a clinically isolated syndrome suggestive of MS. EMG revealed continuous resting activity of irregularly firing motor unit potentials, associated with impaired recruitment upon voluntary contraction. SPHC remitted fully in both patients. SPHC, a rare but distinct clinical and EMG entity, can occasionally be the presenting feature of MS. Copyright © 2017 Elsevier B.V. All rights reserved.

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)

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

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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.

Parry-Romberg syndrome (progressive hemifacial atrophy) with spasmodic dysphonia--a rare association.

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Mugundhan, K; Selvakumar, C J; Gunasekaran, K; Thiruvarutchelvan, K; Sivakumar, S; Anguraj, M; Arun, S

2014-04-01

Parry-Romberg syndrome is a rare clinical entity characterised by progressive hemifacial atrophy with appearance of 'saber'. Various neurological and otorhinolaryngological disorders are associated with this syndrome. The association of Parry -Romberg syndrome with Spasmodic dysphonia has rarely been reported. A 37 year old female presented with progressive atrophy of tissues of left side of face for 10 years and change in voice for 1 year. On examination, wasting and atrophy of tissues including tongue was noted on left side of the face. ENT examination revealed adductor spasmodic dysphonia. We report the rare association of Parry -Romberg syndrome with spasmodic dysphonia.

Progression of diffuse esophageal spasm to achalasia: incidence and predictive factors.

Science.gov (United States)

Fontes, L H S; Herbella, F A M; Rodriguez, T N; Trivino, T; Farah, J F M

2013-07-01

The progression of certain primary esophageal motor disorders to achalasia has been documented; however, the true incidence of this decay is still elusive. This study aims to evaluate: (i) the incidence of the progression of diffuse esophageal spasm to achalasia, and (ii) predictive factors to this progression. Thirty-five patients (mean age 53 years, 80% females) with a manometric picture of diffuse esophageal spasm were followed for at least 1 year. Patients with gastroesophageal reflux disease confirmed by pH monitoring or systemic diseases that may affect esophageal motility were excluded. Esophageal manometry was repeated in all patients. Five (14%) of the patients progressed to achalasia at a mean follow-up of 2.1 (range 1-4) years. Demographic characteristics were not predictive of transition to achalasia, while dysphagia (P= 0.005) as the main symptom and the wave amplitude of simultaneous waves less than 50 mmHg (P= 0.003) were statistically significant. In conclusion, the transition of diffuse esophageal spasm to achalasia is not frequent at a 2-year follow-up. Dysphagia and simultaneous waves with low amplitude are predictive factors for this degeneration. © 2012 Copyright the Authors. Journal compilation © 2012, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

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.

Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

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Irina Khamaganova

2018-01-01

Full Text Available Similar clinical and histhopathological features in progressive hemifacial atrophy and linear scleroderma en coup de sabre are well known. Trauma may predispose to the development of both diseases. The lack of association with anti-Borrelia antibodies was shown in both cases as well. The otolaryngological and endocrine disorders may be associated findings in both diseases. However, there are certain differences in neurological and ophthalmological changes in the diseases.

Interactive virtual simulation using a 3D computer graphics model for microvascular decompression surgery.

Science.gov (United States)

Oishi, Makoto; Fukuda, Masafumi; Hiraishi, Tetsuya; Yajima, Naoki; Sato, Yosuke; Fujii, Yukihiko

2012-09-01

The purpose of this paper is to report on the authors' advanced presurgical interactive virtual simulation technique using a 3D computer graphics model for microvascular decompression (MVD) surgery. The authors performed interactive virtual simulation prior to surgery in 26 patients with trigeminal neuralgia or hemifacial spasm. The 3D computer graphics models for interactive virtual simulation were composed of the brainstem, cerebellum, cranial nerves, vessels, and skull individually created by the image analysis, including segmentation, surface rendering, and data fusion for data collected by 3-T MRI and 64-row multidetector CT systems. Interactive virtual simulation was performed by employing novel computer-aided design software with manipulation of a haptic device to imitate the surgical procedures of bone drilling and retraction of the cerebellum. The findings were compared with intraoperative findings. In all patients, interactive virtual simulation provided detailed and realistic surgical perspectives, of sufficient quality, representing the lateral suboccipital route. The causes of trigeminal neuralgia or hemifacial spasm determined by observing 3D computer graphics models were concordant with those identified intraoperatively in 25 (96%) of 26 patients, which was a significantly higher rate than the 73% concordance rate (concordance in 19 of 26 patients) obtained by review of 2D images only (p computer graphics model provided a realistic environment for performing virtual simulations prior to MVD surgery and enabled us to ascertain complex microsurgical anatomy.

Espasmo hemifacial: resultados do tratamento cirúrgico em 14 casos

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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.

Vaginal spasm, pain, and behavior: an empirical investigation of the diagnosis of vaginismus.

Science.gov (United States)

Reissing, Elke D; Binik, Yitzchak M; Khalifé, Samir; Cohen, Deborah; Amsel, Rhonda

2004-02-01

This study investigated the roles of vaginal spasm, pain, and behavior in vaginismus and the ability of psychologists, gynecologists, and physical therapists to agree on a diagnosis of vaginismus. Eighty-seven women, matched on age, relationship status, and parity, were assigned to one of three groups: vaginismus, dyspareunia resulting from vulvar vestibulitis syndrome (VVS), and no pain with intercourse. Diagnostic agreement was poor for vaginismus; vaginal spasm and pain measures did not differentiate between women in the vaginismus and dyspareunia/VVS groups; however, women in the vaginismus group demonstrated significantly higher vaginal/pelvic muscle tone and lower muscle strength. Women in the vaginismus group also displayed a significantly higher frequency of defensive/avoidant distress behaviors during pelvic examinations and recalled past attempts at intercourse with more affective distress. These data suggest that the spasm-based definition of vaginismus is not adequate as a diagnostic marker for vaginismus. Pain and fear of pain, pelvic floor dysfunction, and behavioral avoidance need to be included in a multidimensional reconceptualization of vaginismus.

Bimatoprost (0.03%)-induced accommodative spasm and pseudomyopia

OpenAIRE

Padhy, Debananda; Rao, Aparna

2015-01-01

Bimatoprost is a prostaglandin analogue used topically in the treatment of glaucoma. Commonly known side effects include eyelash growth, iris pigmentation and conjunctival hyperemia. While pseudomyopia is reported to be caused by parasympathomimetics, such an effect precipitated by bimatoprost has not yet been reported. We report a case demonstrating pseudomyopia and accommodative spasm caused after starting bimatoprost 0.03% in a young patient with glaucoma.

The real world in the clinic before and after the establishment of guidelines for coronary artery spasm: a questionnaire for members of the Japanese Cine-angio Association.

Science.gov (United States)

Sueda, Shozo; Kohno, Hiroaki; Yoshino, Hideaki

2017-06-01

We investigated the clinical situations and the present knowledge of Japanese cardiologists about coronary artery spasm before and after the establishment of guidelines for this condition in the real world. A questionnaire was developed regarding the number of cases of coronary angiography, percutaneous coronary intervention, and invasive/non-invasive spasm provocation tests before (2008) and after (2014) the establishment of the Japanese Circulation Society (JCS) guidelines for coronary artery spasm and the status of spasm provocation tests. The questionnaire was sent to members of the Japanese Cine-angio Association in 81 cardiology hospitals in Japan. The completed surveys were returned from 20 hospitals, giving a response rate of 24.7%. Pharmacological spasm provocation tests increased in 2014 and vasospastic angina and variant angina also increased in 2014 compared with 2008, but the increase was not significant. Non-invasive spasm provocation tests such as hyperventilation tests and cold stress tests decreased remarkably in 2014. Spasm provocation tests were initially performed in the left coronary artery was employed in just 30% of the hospitals. The majority of institutions did not perform the spasm provocation testing in patients with unknown causes of heart failure or in survivors of ventricular fibrillation. Although 40% of the hospitals were not satisfied with standard spasm provocation tests, the majority of the hospitals agreed that spasm provocation tests will be necessary in the future. In general, the JCS guidelines contributed to the widespread use of provocative testing for coronary artery spasm in the real world. However, some issues about spasm still remained in the clinic.

Hemifacial Pain and Hemisensory Disturbance Referred from Occipital Neuralgia Caused by Pathological Vascular Contact of the Greater Occipital Nerve.

Science.gov (United States)

Son, Byung-Chul; Choi, Jin-Gyu

2017-01-01

Here we report a unique case of chronic occipital neuralgia caused by pathological vascular contact of the left greater occipital nerve. After 12 months of left-sided, unremitting occipital neuralgia, a hypesthesia and facial pain developed in the left hemiface. The decompression of the left greater occipital nerve from pathological contacts with the occipital artery resulted in immediate relief for hemifacial sensory change and facial pain, as well as chronic occipital neuralgia. Although referral of pain from the stimulation of occipital and cervical structures innervated by upper cervical nerves to the frontal head of V1 trigeminal distribution has been reported, the development of hemifacial sensory change associated with referred trigeminal pain from chronic occipital neuralgia is extremely rare. Chronic continuous and strong afferent input of occipital neuralgia caused by pathological vascular contact with the greater occipital nerve seemed to be associated with sensitization and hypersensitivity of the second-order neurons in the trigeminocervical complex, a population of neurons in the C2 dorsal horn characterized by receiving convergent input from dural and cervical structures.

Immunomodulatory effects of bone marrow-derived mesenchymal stem cells in a swine hemi-facial allotransplantation model.

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Yur-Ren Kuo

Full Text Available BACKGROUND: In this study, we investigated whether the infusion of bone marrow-derived mesenchymal stem cells (MSCs, combined with transient immunosuppressant treatment, could suppress allograft rejection and modulate T-cell regulation in a swine orthotopic hemi-facial composite tissue allotransplantation (CTA model. METHODOLOGY/PRINCIPAL FINDINGS: Outbred miniature swine underwent hemi-facial allotransplantation (day 0. Group-I (n = 5 consisted of untreated control animals. Group-II (n = 3 animals received MSCs alone (given on days -1, +1, +3, +7, +14, and +21. Group-III (n = 3 animals received CsA (days 0 to +28. Group-IV (n = 5 animals received CsA (days 0 to +28 and MSCs (days -1, +1, +3, +7, +14, and +21. The transplanted face tissue was observed daily for signs of rejection. Biopsies of donor tissues and recipient blood sample were obtained at specified predetermined times (per 2 weeks post-transplant or at the time of clinically evident rejection. Our results indicated that the MSC-CsA group had significantly prolonged allograft survival compared to the other groups (P<0.001. Histological examination of the MSC-CsA group displayed the lowest degree of rejection in alloskin and lymphoid gland tissues. TNF-α expression in circulating blood revealed significant suppression in the MSC and MSC-CsA treatment groups, as compared to that in controls. IHC staining showed CD45 and IL-6 expression were significantly decreased in MSC-CsA treatment groups compared to controls. The number of CD4+/CD25+ regulatory T-cells and IL-10 expressions in the circulating blood significantly increased in the MSC-CsA group compared to the other groups. IHC staining of alloskin tissue biopsies revealed a significant increase in the numbers of foxp3(+T-cells and TGF-β1 positive cells in the MSC-CsA group compared to the other groups. CONCLUSIONS: These results demonstrate that MSCs significantly prolong hemifacial CTA survival. Our data indicate the MSCs did not

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

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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

Considerations on patient-related outcomes with the use of botulinum toxins: is switching products safe?

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Fraint A

2016-02-01

Full Text Available Avram Fraint,1 Padmaja Vittal,2 Cynthia Comella2 1Department of Neurological Sciences, 2Section of Movement Disorders, Rush University Medical Center, Chicago, IL, USA Introduction: Botulinum toxin (BoNT is the treatment of choice for many neurologic movement disorders, including blepharospasm, hemifacial spasm, and cervical dystonia. There are two serotypes approved for use by the US Food and Drug Administration: three brands of serotype A and one of serotype B. Many attempts have been made at establishing dose conversion ratios between brands and serotypes. This review focuses on the existing data comparing different formulations of the same BoNT serotypes as well as that comparing different serotypes with one another. We focus on existing data regarding switching from one formulation or serotype to another and will also discuss the issue of immunogenicity of BoNT. With this information as a foundation, recommendations on safety of switching agents are addressed. Method: Literature review searching PubMed and Google Scholar using the search terms “switching botox”, “dosing equivalency in botox”, and “comparing botox”. Results/conclusion: Overall, there are many studies that demonstrate the efficacy and safety of each of the brands of BoNTs used in clinical practice. However, determination of dosing equivalencies among these brands and serotypes is complex with inconsistencies among the studies. When switching from one brand to another, the clinician should be aware of these issues, and not make the assumption that such ratios exist. Tailoring the dosage of each brand of BoNT to the clinical situation is the most prudent treatment strategy rather than focusing closely on conversion factors and concerns for immunogenicity. Keywords: botulinum toxin, BoNT, abobotulinumtoxin A, onabotulinumtoxin A, incobotulinumtoxin A, rimabotulinumtoxin B

Bimatoprost (0.03%)-induced accommodative spasm and pseudomyopia.

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Padhy, Debananda; Rao, Aparna

2015-11-23

Bimatoprost is a prostaglandin analogue used topically in the treatment of glaucoma. Commonly known side effects include eyelash growth, iris pigmentation and conjunctival hyperemia. While pseudomyopia is reported to be caused by parasympathomimetics, such an effect precipitated by bimatoprost has not yet been reported. We report a case demonstrating pseudomyopia and accommodative spasm caused after starting bimatoprost 0.03% in a young patient with glaucoma. 2015 BMJ Publishing Group Ltd.

Esophageal epiphrenic diverticulum associated with diffuse esophageal spasm

OpenAIRE

Matsumoto, Hideo; Kubota, Hisako; Higashida, Masaharu; Manabe, Noriaki; Haruma, Ken; Hirai, Toshihiro

2015-01-01

Introduction: Esophageal diverticulum, a relatively rare condition, has been considered to be associated with motor abnormalities such as conditions that cause a lack of coordination between the distal esophagus and lower esophageal sphincter. Presentation of case: We herein report a case of esophageal epiphrenic diverticulum associated with diffuse esophageal spasm. A 73-year-old woman presented with dysphagia and regurgitation. Imaging examinations revealed a right-sided esophageal diver...

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

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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.

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

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

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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].

Challenges in the management of massive intraorbital and hemifacial arteriovenous malformation as causing life-threatening epistaxis

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Anura Michelle Manuel

2017-03-01

Full Text Available Arteriovenous malformations are congenital lesions that may evolve with time and manifest in a plethora of presentations. They can occur as torrential epistaxis when it extensively involves the facial region. Multi-imaging modalities are available to assist in characterizing the structure of the lesion as well as its location and extent. This complex disease requires a multidisciplinary team approach with preoperative embolization and surgery. We present a rare cause of life-threatening epistaxis in a gentleman with a longstanding orbital and hemifacial arteriovenous malformation and discuss the complexities involved in its management.

Outcome of synthetic adrenocorticotropin hormone treatment in children with infantile spasm

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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.

Coronary spasm: 201Tl scintiscanning following pharmacological provocation

International Nuclear Information System (INIS)

Montz, R.; Mathey, D.; Bleifeld, W.; Hamburg Univ.

1981-01-01

According to the authors' experience so far, 201 Tl myocardial scintiscanning is a sufficiently sensitive non-invasive method for detection of coronary vasospasm provoked by ergotamine administration. Mild incomplete and asymptotic forms of coronary vasospasm were detected by scintiscanning. Indications for myocardial scintiscanning of ergotamine-provoked vasospasm are: Cases of angina pectoris at rest in which electrocardiograms during spasm are not available; elleviated symptoms after nitroglycerine administration; exercise electrocardiograms without any sign of ischaemia; negative results of exercise 201 Tl myocardial scintiscanning. (orig.) [de

CDKL5 mutations cause infantile spasms, early onset seizures, and severe mental retardation in female patients

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Archer, H L; Evans, J; Edwards, S; Colley, J; Newbury‐Ecob, R; O'Callaghan, F; Huyton, M; O'Regan, M; Tolmie, J; Sampson, J; Clarke, A; Osborne, J

2006-01-01

Objective To determine the frequency of mutations in CDKL5 in both male and female patients with infantile spasms or early onset epilepsy of unknown cause, and to consider whether the breadth of the reported phenotype would be extended by studying a different patient group. Methods Two groups of patients were investigated for CDKL5 mutations. Group 1 comprised 73 patients (57 female, 16 male) referred to Cardiff for CDKL5 analysis, of whom 49 (42 female, 7 male) had epileptic seizure onset in the first six months of life. Group 2 comprised 26 patients (11 female, 15 male) with infantile spasms previously recruited to a clinical trial, the UK Infantile Spasms Study. Where a likely pathogenic mutation was identified, further clinical data were reviewed. Results Seven likely pathogenic mutations were found among female patients from group 1 with epileptic seizure onset in the first six months of life, accounting for seven of the 42 in this group (17%). No mutations other than the already published mutation were found in female patients from group 2, or in any male patient from either study group. All patients with mutations had early signs of developmental delay and most had made little developmental progress. Further clinical information was available for six patients: autistic features and tactile hypersensitivity were common but only one had suggestive Rett‐like features. All had a severe epileptic seizure disorder, all but one of whom had myoclonic jerks. The EEG showed focal or generalised changes and in those with infantile spasms, hypsarrhythmia. Slow frequencies were seen frequently with a frontal or fronto‐temporal predominance and high amplitudes. Conclusions The spectrum of the epileptic seizure disorder, and associated EEG changes, in those with CDKL5 mutations is broader than previously reported. CDKL5 mutations are a significant cause of infantile spasms and early epileptic seizures in female patients, and of a later intractable seizure disorder

Hemifacial Pain and Hemisensory Disturbance Referred from Occipital Neuralgia Caused by Pathological Vascular Contact of the Greater Occipital Nerve

OpenAIRE

Son, Byung-chul; Choi, Jin-gyu

2017-01-01

Here we report a unique case of chronic occipital neuralgia caused by pathological vascular contact of the left greater occipital nerve. After 12 months of left-sided, unremitting occipital neuralgia, a hypesthesia and facial pain developed in the left hemiface. The decompression of the left greater occipital nerve from pathological contacts with the occipital artery resulted in immediate relief for hemifacial sensory change and facial pain, as well as chronic occipital neuralgia. Although re...

Painful tonic spasms and brainstem involvement in a patient with neuromyelitis optica spectrum disorder.

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Roman-Filip, Corina; Ungureanu, Aurelian; Cernuşcă-Miţaru, Mihaela

2016-01-01

Neuromyelitis optica (NMO) is an inflammatory-demyelinating disease of the central nervous system classically characterized by optic neuritis and severe myelitis. New diagnostic criteria defined neuromyelitis optica spectrum disorder as limited forms of NMO or diverse neurologic presentations in the presence of specific antiaquaporin-4 antibodies. We report the case of a 57-year-old woman admitted in our department for recurrent attacks of optic neuritis, tetraparesis with severe painful tonic spasms of the left limbs and brainstem involvement. Painful tonic spasms have been described as movement disorders associated with multiple sclerosis, but a growing number of reports describe them in cases of NMO. Copyright © 2015 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Challenges in the management of massive intraorbital and hemifacial arteriovenous malformation as causing life-threatening epistaxis.

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Manuel, Anura Michelle; Kalimuthu, Santhi; Pathmanathan, Sitra Siri; Narayanan, Prepageran; Zainal Abidin, Zurina; Azmi, Khairul; Khalil, Alizan

2017-04-01

Arteriovenous malformations are congenital lesions that may evolve with time and manifest in a plethora of presentations. They can occur as torrential epistaxis when it extensively involves the facial region. Multi-imaging modalities are available to assist in characterizing the structure of the lesion as well as its location and extent. This complex disease requires a multidisciplinary team approach with preoperative embolization and surgery. We present a rare cause of life-threatening epistaxis in a gentleman with a longstanding orbital and hemifacial arteriovenous malformation and discuss the complexities involved in its management. Copyright © 2017. Published by Elsevier Taiwan.

Value of MR cisternography using three-dimensional half-fourier single-shot fast spin-echo sequences in the diagnosis of diseases related to cranial nerves VII and VIII

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Yamakami, Norio [Kyorin Univ., Mitaka, Tokyo (Japan). School of Medicine

1999-05-01

The purpose of this study was to evaluate the value of MR cisternography using three-dimensional half-Fourier single-shot fast spin-echo sequences in the diagnosis of diseasea related to cranial nerves VII and VIII. With a 0.5-T imager, the most appropriate setting of echo time and section thickness was first assessed in five volunteers. This resulted in echo time of 250 msec and section thickness of 2 mm as the most effective parameters. Second, using echo time of 120 msec and section thickness of 1.5 mm that were available from the beginning of this study, the demonstration of four nerves within the audistory canal was assessed in seven volunteers. In all of the volunteers, the facial, cochlear, and vestibular nerves were determined with demonstration of each of superior and inferior vestibular nerves in four of them. Next, MR cisternography using the same echo time and section thickness was applied in 368 patients with suspicion of acoustic neurinoma and 14 with hemifacial spasm. In 28 of the 368 patients, MR cisternograms depicted an acoustic neurinoma that was confirmed on postcontrast T1-weighted images. Meanwhile, in five of the 14 patients with hemifacial spasm, MR cisternograms revealed a vessel compressing the root exit zone of the affected facial nerve. It is concluded that MR cisternography using three-dimensional half-Fourier single-shot fast spin-echo sequences can be a useful means for demonstrating nerves within the auditory nerve as well as for the screening of acoustic neurionoma. (author)

Value of MR cisternography using three-dimensional half-fourier single-shot fast spin-echo sequences in the diagnosis of diseases related to cranial nerves VII and VIII

International Nuclear Information System (INIS)

Yamakami, Norio

1999-01-01

The purpose of this study was to evaluate the value of MR cisternography using three-dimensional half-Fourier single-shot fast spin-echo sequences in the diagnosis of diseasea related to cranial nerves VII and VIII. With a 0.5-T imager, the most appropriate setting of echo time and section thickness was first assessed in five volunteers. This resulted in echo time of 250 msec and section thickness of 2 mm as the most effective parameters. Second, using echo time of 120 msec and section thickness of 1.5 mm that were available from the beginning of this study, the demonstration of four nerves within the audistory canal was assessed in seven volunteers. In all of the volunteers, the facial, cochlear, and vestibular nerves were determined with demonstration of each of superior and inferior vestibular nerves in four of them. Next, MR cisternography using the same echo time and section thickness was applied in 368 patients with suspicion of acoustic neurinoma and 14 with hemifacial spasm. In 28 of the 368 patients, MR cisternograms depicted an acoustic neurinoma that was confirmed on postcontrast T1-weighted images. Meanwhile, in five of the 14 patients with hemifacial spasm, MR cisternograms revealed a vessel compressing the root exit zone of the affected facial nerve. It is concluded that MR cisternography using three-dimensional half-Fourier single-shot fast spin-echo sequences can be a useful means for demonstrating nerves within the auditory nerve as well as for the screening of acoustic neurionoma. (author)

The history of facial palsy and spasm

Science.gov (United States)

Sajadi, Mohamad-Reza M.; Tabatabaie, Seyed Mahmoud

2011-01-01

Although Sir Charles Bell was the first to provide the anatomic basis for the condition that bears his name, in recent years researchers have shown that other European physicians provided earlier clinical descriptions of peripheral cranial nerve 7 palsy. In this article, we describe the history of facial distortion by Greek, Roman, and Persian physicians, culminating in Razi's detailed description in al-Hawi. Razi distinguished facial muscle spasm from paralysis, distinguished central from peripheral lesions, gave the earliest description of loss of forehead wrinkling, and gave the earliest known description of bilateral facial palsy. In doing so, he accurately described the clinical hallmarks of a condition that we recognize as Bell palsy. PMID:21747074

A fetus with hemifacial microsomia and sirenomelia. The same mesodermal defect spectrum?

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López-Valdez, Jaime Asael; Estrada-Juárez, Higinio; Moreno-Verduzco, Elsa Romelia; Aguinaga-Ríos, Mónica

2013-04-01

Sirenomelia is the most severe malformation complex affecting the human caudal pole, although its etiology is unclear, a primary defect of blastogenesis has been proposed. Studies consider sirenomelia as the most severe form of caudal dysgenesis, VACTERL association, or axial mesodermal dysplasia, although others still support the idea of a different pathologic entity. We report the prenatal, clinical, and pathologic features of a fetus with cleft lip and palate, microtia, cardiac, renal and intestinal malformations, radial aplasia, and sirenomelia. Karyotype, chromosomal breakage studies, and SHH sequence analysis were normal. The occurrence of cephalic, midline-paramedial, and caudal malformations in the same patient imply the diagnosis of hemifacial microsomia and sirenomelia. These entities are part of the same mesodermal malformation spectrum and the clinical presentation depends on environmental and genetic interactions in embrionic development. Future clinical and genome wide studies will help to better delineate this spectrum.

Refractive Lens Exchange with Multifocal Intraocular Lens for Treatment of Chronic Intermittent Spasm of the Near Reflex

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Guy Sallet

2017-12-01

Full Text Available We report the case of an emmetropic 32-year-old female with decreased uncorrected visual acuity and diplopia due to intermittent episodes of spasm of the near reflex. Neurologic, general, and ophthalmic examination could not find an organic cause. Attempts at spontaneous recovery, psychogenic therapy, and cycloplegic therapy were unsuccessful and the symptoms persisted for almost 5 years, leading to psychogenic distress. Final treatment with refractive lens exchange and implantation of a toric trifocal intraocular lens resolved the spasm of the near reflex, resulting in an uncorrected distance and near visual acuity of 20/20.

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

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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.

Low-amplitude distal esophageal spasm as a cause of severe dysphagia for solid food

NARCIS (Netherlands)

Breumelhof, R.; Timmer, R.; van Hees, P. A.; Obertop, H.; Smout, A. J.

1996-01-01

This case report presents a patient with progressive dysphagia, accompanied by weight loss, in the absence of organic disease. Esophageal motility studies initially failed to reveal a diagnosis. At simultaneous manometry and fluoroscopy, with bread/barium boluses, the diagnosis of esophageal spasm

Development of a morphing technique for predicting the position and size of an artificial ear in hemifacial microsomia patients.

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Coward, Trevor J; Richards, Robin; Scott, Brendan J J

2014-01-01

People with hemifacial microsomia may be missing an ear on the affected side of the face. The principal aim of the study was to develop a morphing technique and to determine whether it could be used to appropriately position an artificial ear, as well as to give an indication of prosthesis size in comparison with the natural ear. Comparisons also were made between the artificial ears being worn by the patients with their natural ears. Data from stereophotogrammetry images of the faces of 10 people were converted into stereolithographic format. Anthropometric points on the face and ear of the unaffected side were plotted. By a process of scaling, the distance between facial landmarks on the unaffected side was estimated for the affected side so as to identify where the morphed ear would be positioned once generated. Generally, the morphed ears appeared to be in acceptable positions. There was a statistically significant difference between the position of the morphed and natural ears (P = .011), as well as the artificial and natural ears (P = .001), but this was unlikely to have any clinical implications. There were no significant differences among the sizes of the natural, morphed, and artificial ears (P = .072). Morphing appears to offer a more precise way of planning the positioning and construction of an artificial ear on patients with hemifacial microsomia than traditional methods. Differences in facial shape on either side of the face may impact on the process. This requires further study.

Proteomic analysis of adrenocorticotropic hormone treatment of an infantile spasm model induced by N-methyl-D-aspartic acid and prenatal stress.

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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.

Neuromyelitis optica: association with paroxysmal painful tonic spasms.

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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.

Evaluation of MR cisternography of the cerebellopontine angle using a balanced fast-field-echo sequence: preliminary findings

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Tsuchiya, Kazuhiro; Aoki, Chinatsu; Hachiya, Junichi [Department of Radiology, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, 181-8611, Tokyo (Japan)

2004-02-01

We evaluated the feasibility of MR cisternography by the balanced fast-field-echo (bFFE) sequence, comparing with that by a turbo-spin-echo (TSE) sequence, for cerebellopontine angle lesions on a 1.5-T imager (Gyroscan Intera, Philips, Best, The Netherlands). The bFFE MR cisternograms depicted target cranial nerves with less cerebrospinal fluid pulsation artifacts than TSE cisternograms and visualized an acoustic schwannoma in 6 of 44 patients with suspicion and a causative vessel of hemifacial spasm in all of 3 patients in a short scanning time (1 min 53 s). The bFFE sequence can be promising for MR cisternography in the diagnosis of cerebellopontine angle lesions. (orig.)

Botulinum toxin A injection for chronic anal fissures and anal sphincter spasm improves quality of life in recessive dystrophic epidermolysis bullosa

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Cassandra Chaptini, MBBS

2015-12-01

Full Text Available We report a 20-year-old female with generalized, severe, recessive dystrophic epidermolysis bullosa who developed secondary chronic anal fissures. This resulted in anal sphincter spasm and severe, disabling pain. She was treated with five botulinum toxin A injections into the internal anal sphincter over a period of 2 years and gained marked improvement in her symptoms. This case demonstrates the successful use of botulinum toxin A injections to relieve anal sphincter spasm and fissuring, with long-term improvement.

The Result of The Treatment of Infantile Spasm After Two Year Follow-Up Review Of 45 Cases in Ten Past Years

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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.

Spasm of the near reflex: A case report.

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Rhatigan, Maedbh; Byrne, Caroline; Logan, Patricia

2017-06-01

Spasm of the near reflex (SNR) is a triad of miosis, excess accommodation and excess convergence. Primary SNR is most often functional in origin We aim to highlight the clinical features which distinguish primary convergence from other conditions with a similar presentation but more sinister underlying aetiology, for example bilateral abducens nerve palsy. There is a paucity of published data on SNR, in particular diagnostic criteria and treatment. We report a case of SNR of functional origin in an otherwise healthy young female and discuss the clinical features that differentiate this condition from similar conditions with underlying neurological origin. SNR is predominantly a clinical diagnosis, and often leads to patients undergoing unnecessary investigations and sometimes treatment. Recognising the salient features that differentiate it could potentially avoid this.

Functional aspects of distal oesophageal spasm: the role of onset velocity and contraction amplitude on bolus transit

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Pohl, Daniel; Ciolino, Jody; Roberts, Jason; Savarino, Edoardo; Freeman, Janice; Nietert, Paul J; Tutuian, Radu; Castell, Donald

2012-01-01

Background Distal oesophageal spasm (DES) is a rare and under-investigated motility abnormality. Recent studies indicate effective bolus transit in varying percentages of DES patients. Aim Explore functional aspects including contraction onset velocity and contraction amplitude cut-off values for simultaneous contractions to predict complete bolus transit Methods We re-examined data from 107 impedance-manometry recordings with a diagnosis of DES. Receiver operating characteristic (ROC) analysis was conducted, regarding effects of onset velocity on bolus transit taking into account distal oesophageal amplitude (DEA) and correcting for intra-individual repeated measures. Results Mean area under the ROC curve for saline and viscous swallows were 0.84±0.05 and 0.84±0.04, respectively. Velocity criteria of >30cm/s when DEA>100mmHg and 8cm/s when DEADEA>100mmHg and >7cm/s when DEAsensitivity of 75% and specificity of 80% to identify complete bolus transit. Using these criteria, final diagnosis changed in 44.9% of patients. Abnormal bolus transit was observed in 50.9% of newly diagnosed DES patients versus 7.5% of patients classified as normal. DES patients with DEA>100mmHg suffered twice as often from chest pain than those with DEA<100mmHg. Conclusion The proposed velocity cut-offs for diagnosing distal oesophageal spasm improve the ability to identify patients with spasm and abnormal bolus transit. PMID:22475443

[The effect of mandibular distraction on the maxilla growth in children with hemifacial microsomia].

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Yang, Renkai; Tang, Xiaojun; Shi, Lei; Yin, Lin; Yang, Bin; Yin, Hongyu; Liu, Wei; Zhang, Zhiyong

2014-11-01

To analyze the effect of mandibular distraction on the maxilla growth in children with hemifacial microsomia through measurement with the posterior-anterior cephalometric X-ray films and Three-dimensional CT reconstruction images. The deviation angular of maxilla occlusion plane and nasal base plane from the infra-orbital plane were measured on the posterior-anterior cephalometric X-ray films in 22 patients before and half a year after operation. The vertical distance from the midpoint of 5th teeth alveolar and the lowest point of maxillary sinus to reference plane were measured on 3D reconstruction images in 15 patients. The data were statistically analyzed. On posterior-anterior cephalometric X-ray films, the cant of occlusion plane were significantly reduced (P maxillary sinus on normal side. Distraction osteogenesis of mandible can promote the growth of the maxilla in children with HFM, the accelerated growth parts include alveolar bone and maxillary sinus.

Maxillary-driven simultaneous maxillo-mandibular distraction for hemifacial microsomia.

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Nakajima, Hideo; Sakamoto, Yoshiaki; Tamada, Ikkei; Ogata, Hisao; Kishi, Kazuo; Sakamoto, Teruo

2011-12-01

We treat hemifacial microsomia with a combination of surgery and orthodontic treatment during the growth period, resulting in early improvement in facial asymmetry and the induction of normal growth. We previously used gradual distraction of the mandibular ramus for Pruzansky's type II classification (Pruzansky, 1969). In type II cases, the maxilla should also be treated actively as, using this technique, improvement of the occlusal plane is difficult to achieve, resulting in a cross bite and difficulties in post-operative orthodontic treatment-especially in older patients. Morphologically, the mandibular angle region of the operative side is flat, and the angle of the mouth remains elevated. We performed mandibular-driven simultaneous maxillo-mandibular distraction while the occlusion was maintained using intermaxillary anchorage. However, mandibular-driven distraction tended to elongate the face because the mandible only elongated downwards and the mandibular ramus did not reach the glenoid. Furthermore, external distraction devices produce significant distress for patients until removal of the device and cause scars on the face. We developed a new internal distraction device with a variable angle and performed maxillary-driven simultaneous maxillo-mandibular distraction using this device. The result was morphologically satisfactory and solved the above problems. Because the patient was in the growth period, careful follow-up and induction to normal growth were important while the inferior growth of the affected side was monitored. Copyright © 2010 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Simultaneous right coronary artery spasm in a patient with Anterior ST-Segment Elevation Myocardial Infarction: a case report

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Zhiva Taherpour

2013-05-01

Full Text Available Please cite this article as: Taherpour Z, Seyedian M, Alasti M. Simultaneous right coronary artery spasm in a patient with Anterior ST-Segment Elevation Myocardial Infarction: a case report. Novel Biomed 2013;1:29-33.Simultaneous occlusion of two vessels causing infarction at different territories is an uncommon finding. We report simultaneous right ventricular and anterior ST-segment elevation myocardial infarction in a previously healthy young man.The angiographic results demonstrated the simultaneous occlusion of the right and left coronary arteries because of simultaneous occlusion of left anterior descending artery (LADA and spasm of right coronary artery (RCA. In this patient, we found simultaneous ST elevations in right and precordial leads so everyone should be careful about all leads of the surface electrocardiogram for decision making in the management of a patient.

Use of various free flaps in progressive hemifacial atrophy.

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Baek, Rongmin; Heo, Chanyeong; Kim, Baek-kyu

2011-11-01

Romberg disease is an uncommon condition manifested by progressive hemifacial atrophy of the skin, soft tissue, and bone. Facial asymmetry with soft tissue deficiency in Romberg disease causes a significant disability affecting the social life and can bring about many psychological problems. The aim of surgical treatment is cosmetic amelioration of the defect. Several conventional reconstructive procedures have been used for correcting facial asymmetry. They include fat injections, dermal fat grafts, filler injections, cartilage and bone grafts, and pedicled and free flaps. We report our experiences with 11 patients involving 11 free flaps with a minimum 1-year follow-up. All patients were classified as having moderate to severe atrophy. The average age at disease onset was 4.5 years; the average duration of atrophy was 5.2 years. No patients were operated on with a quiescent interval of less than 1 year. The average age at operation was 20.1 years, ranging from 10 to 55 years. Reconstruction was performed using 4 groin dermofat free flaps, 4 latissimus dorsi muscle free flaps, and 3 other perforator flaps. To achieve the finest symmetrical and aesthetic results, several ancillary procedures were performed in 4 patients. These procedures included Le Fort I leveling osteotomy, sagittal split ramus osteotomy, reduction malarplasty and angle plasty, rib and calvarial bone graft, correction of alopecia, and additional fat graft. All patients were satisfied with the results. We believe that a free flap transfer is the requisite treatment modality for severe degree of facial asymmetry in Romberg disease.

Coronary spasm induced by dipyridamole

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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)

Efecto a corto plazo de la vigabatrina en los espasmos infantiles Short term effect of vigabatrin in infantile spasms

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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

Spasm of the near reflex: a spectrum of anomalies.

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Goldstein, J H; Schneekloth, B B

1996-01-01

Spasm of the near reflex has been characterized as the variable appearance of pseudomyopia, convergent strabismus and miosis. These characteristics may appear together or separately. In addition, abnormalities of accommodation may appear not only as pseudomyopia, but may also be manifest in cases with significant hypermetropia in which the patient is unable to relax accommodation even when plus lenses are used. The intent of this review is to identify the various clinical presentations of anomalies of the entire near reflex as well as the component parts. The relationship to functional and organic disorders are discussed as well as the related neuroanatomy. We suggest that one may more readily understand the clinical manifestations as a spectrum of anomalies of the near reflex rather than a multitude of disconnected entities.

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...

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

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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.

Vascular anomalies of the cerebellopontine angle

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Papanagiotou, P.; Grunwald, I.Q.; Politi, M.; Struffert, T.; Ahlhelm, F.; Reith, W.

2006-01-01

Vascular anomalies of the cerebellopontine angle are rare compared to tumors in this area. Irritation of the trigeminal, facial, or vestibulocochlear nerve may cause trigeminal neuralgia, hemifacial spasm and vertigo, or tinnitus accordingly. Vessel loops in the cerebellopontine cisterns may cause compression at the root entry or exit zone of the cranial nerves V, VII, and VIII, a phenomenon which is called ''vascular loop syndrome.'' Megadolichobasilar artery and aneurysms of the vertebrobasilar system can also lead to dislocation and compression of the cranial nerves and brain stem. Three-dimensional CISS MR imaging and MR angiography are useful in the detection of neurovascular compression. Microvascular decompression is an effective surgical procedure in the management of compression syndromes of the cranial nerves V, VII, and VIII. (orig.) [de

Manifestation of J wave induced by acetylcholine applied for a coronary spasm provocation test in a patient with aborted sudden cardiac death.

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Kodama, Hiroyuki; Fujita, Kazumasa; Moriyama, Shouhei; Irie, Kei; Noda, Hirotaka; Yokoyama, Taku; Fukata, Mitsuhiro; Arita, Takeshi; Odashiro, Keita; Maruyama, Toru; Akashi, Koichi

2017-06-01

A 51-year-old man with a resuscitation episode was referred to our hospital. Coronary angiography revealed a focal spasm overlapped with organic stenosis where a bare metal stent was implanted. Acetylcholine (ACh) provocation test did not induce chest pain. It revealed no discernible ST-T changes but unmasked a J wave at the end of the QRS complex, which was associated with short-coupled repetitive premature ventricular beats. A J wave reportedly appears immediately before the onset of ventricular fibrillation caused by vasospastic angina. However, a J wave observed newly after a coronary spasm provocation test using ACh without ST-T changes is informative when considering the mechanisms of the J wave.

Clear lens extraction for the treatment of persistent accommodative spasm after head trauma.

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McMurray, Catherine J; Burley, Celeste D; Elder, Mark J

2004-12-01

We report the case of a 28-year-old man with decreased visual acuity after closed head trauma sustained in a motor vehicle accident 16 weeks earlier. Several structures thought to be associated with the control of accommodation were injured. The patient had a persistent accommodative spasm causing up to 7.0 diopters of pseudomyopia. We present the patient's progress through the clinic, including manifest and cycloplegic refractions and results of a trial with atropine drops, and successful transition to bilateral pseudophakia 2 years and 9 months after the accident.

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

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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.

Shaken baby syndrome manifesting as infantile spasms seizure type

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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.

Tonic spasms are a common clinical manifestation in patients with neuromyelitis optica Espasmos tônicos são manifestações clínicas frequentes em pacientes com neuromielite óptica

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Luz Abaroa

2013-05-01

Full Text Available Tonic spasms have been most commonly associated with multiple sclerosis. To date, few reports of series of patients with neuromyelitis optica and tonic spasms have been published. Methods: We analyzed the characteristics and frequency of tonic spasms in 19 subjects with neuromyelitis optica. Data was collected using a semi-structured questionnaire for tonic spasms, by both retrospectively reviewing medical records and performing clinical assessment. Results: All patients except one developed this symptom. The main triggering factors were sudden movements and emotional factors. Spasms were commonly associated to sensory disturbances and worsened during the acute phases of the disease. Carbamazepine was most commonly used to treat the symptom and patients showed good response to the drug. Conclusions: Tonic spasms are a common clinical manifestation in patients with neuromyelitis optica.Espasmos tônicos têm sido mais frequentemente associados com esclerose múltipla. Foram publicados até agora poucos relatos de série de pacientes com neuromielite óptica e espasmos tônicos. Métodos: Foram analisadas as características e a frequência de espasmos tônicos em 19 indivíduos com neuromielite óptica. Os dados foram coletados por meio de um questionário semiestruturado para espasmos tônicos, mediante a avaliação retrospectiva dos prontuários e a análise dos dados clínicos Resultados: Todos os pacientes com neuromielite óptica exceto um apresentaram espasmos tônicos. Os principais fatores desencadeantes foram movimentos bruscos e fatores emocionais. Espasmos foram frequentemente associados a perturbações sensoriais e se agravaram durante a fase aguda da doença. A carbamazepina foi utilizada frequentemente para tratar os sintomas, com boa resposta. Conclusões: Os espasmos tônicos são manifestações clínicas frequentes em pacientes com neuromielite óptica.

Combined maxillary and mandibular distraction osteogenesis in patients with hemifacial microsomia.

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Sant'Anna, Eduardo Franzotti; Lau, Geórgia W T; Marquezan, Mariana; de Souza Araújo, Mônica Tirre; Polley, John W; Figueroa, Alvaro A

2015-05-01

Hemifacial microsomia is a deformity of variable expressivity with unilateral hypoplasia of the mandible and the ear. In this study, we evaluated skeletal soft tissue changes after bimaxillary unilateral vertical distraction. Eight patients (4 preadolescents 4 adolescents) each with a grade II mandibular deformity underwent a LeFort I osteotomy and an ipsilateral horizontal mandibular ramus osteotomy. A semiburied distraction device was placed over the ramus, and intermaxillary fixation was applied. Anteroposterior cephalometric and frontal photographic analyses were conducted before and after distraction. Statistics were used to analyze the preoperative and postoperative changes. Cephalometrically, the nasal floor and the occlusal and gonial plane angles decreased. The ratios of affected-unaffected ramus and gonial angle heights improved by 15% and 20%, respectively. The position of menton moved toward the midline. The photographic analysis showed a decrease of the nasal and commissure plane angles, and the chin moved to the unaffected side. The parallelism between the horizontal skeletal and soft tissue planes improved, with an increase in the affected side ramus height and correction of the chin point toward the midline. Simultaneous maxillary and mandibular distraction improved facial balance and symmetry. Patients in the permanent dentition with fixed orthodontic appliances and well-aligned dental arches responded well to this intervention. Copyright © 2015 American Association of Orthodontists. Published by Elsevier Inc. All rights reserved.

The effect of vasodilatory medications on radial artery spasm in patients undergoing transradial coronary artery procedures: a systematic review.

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Curtis, Elizabeth; Fernandez, Ritin; Lee, Astin

2017-07-01

The uptake of percutaneous coronary procedures via the radial artery has increased internationally due to the decreased risk of complications and increased patient satisfaction. The increased susceptibility of the radial artery to spasm however presents a potential risk for procedural failure. Although most experts agree on the need for prophylactic medications to reduce radial artery spasm, currently there is inconsistency in literature regarding the most effective vasodilatory medication or combination of medications. The objective of this study is to identify the effectiveness of vasodilatory medications on radial artery spasm in patients undergoing transradial coronary artery procedures. This review considered studies that included participants aged 18 years and over undergoing non-emergent transradial percutaneous coronary artery procedures. This review considered studies that used vasodilating intravenous and intra-arterial medications or combinations of medications prior to commencing and during transradial coronary approaches to reduce radial artery spasm. The outcomes of interest were the incidence of radial artery spasm during percutaneous coronary procedure using objective and/or subjective measures and its effect on the successful completion of the procedure. Randomized controlled trials published in the English language between 1989 to date were considered for inclusion. The search strategy aimed to find both published and unpublished studies. A three-step search strategy was utilized in this review. An initial search of MEDLINE, CINAHL and Scopus was undertaken, followed by a search for unpublished studies. Papers selected for retrieval were assessed by two independent reviewers for methodological validity prior to inclusion in the review using standardized critical appraisal instruments. Any disagreements that arose between the reviewers were resolved through discussion. Quantitative data was extracted from papers included in the review using the

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

Botulinum Toxin for the Treatment of Tremor and Tics.

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Lotia, Mitesh; Jankovic, Joseph

2016-02-01

The therapeutic applications of botulinum toxin (BoNT) have grown manifold since its initial approval in 1989 by the U.S. Food and Drug Administration for the treatment of strabismus, blepharospasm, and other facial spasms. Although it is the most potent biologic toxin known to man, long-term studies have established its safety in the treatment of a variety of neurologic and nonneurologic disorders. Despite a paucity of randomized controlled trials, BoNT has been found to be beneficial in treating a variety of tremors and tics when used by clinicians skilled in the administration of the drug for these hyperkinetic movement disorders. Botulinum toxin injections can provide meaningful improvement in patients with localized tremors and tics; in some cases, they may be an alternative to other treatments with more undesirable adverse effects. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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

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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.

Modified Ashworth scale and spasm frequency score in spinal cord injury

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

2016-01-01

.94 and inter-rater κweighted=0.93. Correlation between MAS and SFS showed non-significant correlation coefficients from-0.11 to 0.90. CONCLUSION: 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......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....... SETTING: Clinic for Spinal Cord Injuries, Rigshospitalet, Hornbaek, Denmark. METHODS: 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...

Neuroimaging findings in movement disorders

International Nuclear Information System (INIS)

Topalov, N.

2015-01-01

Full text: Neuroimaging methods are of great importance for the differential diagnostic delimitation of movement disorders associated with structural damage (neoplasms, ischemic lesions, neuroinfections) from those associated with specific pathophysiological mechanisms (dysmetabolic disorders, neurotransmitter disorders). Learning objective: Presentation of typical imaging findings contributing to nosological differentiation in groups of movement disorders with similar clinical signs. In this presentation are discussed neuroimaging findings in Parkinson‘s disease, atypical parkinsonian syndromes (multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration), parkinsonism in genetically mediated diseases (Wilson’s disease, pantothenate kinase-associated neurodegeneration – PKAN), vascular parkinsonism, hyperkinetic movement disorders (palatal tremor, Huntington‘s chorea, symptomatic chorea in ischemic stroke and diabetes, rubral tremor, ballismus, hemifacial spasm). Contemporary neuroimaging methods enable support for diagnostic and differential diagnostic precision of a number of hypo- and hyperkinetic movement disorders, which is essential for neurological clinical practice

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

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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.

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

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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.

Infantile spasms with periventricular nodular heterotopia, unbalanced chromosomal translocation 3p26.2 -10p15.1 and 6q22.31 duplication.

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Jones, Kevin; Weiss, Shelly K; Minassian, Berge

2016-07-01

Patients presenting with infantile spasms, dysmorphic features, and periventricular nodular heterotopia may benefit from genetic copy number variation microarray, or whole-exome sequencing to identify candidate genes. This will allow personalized diagnosis and prognostication and the eventual understanding of single and combined gene functions in brain health and disease.

The research on 3D printing fingerboard and the initial application on cerebral stroke patient's hand spasm.

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Wang, Kai; Shi, Yiting; He, Wen; Yuan, Jing; Li, Yuan; Pan, Xiaolin; Zhao, Cuilian

2018-06-26

To research the possibility of designing customized 3D printing fingerboard to apply to the limb rehabilitation of cerebral stroke patients as well as the prevention and treatment of finger spasm, through 3D printing technology. Taking 18 hospitalized cerebral stroke patients for example, through scanning, molding and printing, to make and wear 3D printing fingerboard for them, and then observe the compliance, main complaint, muscular tension of affected hand and changes on range of motion after they wear the fingerboard for 3 weeks and 3 months. Have acquired completed data from 13 patients. The time of them wearing the fingerboard every day varied from 1 to 8 h, and most of them reflected that they felt comfortable and there was no feeling of worsened pain or finger skin allergy. In addition, the patients' grip strength, hand function and range of motion improved by varying degrees while their muscular tensions declined by varying degrees. The tension and bending resistance of the fingerboard all met the patients' treatment requirements. With the advantages of being accurate and customized, 3D printing fingerboard can benefit patients fixing and orthopedic treatment, and even prevent and treat cerebral stroke patient's finger spasm. Trial registration The research topic has been registered in Chinese Clinic Trial Registry. Registration time: January 15, 2016. Registration topic: The Use of 3D Printing Technology in the Orthotic of Extremity Rehabilitation of Stroke Patient. Registration Number: ChiCTR-INR-16007774.

MRI-based diagnostic imaging of the intratemporal facial nerve

International Nuclear Information System (INIS)

Kress, B.; Baehren, W.

2001-01-01

Detailed imaging of the five sections of the full intratemporal course of the facial nerve can be achieved by MRI and using thin tomographic section techniques and surface coils. Contrast media are required for tomographic imaging of pathological processes. Established methods are available for diagnostic evaluation of cerebellopontine angle tumors and chronic Bell's palsy, as well as hemifacial spasms. A method still under discussion is MRI for diagnostic evaluation of Bell's palsy in the presence of fractures of the petrous bone, when blood volumes in the petrous bone make evaluation even more difficult. MRI-based diagnostic evaluation of the idiopatic facial paralysis currently is subject to change. Its usual application cannot be recommended for routine evaluation at present. However, a quantitative analysis of contrast medium uptake of the nerve may be an approach to improve the prognostic value of MRI in acute phases of Bell's palsy. (orig./CB) [de

Painful tic convulsif syndrome due to vertebrobasilar dolichoectasia

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Puneet Mittal

2011-01-01

Full Text Available Combined clinical presentation of hemifacial spasm and ipsilateral trigeminal neuralgia is also known as painful tic convulsif (PTC. It is a rare condition and the most common cause is vascular compression. Vertebrobasilar dolichoectasia (VBD is characterized by dilated and tortuous vertebral and basilar arteries. VBD is an uncommon and rarely reported cause of PTC. Magnetic resonance imaging (MRI, due to its inherent excellent contrast resolution, is an excellent modality for demonstrating the nerve compression by dilated and tortuous vessels seen in this condition. For this purpose, 3D MRI sequences are especially useful like constructive interference in steady state (CISS and MR angiography. Both of these have been reported to be helpful in the diagnosis of this condition. We report a case of PTC in which we were able to document facial and trigeminal nerve compression by VBD on MRI, using CISS and time-of-flight MR angiography.

Presentation of 60 Cases of Infantile Spasms Based on Etiology, Clinical Manifestation EEG and Brain CT Scan in Mofid Children Hospital

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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.

[Clinical and pathogenetic features of esophageal spasm].

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Firsova, L D; Pichugina, I M; Yanova, O B; Berezina, O I; Bordin, D S

2015-01-01

To comparatively analyze clinical manifestations in patients with primary esophageal spasm (ES) and its concurrence with gastroesophageal reflux disease (GERD) and the results of their instrumental examinations and psychodiagnostic tests. A total of 104 patients with the clinical and manometric signs of ES were examined and divided into two groups: 1) 42 patients with primary ES; 2) 62 patients with ES concurrent with GERD. The examination encompassed esophageal manometry, esophagogastroduodenoscopy, 24-hour pH metry, and an interview using a questionnaire to identify autonomic disorders, and the Mini-Mult test. The patients with primary ES compared to those with ES concurrent with GERD significantly more frequently showed severe pain syndrome (p = 0.009) and a paradoxical dysphagia pattern (p = 0.03); manometry revealed an incoordination in the motility of the entire esophagus (p = 0.001). Comparison of the statistical series of values for contraction amplitude and duration in the distal esophagus found no significant difference in the patients of both groups. Autonomic disturbances were detected in 76.0% of the patients with ES; but the intergroup differences were insignificant. Mental maladaptation was observed in 81.7% of the patients in the absence of intergroup differences. The etiopathogenetic factor of ES is a psychoautonomic response to chronic stress in both primary ES and its concurrence with GERD. The reflux of gastric contents into the esophagus does not appear to be one of the leading causes of ES. In primary ES, esophageal motor function is generally impaired to a much greater extent than that in ES concurrent with GERD. The degree of motor disorders is embodied in the specific clinical features of the disease.

Stress in Parents of a Child with Hemifacial Microsomia: The Role of Child Characteristics and Parental Coping Strategies.

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Ongkosuwito, Edwin; van der Vlies, Lieneke; Kraaij, Vivian; Garnefski, Nadia; van Neck, Han; Kuijpers-Jagtman, Anne Marie; Hovius, Steven

2018-01-01

Objective Examine stress levels of parents of children with hemifacial microsomia (HFM) and the relationship of parental stress to child characteristics and cognitive coping strategies. Design Prospective cross-sectional study. Participants and Setting Parents with a child (age 3-19 years) with HFM (N = 31) were recruited through the Department of Orthodontics and the Craniofacial Center, Sophia-Erasmus Medical Center, Rotterdam, The Netherlands. Intervention and Outcome Measures The adapted and shortened Dutch version of the parental stress index (NOSI-K) was used to measure parental stress, and the cognitive emotion-regulation questionnaire was used to measure cognitive coping strategies. Pearson correlations and a multiple regression analysis were performed. Results The hierarchical multiple regression analysis showed associations between increased parental stress and learning difficulties and use of acceptance as a coping strategy. This suggests that problems other than the characteristic visual appearance of the child's face in HFM have a greater influence on parental stress. Conclusions Learning difficulties of the child with HFM and parental acceptance affect stress in parents with a child with HFM the most and are important in the search for a targeted tailoring of intervention for parents with high levels of parental stress.

The Successful Treatment of Opioid Withdrawal-Induced Refractory Muscle Spasms with 5-HTP in a Patient Intolerant to Clonidine.

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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.

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

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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

Prospective Study Evaluating IncobotulinumtoxinA for Cervical Dystonia or Blepharospasm: Interim Results from the First 145 Subjects with Cervical Dystonia

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Hubert Fernandez

2013-05-01

Full Text Available Background: We report the interim results from XCiDaBLE: A large prospective, observational "naturalistic" study evaluating Xeomin® (incobotulinumtoxinA for cervical dystonia or blepharospasm in the United States.Methods: Subjects with CD are followed for 2 treatment cycles and monitored via Interactive Voice/Web Response. Subject-reported scales include the Subject Global Impression-Severity and Improvement; Cervical Dystonia Impact Profile (CDIP-58; and Work Productivity and Quality of Life (QoL are assessed by means of an employment questionnaire and work history and the SF-12v2.Results: This ongoing study includes 145 subjects with a diagnosis of CD. The majority were female (82.3% and White (91.0% and had previously been treated with botulinum toxins (77.2%. There were 106 employed at the time of onset of the disease, but 12.6 years later only 44% were still employed at the time of enrolment into the study and 20% were either receiving or seeking disability benefits. However, only 44% were still employed at the time of recruitment for study participation. The mean total dose/treatment of CD was 225.2 units for the 1st injection. The CDIP-58 total score was significantly improved at four weeks post the first injection compared to baseline (p=<0.0001. Most subjects noted improvement in their global impression assessment. No new or unexpected adverse events occurred. Discussion: The results from these interim analyses confirm previous controlled single-dose studies of incobotulinumtoxinA in terms of efficacy and safety.

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

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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.

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease)

International Nuclear Information System (INIS)

Uki, Jiro; Kanda, Shinji; Asakura, Ken; Takeda, Fumikazu

1985-01-01

A case of dysplastic gangliocytoma of the cerebellum, or Lhermitte-Duclos disease, is reported along with its CT findings, and the cases so far reported in the literature are reviewed. This is the 50th case report since the first description in 1920. This 61-year-old female had suffered from right hemifacial spasms for more than 20 years and from bilateral tinnitus with auditory disturbances for two years. Four years before admission, she underwent gastric resection and cancer chemotherapy for gastric cancer. Plain craniograms showed a thinned and ballooned occipital squama on the right side. Vertebral angiograms revealed a large tumor stain, with early venous filling, in the right posterior fossa. A CT scan showed a large, low-density mass, with small calcified areas in it, in the right posterior fossa. A postcontrast CT scan revealed no contrast enhancement, except for dilated vascular enhancement, within the tumor. No hydrocephalus was observed. Metrizamide CT cisternography revealed a huge intraaxial mass compressing the brain stem. (J.P.N.)

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). Case report with CT findings

Energy Technology Data Exchange (ETDEWEB)

Uki, Jiro; Kanda, Shinji; Asakura, Ken; Takeda, Fumikazu

1985-08-01

A case of dysplastic gangliocytoma of the cerebellum, or Lhermitte-Duclos disease, is reported along with its CT findings, and the cases so far reported in the literature are reviewed. This is the 50th case report since the first description in 1920. This 61-year-old female had suffered from right hemifacial spasms for more than 20 years and from bilateral tinnitus with auditory disturbances for two years. Four years before admission, she underwent gastric resection and cancer chemotherapy for gastric cancer. Plain craniograms showed a thinned and ballooned occipital squama on the right side. Vertebral angiograms revealed a large tumor stain, with early venous filling, in the right posterior fossa. A CT scan showed a large, low-density mass, with small calcified areas in it, in the right posterior fossa. A postcontrast CT scan revealed no contrast enhancement, except for dilated vascular enhancement, within the tumor. No hydrocephalus was observed. Metrizamide CT cisternography revealed a huge intraaxial mass compressing the brain stem. (J.P.N.).

Combined Bimaxillary Distraction Osteogenesis Associated with Orthognathic Surgery for Hemifacial Microsomia in Adults.

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Liu, Hanghang; Zhang, Xiaohui; Liu, Li; Chen, Qianming; Shao, Jun; Luo, En

2017-06-01

Hemifacial microsomia (HFM) is one of the most common congenital craniofacial asymmetries. Distraction osteogenesis (DO) is always recommended as an effective option for the treatment of HFM, but some studies showed that the asymmetry remained after DO. In comparison with DO, stable clinical outcomes could be achieved by orthognathic surgery approach as well. However, soft tissue expansion cannot be performed. In this study, we aim to assess the radiographic and clinical results of bimaxillary DO associated with staged orthognathic surgery in the treatment of craniofacial asymmetry for adult HFM patients. Twelve HFM patients who had been treated with bimaxillary DO and secondary orthognathic surgery between 2006 and 2013 were included in this study. Bimaxillary DO and staged orthognathic surgery were performed according to the particular condition. Clinical and cephalometric photographs were carried out pre- and postoperatively to evaluate the outcomes. No significant complications occurred postoperatively. Excellent cosmetic outcomes such as notable improvement in facial pattern and occlusion were achieved. Cephalometrically, the height ratios of the affected/unaffected ramus were improved by 23.81% on average, and the photographic analysis demonstrated a rotation of the chin point toward the mid-line. Moreover, the occlusal cant was corrected from the slant to level by 8.95° on average. The application of bimaxillary DO associated with staged orthognathic surgery in correcting the asymmetry of adult patients with HFM could have satisfactory aesthetic results, and it should be considered an alternative option for these patients. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266.

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

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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.

Functional and morphological changes in the pharynx in achalasia and in diffuse esophageal spasm

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Hannig, C.; Wuttge-Hannig, A.; Amon, K.; Feussner, H.

1989-08-01

Since the pharynx and the esophagus are a functional unit, functional radiodiagnosis has to be directed at pharyngo-esophageal interaction. Among our collective of 73 patients suffering from achalasia or diffuse esophageal spasm, we were able to recognize a substantially increased incidence of morphological or functional pharyngeal disorders by means of cineradiography. The functional alterations in particular were often not revealed by conventional fluoroscopy. High-speed cineradiography, with its high temporal and spatial resolution, turned out to be a valuable tool in analysis of the origin of pharyngeal dysphagia. Manometry correlated very well with the radiologic findings in tubular esophagus, but proved unreliable in the detection of alterations of the upper esophageal sphincter region, because of problems inherent in the method. Furthermore, membranous stenosis (webs), lateral or dorsal diverticula, and asymmetry of the pharynx were observed strikingly often. (orig.).

Immune-mediated steroid-responsive epileptic spasms and epileptic encephalopathy associated with VGKC-complex antibodies.

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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, normalVGKC-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.

Evaluation of eperisone hydrochloride in the treatment of acute musculoskeletal spasm associated with low back pain: A randomized, double-blind, placebo-controlled trial

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A S Chandanwale

2011-01-01

Full Text Available Background : Eperisone hydrochloride is a centrally acting muscle relaxant inhibiting the pain reflex pathway, having a vasodilator effect. Aims : To evaluate the efficacy and tolerability of eperisone in patients with acute musculoskeletal spasm associated with low back pain. Settings and Design : Prospective, randomized, double-blind, placebo-controlled, multicentric trial conducted at five tertiary care orthopedic centers across India. Materials and Methods : It was planned to enroll 240 patients of either sex between 18-60 years with acute musculoskeletal spasm (AMSP with low back pain (LBP due to spondylosis deformans, prolapsed disc or muscle sprain. Patients with other associated unrelated spasm conditions were excluded. Assessments were done for finger-to-floor distance (FFD, lumbar pain, Lasegue′s sign, tenderness of vertebral muscles, need for rescue medication and response to therapy for efficacy and tolerability. Statistical Analysis : Parametric data were analyzed by ′t′ test and ANOVA, and non-parametric data were analyzed using Mann-Whitney ′U′ test and Kruskall-Wallis test. Proportions were compared using Fischer′s (Chi-square test. Results : Two hundred and forty patients were randomized to receive eperisone 150 mg/day in three divided doses (n=120 or placebo (n=120 for 14 days, of which 15 patients did not complete and 225 patients completed the study (eperisone, 112 and placebo, 113. Significantly greater improvement in FFD (P<0.001 from baseline on Day 14 was seen with eperisone (150.66 to 41.75 compared to placebo (138.51 to 101.60. Improvements in other parameters were greater with the eperisone group. For 89 (79.46% patients the therapy was rated as good-excellent with eperisone compared to 43 (38.05% patients with placebo. Nausea, abdominal pain, headache and dizziness were the common adverse events with both therapies. Rescue drug was needed by 40 (35.71% eperisone patients and 83 (73.45% placebo patients

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

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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...... ST-segment depression and echocardiography showed uniform hypokinesia of all left ventricular (LV) myocardial segments without signs of regional LV ballooning. Coronary angiography was normal and the patient gained full recovery after receiving treatment with heart failure medication. Interestingly...

Crotoxin in humans: analysis of the effects on extraocular and facial muscles Crotoxina em humanos: estudo da ação em músculos extraoculares e faciais

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Geraldo de Barros Ribeiro

2012-12-01

Full Text Available PURPOSE: Crotoxin is the main neurotoxin of South American rattlesnake Crotalus durissus terrificus. The neurotoxic action is characterized by a presynaptic blockade. The purpose of this research is to assess the ability of crotoxin to induce temporary paralysis of extraocular and facial muscles in humans. METHODS: Doses of crotoxin used ranged from 2 to 5 units (U, each unit corresponding to one LD50. We first applied 2U of crotoxin in one of the extraocular muscles of 3 amaurotic individuals to be submitted to ocular evisceration. In the second stage, we applied crotoxin in 12 extraocular muscles of 9 patients with strabismic amblyopia. In the last stage, crotoxin was used in the treatment of blepharospasm in another 3 patients. RESULTS: No patient showed any systemic side effect or change in vision or any eye structure problem after the procedure. The only local side effects observed were slight conjunctival hyperemia, which recovered spontaneously. In 2 patients there was no change in ocular deviation after 2U crotoxin application. Limitation of the muscle action was observed in 8 of the 12 applications. The change in ocular deviation after application of 2U of crotoxin (9 injections was in average 15.7 prism diopters (PD. When the dose was 4U (2 applications the change was in average 37.5 PD and a single application of 5U produced a change of 16 PD in ocular deviation. This effect lasted from 1 to 3 months. Two of the 3 patients with blepharospasm had the hemifacial spasm improved with crotoxin, which returned after 2 months. CONCLUSIONS: This study provides data suggesting that crotoxin may be a useful new therapeutic option for the treatment of strabismus and blepharospasm. We expect that with further studies crotoxin could be an option for many other medical areas.OBJETIVO: A crotoxina é a principal neurotoxina da cascavel sul-americana Crotalus durissus terrificus e sua ação neurotóxica caracteriza-se por um bloqueio pr

A novel ANO3 variant identified in a 53-year-old woman presenting with hyperkinetic dysarthria, blepharospasm, hyperkinesias, and complex motor tics.

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Blackburn, Patrick R; Zimmermann, Michael T; Gass, Jennifer M; Harris, Kimberly G; Cousin, Margot A; Boczek, Nicole J; Ross, Owen A; Klee, Eric W; Brazis, Paul W; Van Gerpen, Jay A; Atwal, Paldeep S

2016-12-05

Cervical dystonias have a variable presentation and underlying etiology, but collectively represent the most common form of focal dystonia. There are a number of known genetic forms of dystonia (DYT1-27); however the heterogeneity of disease presentation does not always make it easy to categorize the disease by phenotype-genotype comparison. In this report, we describe a 53-year-old female who presented initially with hand tremor following a total hip arthroplasty. The patient developed a mixed hyperkinetic disorder consisting of chorea, dystonia affecting the upper extremities, dysarthria, and blepharospasm. Whole exome sequencing of the patient revealed a novel heterozygous missense variant (Chr11(GRCh38): g.26525644C > G; NM_031418.2(ANO3): c.702C > G; NP_113606.2. p.C234W) in exon 7 in the ANO3 gene. ANO3 encodes anoctamin-3, a Ca +2 -dependent phospholipid scramblase expressed in striatal-neurons, that has been implicated in autosomal dominant craniocervical dystonia (Dystonia-24, DYT24, MIM# 615034). To date, only a handful of cases of DYT-24 have been described in the literature. The complex clinical presentation of the patient described includes hyperkinesias, complex motor movements, and vocal tics, which have not been reported in other patients with DYT24. This report highlights the utility of using clinical whole exome sequencing in patients with complex neurological phenotypes that would not normally fit a classical presentation of a defined genetic disease.

Hemimasticatory spasm treated with botulinum toxin: case report Espasmo hemimastigatório tratado com toxina botulínica: relato de caso

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Hélio A.G. Teive

2002-06-01

Full Text Available We describe a female patient with hemimasticatory spasm, a rare movement disorder due to dysfunction of the motor trigeminal nerve of unknown origin. This patient had an excellent response to botulinum toxin therapy.Relatamos o caso de paciente feminina com espasmo hemimastigatório, distúrbio do movimento raro decorrente de disfunção da porção motora do nervo trigeminal, de etiologia desconhecida. A paciente teve excelente resposta clínica ao tratamento com toxina botulínica.

Changes in ocular higher-order aberrations following botulinum toxin treatment in patients with blepharospasm : BTX improves dry eye in patients with BEB.

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Isshiki, Yoshihiko; Ishikawa, Hiroto; Mimura, Osamu

2016-11-01

To evaluate the effects of botulinum toxin type A (BTX-A) treatment in patients with benign essential blepharospasm (BEB) by monitoring the ocular surface and ocular higher-order aberrations (HOAs) before and after treatment. The present study reports a prospective case series of 38 patients (76 eyes, 11 men and 27 women; mean age 66.8 ± 9.8 years) with BEB who underwent BTX-A treatment at Kokura Memorial Hospital between 2013 and 2014. Patients were evaluated for ophthalmoscopic findings, Schirmer I test, tear film break-up time (t-BUT), HOAs, fluctuation index (FI), stability index (SI) using a wavefront aberrometer, 15 subjective symptoms using the Dry Eye-Related Quality of Life Score (DEQS), and complications before and after the treatment. After BTX-A treatment, the Schirmer I test score improved significantly from 5.9 ± 5.4 to 8.7 ± 6.1 mm and t-BUT recovered from 5.2 ± 1.7 to 7.3 ± 1.7 s. HOAs were classified into four patterns: stable (60.5 %), small fluctuation (14.5 %), sawtooth (17.1 %), and reverse sawtooth (7.9 %), and they significantly reduced after the treatment. Only FI (not SI) showed a marked reduction, and the DEQS significantly improved from 44.7 ± 21.6 to 37.6 ± 21.0 after the treatment (p treatment with a high potential to improve ocular surface disorders induced by BEB.

Cleft characteristics and treatment outcomes in hemifacial microsomia compared to non-syndromic cleft lip/palate.

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Dentino, K M; Valstar, A; Padwa, B L

2016-06-01

The goal of this study was to describe the clinical characteristics and treatment outcomes of patients with hemifacial microsomia (HFM) and cleft lip/palate (CL/P), and to compare them to a historic cohort of patients with non-syndromic CL/P treated at the same centre. A retrospective review of patients with HFM and CL/P was performed; the main outcome measures assessed were cleft type/side, surgical outcome, midfacial retrusion, and speech. Twenty-six patients (13 male, 13 female; mean age 22.7±14.9, range 1-52 years) with cleft lip with/without cleft palate (CL±P) were identified: three with cleft lip (12%), two with cleft lip and alveolus and an intact secondary palate (8%), and 21 with cleft lip and palate (CLP) (81%; 15 unilateral and six bilateral). Four patients (19%) had a palatal fistula after palatoplasty. Twelve of 22 patients aged >5 years (55%) had midfacial retrusion and two (9%) required a pharyngeal flap for velopharyngeal insufficiency (VPI). Fisher's exact test demonstrated a higher frequency of complete labial clefting (P=0.004), CLP (P=0.009), midfacial retrusion (P=0.0009), and postoperative palatal fistula (P=0.03) in HFM compared to non-syndromic CL±P. There was no difference in VPI prevalence. This study revealed that patients with HFM and CL±P have more severe forms of orofacial clefting than patients with non-syndromic CL±P. Patients with HFM and CL±P have more severe midfacial retrusion and a higher palatal fistula rate compared to patients with non-syndromic CL±P. Copyright © 2015 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Botulinum Toxin as an Alternative to Treat the Spasm of the Near Reflex.

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Laria, Carlos; Merino-Suárez, María L; Piñero, David P; Gómez-Hurtado, Arantxa; Pérez-Cambrodí, Rafael J

2015-01-01

We describe the case of an eight-year-old girl with complaints of headaches and blurred vision (uncorrected visual acuity: 0.1 decimal) that showed on examination miotic pupils, pseudomyopia, no ocular motility restrictions, and no associated neurological disease. After initial treatment with cyclopentolate for two months, pseudomyopia persisted with an intermittent and variable esotropia. Spectacles of +1 both eyes and atropine 1% one drop daily were then prescribed. The situation improved and remained stable for several weeks, with pseudomyopia and esotropia reappearing later. Finally, botulinum toxin (2.5 iu Botox) was injected in the medial rectus muscle on two occasions and a visual therapy program based on the stimulation of fusional divergence, diplopia, and stereopsis consciousness was recommended. This prescription was combined with the use of atropine during the first few weeks. Orthotropia and corrected distance visual acuity of 1.0 were found three months after treatment. The evolution and clinical results of this case report suggest that botulinum toxin in combination with other therapeutic alternatives may be useful in the treatment of spasm of the near reflex.

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

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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.

[Evidence of facial palsy and facial malformations in pottery from Peruvian Moche and Lambayeque pre-Columbian cultures].

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Carod-Artal, F J; Vázquez Cabrera, C B

2006-01-01

Moche (100-700 AD) and Lambayeque-Sicán (750-1100 AD) are pre-Columbian cultures from Regional States Period, developed in Northern Peru. Information about daily life, religion and medicine has been obtained through the study of Moche ceramics found in lords and priests tombs, pyramids and temples. To analyze archeological evidences of Moche Medicine and neurological diseases through ceramics. Representations of diseases in Moche and Lambayeque iconography and Moche pottery collections exposed in Casinelli museum from Trujillo, and Brüning National Archeological museum from Lambayeque, Peru, were studied. The most representative cases were analyzed and photographed, previous authorization from authorities and curators of the museums. The following pathologies were observed in ceramic collections: peripheral facial palsy, facial malformations such as cleft lip, hemifacial spasm, legs and arm amputations, scoliosis and Siamese patients. Male and females Moche doctors were also observed in the ceramics in ritual ceremonies treating patients. The main pathologies observed in Moche and Lambayeque pottery are facial palsy and cleft lip. These are one of the earliest registries of these pathologies in pre-Columbian cultures in South-America.

Harmonic scalpel versus electrocautery for harvest of radial artery conduits: Reduced risk of spasm and intimal injury on optical coherence tomography

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Brazio, Philip S.; Laird, Patrick C.; Xu, Chenyang; Gu, Junyan; Burris, Nicholas S.; Brown, Emile N.; Kon, Zachary N.; Poston, Robert S.

2009-01-01

Objective Vasospasm is the primary obstacle to widespread adoption of the radial artery as a conduit in coronary artery bypass grafting. We used optical coherence tomography, a catheter-based intravascular imaging modality, to measure the degree of radial artery spasm induced by means of harvest with electrocautery or a harmonic scalpel in patients undergoing coronary artery bypass grafting. Methods Radial arteries were harvested from 44 consecutive patients with a harmonic scalpel (n = 15) or electrocautery (n = 29). Vessels were imaged before harvesting and after removal from the arm, with saphenous vein tracts serving as internal controls. Optical coherence tomographic findings for the degree of harvesting-induced injury were validated against histologic measures. Results Optical coherence tomographic measures of endovascular dimensions and injury correlated strongly with histologic findings. Mean luminal volume, a measure of vasospasm, decreased significantly less after harvesting with a harmonic scalpel (9% ± 7%) than with electrocautery (35% ± 6%, P = .015). Completely intact intima was present in 11 (73%) of 15 radial arteries harvested with a harmonic scalpel (73%) compared with 9 of 29 arteries harvested by means of electrocautery (31%, P = .011). Intraoperative flow measurements and patency rates at 5 days postoperatively were not significantly different among groups. Conclusions Optical coherence tomography provides a level of speed and accuracy for quantifying endothelial injury and vasospasm that has not been described for any other modality, suggesting potential as an intraoperative quality assurance tool. Our optical coherence tomographic findings suggest that the harmonic scalpel induces less spasm and intimal injury compared with electrocautery. PMID:19026820

Association of Live Donor Nephrectomy and Reversal of Renal Artery Spasm

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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.

Prefabricated Cervical Skin Flaps for Hemi-Facial Resurfacing: Elucidating the Natural History of Postoperative Edema Using Indocyanine Green.

Science.gov (United States)

Li, Ke; Min, Peiru; Sadigh, Parviz; Grassetti, Luca; Lazzeri, Davide; Torresetti, Matteo; Marsili, Riccardo; Feng, Shaoqing; Liu, Ningfei; Zhang, Yi Xin

2018-02-01

The increases in capillary wall permeability and capillary hydrostatic pressure are considered to be the causes for the acute swelling seen in flaps; however, disruption of the circulating flap lymphatics could be another contributory factor. In this study we monitor the development of flap edema in a series of 18 prefabricated flaps and aim to delineate the natural history of this phenomenon by use of lymphography. Postoperative swelling was monitored in a series of 18 pre-expanded prefabricated cervical skin flaps used for hemi-facial burns-scar resurfacing. Time to spontaneous resolution, presence or absence of venous congestion, and clinical outcome were recorded. In two cases, indocyanine-green (ICG) lymphography was used to monitor the dermal backflow pattern until swelling had completely resolved. Average moving velocity of ICG after injection as well as flap thickness was also recorded over the follow-up period. The average moving velocity of ICG in the flap lymphatics improved from 0.48 cm/min to 1.5 cm/min in the first 12 days after flap transfer. The dermal backflow pattern was stardust in the first 12 days, indicating moderate lymphedema, transforming to splash from week three, and a robust collecting lymphatic vessel occurring from the fifth month, indicating mild lymphedema and lymphatic channel recovery, respectively. Transient swelling was observed in all prefabricated flaps in our series. We postulate that this is mostly secondary to lymphatic disruption that subsides as lymphangiogenesis takes place. ICG lymphography is an inexpensive, safe, and easy-to-use imaging technology that could be used in the monitoring of postoperative lymphedema seen in prefabricated flaps.

Virtual MRI endoscopy of the intracranial cerebrospinal fluid spaces

International Nuclear Information System (INIS)

Shigematsu, Y.; Korogi, Y.; Hirai, T.; Okuda, T.; Ikushima, I.; Sugahara, T.; Liang, L.; Ge, Y.; Takahashi, M.

1998-01-01

We used constructive interference in steady state (CISS) 3D Fourier transform (3DFT) MRI data sets to obtain three-dimensional (3D) virtual MRI endoscopic views of the intracranial cerebrospinal fluid (CSF) spaces, processing them with a commercially available perspective endoscopic algorithm. We investigated the potential of the intracranial virtual MRI endoscopy applied to visualisation of the pathology in 13 patients with surgically confirmed trigeminal neuralgia (3), hemifacial spasm (3), acoustic neuroma (3), suprasellar germinoma (1), Langerhans cell histiocytosis (1), lateral ventricle nodules (1) and pituitary dwarfism (1). All images were acquired using a 1.5-T imager employing a circular polarised head coil. The CISS-3DFT data sets were transferred to a workstation for processing with the perspective endoscopic algorithm. Postprocessing for virtual MRI endoscopy was possible for all data sets. The lesions in 12 patients, and their complex anatomical relationships with the surrounding structures, were well seen on the 3D images. A small acoustic neuroma in the internal auditory meatus was not seen using virtual endoscopy. Although virtual MRI endoscopy has limitations, it provides 3D images which cannot be acquired using any other procedure. (orig.)

Virtual endoscopic images by 3D FASE cisternography for neurovascular compression

International Nuclear Information System (INIS)

Ishimori, Takashi; Nakano, Satoru; Kagawa, Masahiro

2003-01-01

Three-dimensional fast asymmetric spin echo (3D FASE) cisternography provides high spatial resolution and excellent contrast as a water image acquisition technique. It is also useful for the evaluation of various anatomical regions. This study investigated the usefulness and limitations of virtual endoscopic images obtained by 3D FASE MR cisternography in the preoperative evaluation of patients with neurovascular compression. The study included 12 patients with neurovascular compression: 10 with hemifacial spasm and two with trigeminal neuralgia. The diagnosis was surgically confirmed in all patients. The virtual endoscopic images obtained were judged to be of acceptable quality for interpretation in all cases. The areas of compression identified in preoperative diagnosis with virtual endoscopic images showed good agreement with those observed from surgery, except in one case in which the common trunk of the anterior inferior cerebellar artery and posterior inferior cerebellar artery (AICA-PICA) bifurcated near the root exit zone of the facial nerve. The veins are displayed in some cases but not in others. The main advantage of generating virtual endoscopic images is that such images can be used for surgical simulation, allowing the neurosurgeon to perform surgical procedures with greater confidence. (author)

Single Neurosurgeon Operative Experience at Craig Joint Theater Hospital During the Afghanistan Surge (November 2010 to April 2011), Part II: Humanitarian Cases.

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Steele, John Joseph

2017-01-01

The Afghanistan Surge saw NATO troops working with their Afghan partners to remove Taliban governance and replace it with a more democratic model. As part of this endeavor, medical support for both trauma and humanitarian cases was needed. Identify and discuss disease trends to better prepare for future combat medical treatments. Retrospective review of operative experience from a neurosurgeon from November 2010 to April 2011. 63 cases were performed on 20 NATO and 43 Afghan patients. Combat-related neurotrauma represented 73% (46/63) of cases and humanitarian cases represented the remainder. The most common diseases among humanitarian cases were benign tumors (29%, 5/17), cranioplasty (23%, 4/17), obstructive hydrocephalus (11%, 2/17), nonobstructive hydrocephalus (11%, 2/17), hemifacial spasm (11%, 2/17), and cerebral angiography (11%, 2/17). There was 1 death from ventriculitis for a complication rate of 6%. In select well-nourished, patients with minimal risk of needing tracheostomy, humanitarian neurosurgery can be safely performed in theater with a complication rate (6%) no worse than patients operated on in the United States. Reprint & Copyright © 2017 Association of Military Surgeons of the U.S.

Botulinum toxin

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Nigam P

2010-01-01

Full Text Available Botulinum toxin, one of the most poisonous biological substances known, is a neurotoxin produced by the bacterium Clostridium botulinum. C. botulinum elaborates eight antigenically distinguishable exotoxins (A, B, C 1 , C 2 , D, E, F and G. All serotypes interfere with neural transmission by blocking the release of acetylcholine, the principal neurotransmitter at the neuromuscular junction, causing muscle paralysis. The weakness induced by injection with botulinum toxin A usually lasts about three months. Botulinum toxins now play a very significant role in the management of a wide variety of medical conditions, especially strabismus and focal dystonias, hemifacial spasm, and various spastic movement disorders, headaches, hypersalivation, hyperhidrosis, and some chronic conditions that respond only partially to medical treatment. The list of possible new indications is rapidly expanding. The cosmetological applications include correction of lines, creases and wrinkling all over the face, chin, neck, and chest to dermatological applications such as hyperhidrosis. Injections with botulinum toxin are generally well tolerated and side effects are few. A precise knowledge and understanding of the functional anatomy of the mimetic muscles is absolutely necessary to correctly use botulinum toxins in clinical practice.

Virtual MRI endoscopy of the intracranial cerebrospinal fluid spaces

Energy Technology Data Exchange (ETDEWEB)

Shigematsu, Y.; Korogi, Y.; Hirai, T. [Kumamoto Univ. (Japan). Dept. of Radiology; Okuda, T.; Ikushima, I.; Sugahara, T.; Liang, L.; Ge, Y.; Takahashi, M.

1998-10-01

We used constructive interference in steady state (CISS) 3D Fourier transform (3DFT) MRI data sets to obtain three-dimensional (3D) virtual MRI endoscopic views of the intracranial cerebrospinal fluid (CSF) spaces, processing them with a commercially available perspective endoscopic algorithm. We investigated the potential of the intracranial virtual MRI endoscopy applied to visualisation of the pathology in 13 patients with surgically confirmed trigeminal neuralgia (3), hemifacial spasm (3), acoustic neuroma (3), suprasellar germinoma (1), Langerhans cell histiocytosis (1), lateral ventricle nodules (1) and pituitary dwarfism (1). All images were acquired using a 1.5-T imager employing a circular polarised head coil. The CISS-3DFT data sets were transferred to a workstation for processing with the perspective endoscopic algorithm. Postprocessing for virtual MRI endoscopy was possible for all data sets. The lesions in 12 patients, and their complex anatomical relationships with the surrounding structures, were well seen on the 3D images. A small acoustic neuroma in the internal auditory meatus was not seen using virtual endoscopy. Although virtual MRI endoscopy has limitations, it provides 3D images which cannot be acquired using any other procedure. (orig.) With 6 figs., 16 refs.

Clinical application of human adipose tissue-derived mesenchymal stem cells in progressive hemifacial atrophy (Parry-Romberg disease) with microfat grafting techniques using 3-dimensional computed tomography and 3-dimensional camera.

Science.gov (United States)

Koh, Kyung Suk; Oh, Tae Suk; Kim, Hoon; Chung, In Wook; Lee, Kang Woo; Lee, Hyo Bo; Park, Eun Jung; Jung, Jae Seob; Shin, Il Seob; Ra, Jeong Chan; Choi, Jong Woo

2012-09-01

Parry-Romberg disease is a rare condition that results in progressive hemifacial atrophy, involving the skin, dermis, subcutaneous fat, muscle, and, finally, cartilage and bone. Patients have been treated with dermofat or fat grafts or by microvascular free flap transfer. We hypothesized that adipose-derived stem cells (ASCs) may improve the results of microfat grafting through enhancing angiogenesis. We evaluated the utility of ASC in microfat grafting of patients with Parry-Romberg disease by measuring the change in the hemifacial volumes after injection of ASCs with microfat grafts or microfat grafts alone. In April 2008, this investigation was approved by the Korean Food and Drug Administration and the institutional review board of the Asan Medical Center (Seoul, Korea) that monitor investigator-initiated trials. Between May 2008 and January 2009, 10 volunteers with Parry-Romberg disease (5 men and 5 women; mean age, 28 y) were recruited; 5 received ASC and microfat grafts and 5 received microfat grafts only. The mean follow-up period was 15 months. Adipose-derived stem cells were obtained from abdominal fat by liposuction and were cultured for 2 weeks. On day 14, patients were injected with fat grafts alone or plus (in the test group) 1 × 10 ASCs. Patients were evaluated postoperatively using a 3-dimensional camera and 3-dimensional CT scans, and grafted fat volumes were objectively calculated. Successful outcomes were evident in all 5 patients receiving microfat grafts and ASCs, and the survival of grafted fat was better than in patients receiving microfat grafts alone. Before surgery, the mean difference between ipsilateral and contralateral hemiface volume in patients receiving microfat grafts and ASCs was 21.71 mL decreasing to 4.47 mL after surgery. Overall resorption in this ASC group was 20.59%. The mean preoperative difference in hemiface volume in those receiving microfat grafts alone was 8.32 mL decreasing to 3.89 mL after surgery. Overall

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.)

Facts about Blepharospasm

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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

2016-10-19

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.

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

A novel missense mutation in the NDP gene in a child with Norrie disease and severe neurological involvement including infantile spasms.

Science.gov (United States)

Lev, Dorit; Weigl, Yuval; Hasan, Mariana; Gak, Eva; Davidovich, Michael; Vinkler, Chana; Leshinsky-Silver, Esther; Lerman-Sagie, Tally; Watemberg, Nathan

2007-05-01

Norrie disease (ND) is a rare X-linked recessive disorder characterized by congenital blindness and in some cases, mental retardation and deafness. Other neurological complications, particularly epilepsy, are rare. We report on a novel mutation identified in a patient with ND and profound mental retardation. The patient was diagnosed at the age of 6 months due to congenital blindness. At the age of 8 months he developed infantile spasms, which were diagnosed at 11 months as his EEG demonstrated hypsarrhythmia. Mutation analysis of the ND gene (NDP) of the affected child and his mother revealed a novel missense mutation at position c.134T > A resulting in amino acid change at codon V45E. To the best of our knowledge, such severe neurological involvement has not been previously reported in ND patients. The severity of the phenotype may suggest the functional importance of this site of the NDP gene.

Descompresión microvascular en espasmo hemifacial: Reporte de 13 casos y revisión de la literatura

Science.gov (United States)

Campero, Alvaro; Herreros, Isabel Cuervo-Arango; Barrenechea, Ignacio; Andjel, Germán; Ajler, Pablo; Rhoton, Albert

2016-01-01

Objetivo: El propósito del presente trabajo es presentar los resultados de 13 pacientes con diagnóstico de espasmo hemifacial (EHF), en los cuales se realizó una descompresión microvascular (DMV). Material y Método: Desde Junio de 2005 a Mayo de 2014, 13 pacientes con diagnóstico de EHF fueron intervenidos quirúrgicamente, realizando una DMV. Se evaluó: edad, sexo, tiempo de evolución de la sintomatología, hallazgos intraoperatorios y resultados postoperatorios. Resultados: De los 13 pacientes intervenidos, 7 fueron mujeres y 6 varones. La media de edad fue de 53 años. El tiempo medio entre el inicio de la sintomatología y la intervención quirúrgica osciló entre 3 y 9 años. En todos los casos el EHF era típico, uno de ellos con neuralgia trigeminal concomitante, observándose en todos compresión neurovascular intraoperatoria. Por orden decreciente de frecuencia la causa de la compresión fue arteria cerebelosa anteroinferior, arteria cerebelosa posteroinferior, arteria dolicomega basilar y arteria dolicomega vertebral. El seguimiento postoperatorio fue en promedio de 24 meses. El 62% presentó desaparición postquirúrgica inmediata de la sintomatología preoperatoria, el 30% desaparición tras un período de 3 semanas a 2 meses (8% con mejoría parcial), y en el 8% no hubo mejoría. En cuanto a las complicaciones postoperatorias: 3 pacientes presentaron paresia facial II-III en la escala de House-Brackman (se recuperaron en un período de 6 meses), y 1 paciente presentó fístula de líquido cefalorraquídeo. Ninguno de los pacientes de la serie presentaron hipoacusia transitorio o permanente. Conclusión: La DMV como tratamiento del EHF es un procedimiento efectivo y seguro, que permite la resolución completa de la patología en la mayoría de los casos. PMID:27127708

Exploratory structural assessment in craniocervical dystonia: Global and differential analyses.

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Larissa Vilany

Full Text Available Our goal was to investigate the cortical thickness and subcortical volume in subjects with craniocervical dystonia and its subgroups.We studied 49 subjects, 17 with cervical dystonia, 18 with blepharospasm or oromandibular dystonia, and 79 healthy controls. We performed a whole group analysis, followed by a subgroup analysis. We used Freesurfer software to measure cortical thickness, subcortical volume and to perform a primary exploratory analysis in the craniocervical dystonia group, complemented by a region of interest analysis. We also performed a secondary analysis, with data generated from Freesurfer for subgroups, corrected by false discovery rate. We then performed an exploratory generalized linear model with significant areas for the previous steps using clinical features as independent variables.The primary exploratory analysis demonstrated atrophy in visual processing regions in craniocervical dystonia. The secondary analysis demonstrated atrophy in motor, sensory, and visual regions in blepharospasm or oromandibular dystonia, as well as in limbic regions in cervical dystonia. Cervical dystonia patients also had greater cortical thickness than blepharospasm or oromandibular dystonia patients in frontal pole and medial orbitofrontal regions. Finally, we observed an association between precuneus, age of onset of dystonia and age at the MRI exam, in craniocervical dystonia; between motor and limbic regions and age at the exam, clinical score and time on botulinum toxin in cervical dystonia and sensory regions and age of onset and time on botulinum toxin in blepharospasm or oromandibular dystonia.We detected involvement of visual processing regions in craniocervical dystonia, and a pattern of involvement in cervical dystonia and blepharospasm or oromandibular dystonia, including motor, sensory and limbic areas. We also showed an association of cortical thickness atrophy and younger onset age, older age at the MRI exam, higher clinical

Microvascular decompression in treating cranial nerve diseases%微血管减压术治疗颅神经疾病疗效分析

Institute of Scientific and Technical Information of China (English)

张志强; 黄涛; 罗小川; 谢才军; 谢绍盈; 沈有碧; 隋立森; 韩富

2008-01-01

Objective To study the efficacy of microvascular decompression in treating cranial nerve diseases. Methods 156 patients were treated with microvaseular decompression,of whom 119 were with trigeminal neuralgia,34 with hemifacial spasm and three with glossopharyngeal neuralgia.Rusults The overall effective rate was 96.8%(151/156) and the corresponding effective rate for the above three conditions were 94.2%,97.1% and 66.7%. Conlusions Mierovaseular decompression iS an effective treatment for cranial nerve diseases.%目的 探讨微血管减压术治疗三叉神经痛、面肌痉挛、舌咽神经痛的疗效. 方法 微血管减压手术治疗颅神经疾病156例(三叉神经痛119例,面肌痉挛34例,舌咽神经痛3例). 结果 总有效151例,有效率96.8%,其中三又神经痛、面肌痉挛和舌咽神经痛患者手术有效率分别为94.2%、97.1%和66.7%. 结论 微血管减压术治疗颅神经疾病疗效确切.

Oromandibular Dystonia—Meige′s Syndrome: Report of a Rare Case with Review

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Siddharth Gupta

2010-01-01

Full Text Available Meige′s syndrome is a combination of two forma of dystonia; blepharospasm and oromandibular dystonia (OMD. Oromandibular dystonia (OMD is a form of focal dystonia affecting head and neck region, including the lower face, jaw, tongue and larynx. It may be a factor contributing to muscle stiffness, degenerative changes in temporomandibular joint, mucosal lesions, damage to teeth, and dental prosthesis. We take this opportunity to present a patient with oromandibular dystonia associated with blepharospasm and spasmodic dysphonia.

Painful tonic spasm in neuromyelitis optica spectrum disorders: Prevalence, clinical implications and treatment options.

Science.gov (United States)

Liu, Ju; Zhang, Qin; Lian, Zhiyun; Chen, Hongxi; Shi, Ziyan; Feng, Huiru; Miao, Xiaohui; Du, Qin; Zhou, Hongyu

2017-10-01

Painful tonic spasm (PTS) is a common symptom in patients with neuromyelitis optica spectrum disorders (NMOSD). This study aimed to obtain further insights into the prevalence, characteristics, and treatment of PTS in patients with NMOSD, and to systematically investigate and compare the clinical features and prognosis of NMOSD with and without PTS. We reviewed the medical records and prospectively interviewed patients with NMOSD who attended the West China Hospital of Sichuan University in Chengdu, China between September 2014 and December 2016. In total, 52 of the 230 patients with NMOSD experienced PTS (22.61%). Patients with NMOSD and PTS were characterized by a higher age at onset (P = 0.017), higher annual relapse rate (ARR) (P = 0.003), higher ARR of myelitis (P = 0.011), and a tendency to experience pruritus (P = 0.025). Sodium channel blocking antiepileptic drugs (carbamazepine or oxcarbazepine) had higher efficacy than gabapentin in the treatment of PTS (P = 0.001). Although the progression index was higher in patients with PTS, this difference did not reach statistical significance (P = 0.05). Our study suggested that immunosuppressors for the prevention of relapse should be administered without delay in patients with NMOSD and PTS. Owing to the side effects of carbamazepine, we recommend oxcarbazepine as the first-line of treatment for PTS in patients with NMOSD. Whether PTS is a marker of disease severity in NMOSD remains to be determined, requiring a long-term prospective observational study. Copyright © 2017 Elsevier B.V. All rights reserved.

The importance of the time of digitalization for the incidence of spasms evoked by ouabain in strips of human saphenous vein.

Science.gov (United States)

Zerkowski, H R; Wagner, J

1982-10-01

The extent of contracture induced by ouabain on preparations of the greater saphenous vein obtained from patients undergoing elective coronary bypass surgery was investigated. The medical pretreatment of the various donor patients was similar but differed with regard to the duration of preoperative digitalization ranging from several days to months. Whereas the maximal contraction induced by noradrenaline was not influenced by prior digitalization, the contracture evoked by ouabain showed a strong dependency on the duration of preoperative digitalization. In patients without or with only short-term preoperative digitalization the spasm exerted by ouabain amounted to 48.8% and 49.2%, respectively, of the maximal contraction induced by noradrenaline, and decreased to zero in patients with long-term digitalization. From this result it is concluded that, in patients after coronary artery bypass grafting who did not receive cardiac glycosides for long-term treatment, the acute administration of glycosides may be a mechanism responsible for the early occlusion of saphenous vein bypass grafts.

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

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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

Advanced and standardized evaluation of neurovascular compression syndromes

Science.gov (United States)

Hastreiter, Peter; Vega Higuera, Fernando; Tomandl, Bernd; Fahlbusch, Rudolf; Naraghi, Ramin

2004-05-01

Caused by a contact between vascular structures and the root entry or exit zone of cranial nerves neurovascular compression syndromes are combined with different neurological diseases (trigeminal neurolagia, hemifacial spasm, vertigo, glossopharyngeal neuralgia) and show a relation with essential arterial hypertension. As presented previously, the semi-automatic segmentation and 3D visualization of strongly T2 weighted MR volumes has proven to be an effective strategy for a better spatial understanding prior to operative microvascular decompression. After explicit segmentation of coarse structures, the tiny target nerves and vessels contained in the area of cerebrospinal fluid are segmented implicitly using direct volume rendering. However, based on this strategy the delineation of vessels in the vicinity of the brainstem and those at the border of the segmented CSF subvolume are critical. Therefore, we suggest registration with MR angiography and introduce consecutive fusion after semi-automatic labeling of the vascular information. Additionally, we present an approach of automatic 3D visualization and video generation based on predefined flight paths. Thereby, a standardized evaluation of the fused image data is supported and the visualization results are optimally prepared for intraoperative application. Overall, our new strategy contributes to a significantly improved 3D representation and evaluation of vascular compression syndromes. Its value for diagnosis and surgery is demonstrated with various clinical examples.

Hemifacial Microsomia

African Journals Online (AJOL)

GB

2017-01-01

Jan 1, 2017 ... Goldenhar syndrome is a variant of HFM, which includes vertebral anomalies and ... maxillary teeth and carious left central and lateral incisors. Figure 3: ... condition and proposed treatment were explained to the patient and ...

Characterization of basal hepatic bile flow and the effects of intravenous cholecystokinin on the liver, sphincter, and gallbladder in patients with sphincter of Oddi spasm.

Science.gov (United States)

Krishnamurthy, Gerbail T; Krishnamurthy, Shakuntala; Watson, Randy D

2004-01-01

The major objectives of this project were to establish the pattern of basal hepatic bile flow and the effects of intravenous administration of cholecystokinin on the liver, sphincter of Oddi, and gallbladder, and to identify reliable parameters for the diagnosis of sphincter of Oddi spasm (SOS). Eight women with clinically suspected sphincter of Oddi spasm (SOS group), ten control subjects (control group), and ten patients who had recently received an opioid (opioid group) were selected for quantitative cholescintigraphy with cholecystokinin. Each patient was studied with 111-185 MBq (3-5 mCi) technetium-99m mebrofenin after 6-8 h of fasting. Hepatic phase images were obtained for 60 min, followed by gallbladder phase images for 30 min. During the gallbladder phase, 10 ng/kg octapeptide of cholecystokinin (CCK-8) was infused over 3 min through an infusion pump. Hepatic extraction fraction, excretion half-time, basal hepatic bile flow into the gallbladder, gallbladder ejection fraction, and post-CCK-8 paradoxical filling (>30% of basal counts) were identified. Seven of the patients with SOS were treated with antispasmodics (calcium channel blockers), and one underwent endoscopic sphincterotomy. Mean (+/-SD) hepatic bile entry into the gallbladder (versus GI tract) was widely variable: it was lower in SOS patients (32%+/-31%) than in controls (61%+/-36%) and the opioid group (61%+/-25%), but the difference was not statistically significant. Hepatic extraction fraction, excretion half-time, and pattern of bile flow through both intrahepatic and extrahepatic ducts were normal in all three groups. Gallbladder mean ejection fraction was 9%+/-4% in the opioid group; this was significantly lower (Pgallbladder refluxed into intrahepatic ducts; it reentered the gallbladder after cessation of CCK-8 infusion (paradoxical gallbladder filling) in all eight patients with SOS, but in none of the patients in the other two groups. Mean paradoxical filling was 204% (+/-193%) in the

Videonystagmography to assess eyelid dynamic disorders.

Science.gov (United States)

Casse, Guillaume; Adenis, Jean-Paul; Sauvage, Jean-Pierre; Robert, Pierre-Yves

2009-01-01

Apply a videonystagmography (VNG) device to study indirectly blinking in patients with essential blepharospasm. A VNG device was used to study the pupillary occlusion. It was recorded and analyzed as indirect parameters of blinking in 23 essential blepharospasm on day 0, month 1, and month 3 following botulinum toxin injection using VNG. Botulinum toxin significantly reduced pupillary occlusions (PO) frequency (9.6 PO/min on D0 and 4.7 PO/min on M1 (p = 0.004), and average PO time (0.95 sec on D0 and 0.58 sec on M1 (p = 0.03) for PO higher than 0.3 sec. On the other hand, it does not have any action on frequency (32.4 PO/min on D0 and 31.3 PO/min on M1 (p = 0.7) and average PO time (0.16 sec on D0 and 0.15 sec on M1 (p = 0.4) of PO lower than 0.3 sec. Video acquisition computer-assisted makes it possible to measure blinking parameters indirectly in real time and to assess essential blepharospasm objectively. The protocol analysis of PO is attractive for indirect analysis of blinking.

Clinical and pharmacological properties of incobotulinumtoxinA and its use in neurological disorders

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Jost WH

2015-04-01

Full Text Available Wolfgang H Jost,1 Reiner Benecke,2 Dieter Hauschke,3 Joseph Jankovic,4 Petr Kaňovský,5 Peter Roggenkämper,6 David M Simpson,7 Cynthia L Comella81Department of Neurology, University of Freiburg, Freiburg, Germany; 2Clinic and Policlinic for Neurology, University of Rostock, Rostock, Germany; 3Institute of Medical Biometry and Medical Informatics, University of Freiburg, Freiburg, Germany; 4Department of Neurology, Baylor College of Medicine, Houston, TX, USA; 5Department of Neurology, Palacky University Olomouc, Faculty of Medicine and Dentistry and University Hospital, Olomouc, Czech Republic; 6University Eye Clinic of Bonn, Bonn, Germany; 7Icahn School of Medicine at Mount Sinai, New York, NY, USA; 8Rush University Medical Center, Chicago, IL, USABackground: IncobotulinumtoxinA (Xeomin® is a purified botulinum neurotoxin type A formulation, free from complexing proteins, with proven efficacy and good tolerability for the treatment of neurological conditions such as blepharospasm, cervical dystonia (CD, and post-stroke spasticity of the upper limb. This article provides a comprehensive overview of incobotulinumtoxinA based on randomized controlled trials and prospective clinical studies.Summary: IncobotulinumtoxinA provides clinical efficacy in treating blepharospasm, CD, and upper-limb post-stroke spasticity based on randomized, double-blind, placebo-controlled trials with open-label extension periods (total study duration up to 89 weeks. Adverse events were generally mild or moderate. The most frequent adverse events, probably related to the injections, included eyelid ptosis and dry eye in the treatment of blepharospasm, dysphagia, neck pain, and muscular weakness in patients with CD, and injection site pain and muscular weakness when used for treating spasticity. In blepharospasm and CD, incobotulinumtoxinA was investigated in clinical trials permitting flexible intertreatment intervals based on the individual patient’s clinical need

Tratamento do laringoespasmo em anestesia pediátrica por digitopressão retroauricular: relato de casos Tratamiento del laringoespasmo en anestesia pediátrica por digitopresión retroauricular: relato de casos Treatment of laryngeal spasm in pediatric anesthesia by retroauricular digital pressure: case report

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Raquel Reis Soares

2008-12-01

digitopresión de punto localizado detrás del lóbulo de la oreja. La técnica es fácil, antigua, pero poco divulgada. Puede ser utilizada de forma segura y rápida sin necesidad del acceso venoso periférico que, en algunas situaciones, puede estar ausente. RELATO DE LOS CASOS: Dos casos de anestesia pediátrica en pacientes de tres años y de seis meses de edad, en los cuales ocurrió laringoespasmo. Los dos fueron tratados apenas con la digitopresión de la depresión retroauricular y evolucionaron con una rápida mejora del estándar respiratorio y de la saturación arterial de oxígeno. Como el laringoespasmo es una complicación común y potencialmente grave por su morbimortalidad, se hace necesario un tratamiento seguro, eficaz y rápido. CONCLUSIÓN: El tratamiento clásico del laringoespasmo es la administración de oxígeno a 100% con presión positiva por unidad ventilatoria (globo y máscara y si no hay respuesta, administración venosa de 0,25 a 1 mg.kg-1 de succinilcolina. La técnica presentada para el tratamiento del laringoespasmo es fácil, segura y eficaz, realizada con digitopresión bilateral de la región localizada detrás del lóbulo de las orejas. El laringoespasmo cedió en pocos segundos y los pacientes tuvieron una evolución favorable.BACKGROUND AND OBJECTIVES: Problems with pediatric airways are among the greatest challenges an anesthesiologist can face. Laryngeal spasm, which is twice or three times more frequent in the pediatric population, is paramount. The objective of this work was to report the treatment of laryngeal spasm applying digital pressure on a specific area behind the ear lobe. The technique is old, easy, but little known. It is safe and can be promptly done, and does not require peripheral venous access, which might not be present in some situations. CASE REPORT: We report two cases of pediatric anesthesia, in a three-year old and six month-old patients, who developed laryngeal spasm. Both patients were treated by applying

Espasmos em flexão: estudo clínico e eletrencefalográfico de pacientes tratados com Ro 4-5360 Infantile spasms: clinical and electroencephalographic studies of patients treated with Mogadon (Ro 4-5360

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P. Lison

1967-06-01

anomalias focais, apontando para possível "ponto de impacto" inicial da agressão cerebral, de acordo com o conceito de Vallat e Lepetit.Seven patients with infantile spasms, not controlled by the use of anticonvulsant drugs, were treated with Mogadon, 5 to 10 mg daily. They were studied from the clinical and electroencephalographic points of view. The authors concluded: 1. There was marked decrease on the intensity and number of groups of infantile spasms in all cases treated with Mogadon. 2. The best results were obtained in those children who had normal psychomotor development until the onset of infantile spasms. 3. The interval between the onset of infantile spasms and the treatment with Mogadon, and the previous unsuccessfull treatment with ACTH, did not interfere with the results obtained in our patients. 4. In 5 out of 7 patients there was slight improvement of the mental status, although not marked as the decrease of seizures. This improvement was more evident in those children who had previous normal psychomotor development. 5. In those cases who had hypsarhythmia the EEG changes disappeared at the end of the first week of treatment. 6. The patients who had grand mal discharge in their EEG before treatment experienced increase of this discharge after Mogadon. 7. Two children who had not grand mal seizures before treatment developed this type of seizure after Mogadon, coincident with the increase of grand mal discharges in their EEG. These changes spontaneously disappeared after 3 months of treatment. 8. In 3 cases, after the disappearance of grand mal discharges, the EEG showed focal discharges.

Examination of craniofacial bones associated with auricular anomaly using three-dimensional computer tomography

International Nuclear Information System (INIS)

Ono, Ichiro; Ohura, Takehiko; Iwao, Fumiya

1989-01-01

Three-dimensional computer tomography (3D-CT) was performed in 60 patients with auricular anomaly to determine the site and severity of deformity in the hemifacial microsomia. Auricular anomaly, underdevelopment and malposition of the cranium, temporo-mandibular joint, zygoma, maxilla and mandible were observed in almost all of the patients; however, the severity of these malformations varied from patient to patient. Regarding severest deformity, there were positional differences among patients. According to impairment sites, hemifacial microsomia fell into the following five types: (1) cranium type, (2) maxillary alveolar process type, (3) localized mandibular ramus type, (4) overall mandibular type, and (5) complex type (combination of the aforementioned types). Facial asymmetry accompanied by auricular anomaly was associated with various pathologic conditions. The temporomandibular joint was often deviated towards the anteromedial side for localized mandibular ramus type and overall mandibular type. Posterial deviation was predominant for cranium type. In patients with hemifacial microsomia of the cranium type associated with protrusion mainly in the occipital region on the affected side, deformity was considered attributable to underdevelopment of the temporal bone and delay in closure of the temporo-occipital suture. The deformity for cranium type may be defined as the second branchial syndrome. In conclusion, hemifacial microsomia have various deformities and may fall into five categories. Craniofacial microsomia and hemi-auriculo-temporo-mandibular dysplastic syndrome give a more precise concept for auricular anomaly. (N.K.)

Examination of craniofacial bones associated with auricular anomaly using three-dimensional computer tomography

Energy Technology Data Exchange (ETDEWEB)

Ono, Ichiro; Ohura, Takehiko; Iwao, Fumiya (Hokkaido Univ., Sapporo (Japan). School of Medicine) (and others)

1989-07-01

Three-dimensional computer tomography (3D-CT) was performed in 60 patients with auricular anomaly to determine the site and severity of deformity in the hemifacial microsomia. Auricular anomaly, underdevelopment and malposition of the cranium, temporo-mandibular joint, zygoma, maxilla and mandible were observed in almost all of the patients; however, the severity of these malformations varied from patient to patient. Regarding severest deformity, there were positional differences among patients. According to impairment sites, hemifacial microsomia fell into the following five types: (1) cranium type, (2) maxillary alveolar process type, (3) localized mandibular ramus type, (4) overall mandibular type, and (5) complex type (combination of the aforementioned types). Facial asymmetry accompanied by auricular anomaly was associated with various pathologic conditions. The temporomandibular joint was often deviated towards the anteromedial side for localized mandibular ramus type and overall mandibular type. Posterial deviation was predominant for cranium type. In patients with hemifacial microsomia of the cranium type associated with protrusion mainly in the occipital region on the affected side, deformity was considered attributable to underdevelopment of the temporal bone and delay in closure of the temporo-occipital suture. The deformity for cranium type may be defined as the second branchial syndrome. In conclusion, hemifacial microsomia have various deformities and may fall into five categories. Craniofacial microsomia and hemi-auriculo-temporo-mandibular dysplastic syndrome give a more precise concept for auricular anomaly. (N.K.).

Case of Rapid Progression of Hemiatrophy on the Face: A New Clinical Entity?

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Hisashi Nomura

2015-01-01

Full Text Available A lot of diseases, including lupus profundus, morphea, lipodystrophy, and Parry-Romberg syndrome, may manifest progressive hemifacial atrophy. These diseases usually progress slowly and rapid progression of atrophy is extremely rare. We report a case of elderly-onset rapid progression of hemifacial atrophy only in three weeks. Our case did not meet variable differential diagnoses. We discuss the clinical character of the patient against the past of literature and suppose it may be a new clinical entity.

Functional consequences of mutations in CDKL5, an X-linked gene involved in infantile spasms and mental retardation.

Science.gov (United States)

Bertani, Ilaria; Rusconi, Laura; Bolognese, Fabrizio; Forlani, Greta; Conca, Barbara; De Monte, Lucia; Badaracco, Gianfranco; Landsberger, Nicoletta; Kilstrup-Nielsen, Charlotte

2006-10-20

Mutations in the X-linked cyclin-dependent kinase-like 5 (CDKL5) gene have been identified in patients with Rett syndrome, West syndrome, and X-linked infantile spasms sharing the common features of generally intractable early seizures and mental retardation. Disease-causing mutations are distributed in both the catalytic domain and in the large COOH terminus. In this report, we examine the functional consequences of some Rett mutations of CDKL5 together with some synthetically designed derivatives useful to underline the functional domains of the protein. The mutated CDKL5 derivatives have been subjected to in vitro kinase assays and analyzed for phosphorylation of the TEY (Thr-Glu-Tyr) motif within the activation loop, their subcellular localization, and the capacity of CDKL5 to interact with itself. Whereas wild-type CDKL5 autophosphorylates and mediates the phosphorylation of the methyl-CpG-binding protein 2 (MeCP2) in vitro, Rett-mutated proteins show both impaired and increased catalytic activity suggesting that a tight regulation of CDKL5 is required for correct brain functions. Furthermore, we show that CDKL5 can self-associate and mediate the phosphorylation of its own TEY (Thr-Glu-Tyr) motif. Eventually, we show that the COOH terminus regulates CDKL5 properties; in particular, it negatively influences the catalytic activity and is required for its proper sub-nuclear localization. We propose a model in which CDKL5 phosphorylation is required for its entrance into the nucleus whereas a portion of the COOH-terminal domain is responsible for a stable residency in this cellular compartment probably through protein-protein interactions.

Genetics Home Reference: dystonia 6

Science.gov (United States)

... neck, causing problems with speaking (dysarthria) and eating (dysphagia). Eyelid twitching (blepharospasm) may also occur. Involvement of ... dystonia, DYT6 type The Bachmann-Strauss Dystonia and Parkinson Foundation: What Is Dystonia? Patient Support and Advocacy ...

Characterization of basal hepatic bile flow and the effects of intravenous cholecystokinin on the liver, sphincter, and gallbladder in patients with sphincter of Oddi spasm

International Nuclear Information System (INIS)

Krishnamurthy, Gerbail T.; Krishnamurthy, Shakuntala; Watson, Randy D.

2004-01-01

The major objectives of this project were to establish the pattern of basal hepatic bile flow and the effects of intravenous administration of cholecystokinin on the liver, sphincter of Oddi, and gallbladder, and to identify reliable parameters for the diagnosis of sphincter of Oddi spasm (SOS). Eight women with clinically suspected sphincter of Oddi spasm (SOS group), ten control subjects (control group), and ten patients who had recently received an opioid (opioid group) were selected for quantitative cholescintigraphy with cholecystokinin. Each patient was studied with 111-185 MBq (3-5 mCi) technetium-99m mebrofenin after 6-8 h of fasting. Hepatic phase images were obtained for 60 min, followed by gallbladder phase images for 30 min. During the gallbladder phase, 10 ng/kg octapeptide of cholecystokinin (CCK-8) was infused over 3 min through an infusion pump. Hepatic extraction fraction, excretion half-time, basal hepatic bile flow into the gallbladder, gallbladder ejection fraction, and post-CCK-8 paradoxical filling (>30% of basal counts) were identified. Seven of the patients with SOS were treated with antispasmodics (calcium channel blockers), and one underwent endoscopic sphincterotomy. Mean (±SD) hepatic bile entry into the gallbladder (versus GI tract) was widely variable: it was lower in SOS patients (32%±31%) than in controls (61%±36%) and the opioid group (61%±25%), but the difference was not statistically significant. Hepatic extraction fraction, excretion half-time, and pattern of bile flow through both intrahepatic and extrahepatic ducts were normal in all three groups. Gallbladder mean ejection fraction was 9%±4% in the opioid group; this was significantly lower (P<0.0001) than the values in the control group (54%±18%) and the SOS group (48%±29%). Almost all of the bile emptied from the gallbladder refluxed into intrahepatic ducts; it reentered the gallbladder after cessation of CCK-8 infusion (paradoxical gallbladder filling) in all eight

Characterization of basal hepatic bile flow and the effects of intravenous cholecystokinin on the liver, sphincter, and gallbladder in patients with sphincter of Oddi spasm

Energy Technology Data Exchange (ETDEWEB)

Krishnamurthy, Gerbail T.; Krishnamurthy, Shakuntala [Department of Nuclear Medicine, Tuality Community Hospital, 335 SE 8th Avenue, OR 97123, Hillsboro (United States); Watson, Randy D. [Department of Gastroenterology, Tuality Community Hospital, Hillsboro, OR (United States)

2004-01-01

The major objectives of this project were to establish the pattern of basal hepatic bile flow and the effects of intravenous administration of cholecystokinin on the liver, sphincter of Oddi, and gallbladder, and to identify reliable parameters for the diagnosis of sphincter of Oddi spasm (SOS). Eight women with clinically suspected sphincter of Oddi spasm (SOS group), ten control subjects (control group), and ten patients who had recently received an opioid (opioid group) were selected for quantitative cholescintigraphy with cholecystokinin. Each patient was studied with 111-185 MBq (3-5 mCi) technetium-99m mebrofenin after 6-8 h of fasting. Hepatic phase images were obtained for 60 min, followed by gallbladder phase images for 30 min. During the gallbladder phase, 10 ng/kg octapeptide of cholecystokinin (CCK-8) was infused over 3 min through an infusion pump. Hepatic extraction fraction, excretion half-time, basal hepatic bile flow into the gallbladder, gallbladder ejection fraction, and post-CCK-8 paradoxical filling (>30% of basal counts) were identified. Seven of the patients with SOS were treated with antispasmodics (calcium channel blockers), and one underwent endoscopic sphincterotomy. Mean ({+-}SD) hepatic bile entry into the gallbladder (versus GI tract) was widely variable: it was lower in SOS patients (32%{+-}31%) than in controls (61%{+-}36%) and the opioid group (61%{+-}25%), but the difference was not statistically significant. Hepatic extraction fraction, excretion half-time, and pattern of bile flow through both intrahepatic and extrahepatic ducts were normal in all three groups. Gallbladder mean ejection fraction was 9%{+-}4% in the opioid group; this was significantly lower (P<0.0001) than the values in the control group (54%{+-}18%) and the SOS group (48%{+-}29%). Almost all of the bile emptied from the gallbladder refluxed into intrahepatic ducts; it reentered the gallbladder after cessation of CCK-8 infusion (paradoxical gallbladder filling

[Bell's palsy].

Science.gov (United States)

Prud'hon, S; Kubis, N

2018-03-30

Idiopathic peripheral facial palsy, also named Bell's palsy, is the most common cause of peripheral facial palsy in adults. Although it is considered as a benign condition, its social and psychological impact can be dramatic, especially in the case of incomplete recovery. The main pathophysiological hypothesis is the reactivation of HSV 1 virus in the geniculate ganglia, leading to nerve edema and its compression through the petrosal bone. Patients experience an acute (less than 24 hours) motor deficit involving ipsilateral muscles of the upper and lower face and reaching its peak within the first three days. Frequently, symptoms are preceded or accompanied by retro-auricular pain and/or ipsilateral face numbness. Diagnosis is usually clinical but one should look for negative signs to eliminate central facial palsy or peripheral facial palsy secondary to infectious, neoplastic or autoimmune diseases. About 75% of the patients will experience spontaneous full recovery, this rate can be improved with oral corticotherapy when introduced within the first 72 hours. To date, no benefit has been demonstrated by adding an antiviral treatment. Hemifacial spasms (involuntary muscles contractions of the hemiface) or syncinesia (involuntary muscles contractions elicited by voluntary ones, due to aberrant reinnervation) may complicate the disease's course. Electroneuromyography can be useful at different stages: it can first reveal the early conduction bloc, then estimate the axonal loss, then bring evidence of the reinnervation process and, lastly, help for the diagnosis of complications. Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Anterior Inferior Cerebellar Arteries Juxtaposed with the Internal Acoustic Meatus and Their Relationship to the Cranial Nerve VII/VIII Complex.

Science.gov (United States)

Alonso, Fernando; Kassem, Mohammad W; Iwanaga, Joe; Oskouian, Rod J; Loukas, Marios; Demerdash, Amin; Tubbs, R Shane

2017-08-16

Vascular loops in the cerebellopontine angle (CPA) and their relationship to cranial nerves have been used to explain neurological symptoms. The anterior inferior cerebellar artery (AICA) has variable branches producing vascular loops that can compress the facial cranial nerve (CN) VII and vestibulocochlear (CN VIII) nerves. AICA compression of the facial-vestibulocochlear nerve complex can lead to various clinical presentations, including hemifacial spasm (HFS), tinnitus, and hemiataxia. The formation of arterial loops inside or outside of the internal auditory meatus (IAM) can cause abutment or compression of CN VII and CN VIII. Twenty-five (50 sides) fresh adult cadavers underwent dissection of the cerebellopontine angle in the supine position. In regard to relationships between the AICA and the nerves of the facial/vestibulocochlear complex, 33 arteries (66%) traveled in a plane between the facial/nervus intermedius nerves and the cochlear and vestibular nerves. Five arteries (10%) traveled below the CN VII/VIII complex, six (12%) traveled posterior to the nerve complex, four (8%) formed a semi-circle around the upper half of the nerve complex, and two (4%) traveled between and partially separated the nervus intermedius and facial nerve proper. Our study found that the majority of AICA will travel in a plane between the facial/nervus intermedius nerves and the cochlear and vestibular nerves. Although the relationship between the AICA and porus acusticus and AICA and the nerves of the CN VII/VIII complex are variable, based on our findings, some themes exist. Surgeons should consider these with approaches to the cerebellopontine angle.

Malformação da transição crânio-vertebral como causa de síndrome do ângulo ponto-cerebelar: relato de dois casos Cerebello-pontine angle syndrome associated with cranio-vertebral malformation: report of two cases

Directory of Open Access Journals (Sweden)

Amauri Batista da Silva

1972-03-01

Full Text Available Os autores relatam dois casos de síndrome do ângulo ponto-cerebelar nos quais não havia neoplasia nessa região. Ambos os pacientes apresentavam uma associação de impressão basilar com síndrome de Arnold-Chiari. Após cirurgia descompressiva de fossa posterior os casos evoluiram favoravelmente com a diminuição progressiva dos sintomas neurológicos, restando apenas, no segundo paciente, certo grau de paralisia e de espasmo facial, à direita. São feitas considerações em tôrno das causas mais freqüentes da síndrome do ângulo ponto-cerebelar, bem como acêrca dos quadros clínicos mais usualmente encontrados em doentes que apresentam malformações da transição crânio-vertebral. Finalmente, os autores tecem breves considerações a respeito do possível mecanismo lesionai do VIII, do V e do VII nervos cranianos nos dois casos relatados.Two cases of cerebelo-pontine angle syndrome associated with basilar impression and Arnold-Chiari malformation are reported. In both cases neuroradiological studies and surgical exploration failled to demonstrate any space occupying lesion. After surgery the patients progressively recovered disapearing the neurological symptoms except for a slaight hemifacial spasm that remained in case 2. Clinical signs present in cerebello-pontine angle lesions and the occipito-cervical malformations are discussed. A possible mechanism to explain the association of both conditions is discussed.

Vascular anomalies of the cerebellopontine angle; Vaskulaere Erkrankungen des Kleinhirnbrueckenwinkels

Energy Technology Data Exchange (ETDEWEB)

Papanagiotou, P.; Grunwald, I.Q.; Politi, M.; Struffert, T.; Ahlhelm, F.; Reith, W. [Universitaetsklinikum des Saarlandes, Homburg/Saar (Germany). Klinik fuer Diagnostische und Interventionelle Neuroradiologie

2006-03-15

Vascular anomalies of the cerebellopontine angle are rare compared to tumors in this area. Irritation of the trigeminal, facial, or vestibulocochlear nerve may cause trigeminal neuralgia, hemifacial spasm and vertigo, or tinnitus accordingly. Vessel loops in the cerebellopontine cisterns may cause compression at the root entry or exit zone of the cranial nerves V, VII, and VIII, a phenomenon which is called ''vascular loop syndrome.'' Megadolichobasilar artery and aneurysms of the vertebrobasilar system can also lead to dislocation and compression of the cranial nerves and brain stem. Three-dimensional CISS MR imaging and MR angiography are useful in the detection of neurovascular compression. Microvascular decompression is an effective surgical procedure in the management of compression syndromes of the cranial nerves V, VII, and VIII. (orig.) [German] Gegenueber den Raumforderungen stellen vaskulaere Veraenderungen des Kleinhirnbrueckenwinkels eher eine Ausnahme dar. Trigeminusneuralgie, hemifazialer Spasmus und Schwindel oder Tinnitus koennen bei Irritationen des Nervus trigeminus, facialis und vestibulocochlearis auftreten. Schlingenbildungen der Gefaesse in den Kleinhirnzisternen koennen eine Kompression der Eintritts- oder Austrittszonen der Hirnnerven V, VII und VIII am Hirnstamm hervorrufen, was als ''Vascular-loop-Syndrom'' bezeichnet wird. Zu Verlagerungen und Kompressionen von Hirnnerven und Hirnstamm koennen, wenn auch seltener, die Megadolichobasilaris und Aneurysmen des vertebrobasilaeren Systems fuehren. Bezueglich der Bildgebung ist die Magnetresonanztomographie (MRT) die Methode der Wahl. Die 3D-CISS-Sequenz und die MR-Angiographie sind hilfreich zur Darstellung der neurovaskulaeren Kompression. Die Methode der mikrovaskulaeren Dekompression ist eine wirkungsvolle Methode zur Beseitigung gefaessbedingter Kompressionen der Hirnnerven V, VII und VIII. (orig.)

Vascular anomalies of the cerebellopontine angle; Vaskulaere Erkrankungen des Kleinhirnbrueckenwinkels

Energy Technology Data Exchange (ETDEWEB)

Papanagiotou, P; Grunwald, I Q; Politi, M; Struffert, T; Ahlhelm, F; Reith, W [Universitaetsklinikum des Saarlandes, Homburg/Saar (Germany). Klinik fuer Diagnostische und Interventionelle Neuroradiologie

2006-03-15

Vascular anomalies of the cerebellopontine angle are rare compared to tumors in this area. Irritation of the trigeminal, facial, or vestibulocochlear nerve may cause trigeminal neuralgia, hemifacial spasm and vertigo, or tinnitus accordingly. Vessel loops in the cerebellopontine cisterns may cause compression at the root entry or exit zone of the cranial nerves V, VII, and VIII, a phenomenon which is called ''vascular loop syndrome.'' Megadolichobasilar artery and aneurysms of the vertebrobasilar system can also lead to dislocation and compression of the cranial nerves and brain stem. Three-dimensional CISS MR imaging and MR angiography are useful in the detection of neurovascular compression. Microvascular decompression is an effective surgical procedure in the management of compression syndromes of the cranial nerves V, VII, and VIII. (orig.) [German] Gegenueber den Raumforderungen stellen vaskulaere Veraenderungen des Kleinhirnbrueckenwinkels eher eine Ausnahme dar. Trigeminusneuralgie, hemifazialer Spasmus und Schwindel oder Tinnitus koennen bei Irritationen des Nervus trigeminus, facialis und vestibulocochlearis auftreten. Schlingenbildungen der Gefaesse in den Kleinhirnzisternen koennen eine Kompression der Eintritts- oder Austrittszonen der Hirnnerven V, VII und VIII am Hirnstamm hervorrufen, was als ''Vascular-loop-Syndrom'' bezeichnet wird. Zu Verlagerungen und Kompressionen von Hirnnerven und Hirnstamm koennen, wenn auch seltener, die Megadolichobasilaris und Aneurysmen des vertebrobasilaeren Systems fuehren. Bezueglich der Bildgebung ist die Magnetresonanztomographie (MRT) die Methode der Wahl. Die 3D-CISS-Sequenz und die MR-Angiographie sind hilfreich zur Darstellung der neurovaskulaeren Kompression. Die Methode der mikrovaskulaeren Dekompression ist eine wirkungsvolle Methode zur Beseitigung gefaessbedingter Kompressionen der Hirnnerven V, VII und VIII. (orig.)

Meige's Syndrome: Rare Neurological Disorder Presenting as Conversion Disorder.

Science.gov (United States)

Debadatta, Mohapatra; Mishra, Ajay K

2013-07-01

Meige's syndrome is a rare neurological syndrome characterized by oromandibular dystonia and blepharospasm. Its pathophysiology is not clearly determined. A 35-year-old female presented to psychiatric department with blepharospasm and oromandibular dystonia with clinical provisional diagnosis of psychiatric disorder (Conversion Disorder). After thorough physical examination including detailed neurological exam and psychiatric evaluation no formal medical or psychiatric diagnosis could be made. The other differential diagnoses of extra pyramidal symptom, tardive dyskinesia, conversion disorder, anxiety disorder were ruled out by formal diagnostic criteria. Consequently with suspicion of Meige's syndrome she was referred to the department of Neurology and the diagnosis was confirmed. Hence, Meige's syndrome could be misdiagnosed as a psychiatric disorder such as conversion disorder or anxiety disorder because clinical features of Meige's syndrome are highly variable and affected by psychological factors and also can be inhibited voluntarily to some extent.

Tilt table standing for reducing spasticity after spinal cord injury.

Science.gov (United States)

Bohannon, R W

1993-10-01

A patient with a T12 spinal cord injury and intractable extensor spasms of the lower extremities participated in tilt table standing trial on 5 nonconsecutive days to determine if the intervention would affect his spasticity and spasms. Each day's standing trial was followed by an immediate reduction in lower extremity spasticity (measured using the modified Ashworth scale and pendulum testing). Standing was also accompanied by a reduction in spasms that lasted until the following morning. The reduction of spasms was particularly advantageous to the performance of car transfers. Tilt table standing merits further examination as a physical treatment of spasms that accompany central nervous system lesions.

Infantile Spasms

Science.gov (United States)

... varied programs of research on epilepsy and other seizure disorders. This research is aimed at discovering new ways ... varied programs of research on epilepsy and other seizure disorders. This research is aimed at discovering new ways ...

Clinical profile of infants with hypsarrhythmia.

Science.gov (United States)

Khreisat, Wael Hayel

2011-09-01

The present study was done in order to obtain a baseline profile of infantile spasms and associated neurological disorders. The study included 50 patients with infantile spasm in Queen Rania Hospital for children in Jordan. The following data were obtained: sex, age at onset of spasms, details of seizure, family history of epilepsy, significant pre-/peri/ post-natal insults, Electroencephalography and detailed neuro imaging evaluation , detailed neurological, neuro developmental ,assessment were done by. Broad categories of possible etiologies were used the results were recorded for further study. Age of onset of infantile spasms ranged from 1month to 1 year and 6 months , (mean 4.8 months). The mean time of presentation was 9.4 months . A male preponderance was noted (74 %). flexor spasms (52%) was the commonest . Other types of seizures also accompanied infantile spasm in 44% children . (84%) were born of normal delivery, History of birth asphyxia was obtained in 48%, 3 (6%) had positive family history Developmental delay was recognized prior to onset of spasms in 52%, microcephaly was the commonest associated problem, Imaging studies of the brain revealed abnormality in 18 patients. 78% patients were classified as symptomatic and 22 % as cryptogenic. the pattern of infantile spasm in our country do not differ from that of developed countries, further researches is required to prevent both chronic epilepsy and psychomotor retardation and .preventive measurement to prevent birth asphyxia is recommended.

Distichiasis in a ferret (Mustela putorius furo)

NARCIS (Netherlands)

Verboven, CAPM; Djajadiningrat-Laanen, S.C.; Kitslaar, W.J.P.; Grinwis, G.C.M.; Schoemaker, N.J.; Boevé, M.H.

A 4-year-old intact male ferret was presented to the Ophthalmology Service of the Department of Clinical Sciences of Companion Animals of Utrecht University with chronic blepharospasm, epiphora, and conjunctivitis of the right eye. Examination of the eye revealed mild conjunctivitis and three hairs

Genetisk udredning ved infantile spasmer

DEFF Research Database (Denmark)

Hansen, Lars Kjærsgaard; Ousager, Lilian Bomme; Møller, Rikke Steensbjerre

2012-01-01

Infantile spasms are a symptom of a severe epileptic encephalopathy. It is important to determine the aetiology for a child's disease. When a standard programme for evaluating the aetiology of the infantile spasms is unsuccessful genetic causes should be considered. We suggest array CGH as the fi......Infantile spasms are a symptom of a severe epileptic encephalopathy. It is important to determine the aetiology for a child's disease. When a standard programme for evaluating the aetiology of the infantile spasms is unsuccessful genetic causes should be considered. We suggest array CGH...

Voluntary Control of the Near Reflex: A Case Report

Directory of Open Access Journals (Sweden)

Serpil Akar

2014-03-01

Full Text Available Spasm of the near reflex is a rare disorder that involves intermittent and variable episodes of esotropia, pseudomyopia, and pupillary myosis. It is usually functional in origin and is seen mainly in young patients. Treatment options for spasm of the near reflex have had variable success. In instances where the etiology of spasm of the near reflex was suspected to be hysteria, psychotherapy has proven beneficial. We report the case of an 11-year-old girl who had functional spasm of the near reflex. The symptoms persisted for two years. Symptomatic relief was achieved by cycloplegia and spectacle correction (added plus lenses at near. The patient also underwent psychological counseling. In our case, the functional spasm of the near reflex spontaneously resolved after 2 years. (Turk J Ophthalmol 2014; 44: 161-3

MRI-based diagnostic imaging of the intratemporal facial nerve; Die kernspintomographische Darstellung des intratemporalen N. facialis

Energy Technology Data Exchange (ETDEWEB)

Kress, B.; Baehren, W. [Bundeswehrkrankenhaus Ulm (Germany). Abt. fuer Radiologie

2001-07-01

Detailed imaging of the five sections of the full intratemporal course of the facial nerve can be achieved by MRI and using thin tomographic section techniques and surface coils. Contrast media are required for tomographic imaging of pathological processes. Established methods are available for diagnostic evaluation of cerebellopontine angle tumors and chronic Bell's palsy, as well as hemifacial spasms. A method still under discussion is MRI for diagnostic evaluation of Bell's palsy in the presence of fractures of the petrous bone, when blood volumes in the petrous bone make evaluation even more difficult. MRI-based diagnostic evaluation of the idiopatic facial paralysis currently is subject to change. Its usual application cannot be recommended for routine evaluation at present. However, a quantitative analysis of contrast medium uptake of the nerve may be an approach to improve the prognostic value of MRI in acute phases of Bell's palsy. (orig./CB) [German] Die detaillierte kernspintomographische Darstellung des aus 5 Abschnitten bestehenden intratemporalen Verlaufes des N. facialis gelingt mit der MRI unter Einsatz von Duennschichttechniken und Oberflaechenspulen. Zur Darstellung von pathologischen Vorgaengen ist die Gabe von Kontrastmittel notwendig. Die Untersuchung in der Diagnostik von Kleinhirnbrueckenwinkeltumoren und der chronischen Facialisparese ist etabliert, ebenso wie die Diagnostik des Hemispasmus facialis. In der Diskussion ist die MRI zur Dokumentation der Facialisparese bei Felsenbeinfrakturen, wobei die Einblutungen im Felsenbein die Beurteilung erschweren. Die kernspintomographische Diagnostik der idiopathischen Facialisparese befindet sich im Wandel. In der herkoemmlichen Form wird sie nicht zur Routinediagnostik empfohlen. Die quantitative Analyse der Kontrastmittelaufnahme im Nerv koennte jedoch die prognostische Bedeutung der MRI in der Akutphase der Bell's palsy erhoehen. (orig.)

Botulinum Toxin and Muscle Atrophy: A Wanted or Unwanted Effect.

Science.gov (United States)

Durand, Paul D; Couto, Rafael A; Isakov, Raymond; Yoo, Donald B; Azizzadeh, Babak; Guyuron, Bahman; Zins, James E

2016-04-01

While the facial rejuvenating effect of botulinum toxin type A is well known and widespread, its use in body and facial contouring is less common. We first describe its use for deliberate muscle volume reduction, and then document instances of unanticipated and undesirable muscle atrophy. Finally, we investigate the potential long-term adverse effects of botulinum toxin-induced muscle atrophy. Although the use of botulinum toxin type A in the cosmetic patient has been extensively studied, there are several questions yet to be addressed. Does prolonged botulinum toxin treatment increase its duration of action? What is the mechanism of muscle atrophy and what is the cause of its reversibility once treatment has stopped? We proceed to examine how prolonged chemodenervation with botulinum toxin can increase its duration of effect and potentially contribute to muscle atrophy. Instances of inadvertent botulinum toxin-induced atrophy are also described. These include the "hourglass deformity" secondary to botulinum toxin type A treatment for migraine headaches, and a patient with atrophy of multiple facial muscles from injections for hemifacial spasm. Numerous reports demonstrate that muscle atrophy after botulinum toxin type A treatment occurs and is both reversible and temporary, with current literature supporting the notion that repeated chemodenervation with botulinum toxin likely responsible for both therapeutic and incidental temporary muscle atrophy. Furthermore, duration of response may be increased with subsequent treatments, thus minimizing frequency of reinjection. Practitioners should be aware of the temporary and reversible effect of botulinum toxin-induced muscle atrophy and be prepared to reassure patients on this matter. © 2016 The American Society for Aesthetic Plastic Surgery, Inc. Reprints and permission: journals.permissions@oup.com.

CLINICAL AND RADIOGRAPHIC FEATURES OF PARRY-ROMBERG SYNDROME

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Mithula NAIR

2017-10-01

Full Text Available Parry-Romberg syndrome or progressive hemifacial atrophy is a craniofacial disorder characterized by slow and progressive atrophy, generally unilateral, of facial tissues including muscles, bones and skin. The coup de sabre is a clear line of demarcation seen between the normal and abnormal structures. The severity of the facial deformity is dependent on the age of onset of the disease. Cosmetic management is the only available treatment and has to be delayed until facial growth is completed. The present case report deals with a 43-year-old woman with progressive hemifacial atrophy which started from the age of 10 months. Despite almost complete involvement of the right paramedian area and the early age of onset, she had neither eye changes nor any dental malformations.

Compare Of the West Syndrome with Other Syndromes in the Epileptic Encephalopathy - Kosovo Experience

OpenAIRE

Zeka, Naim; Gërguri, Abdurrahim; Bejiqi, Ramush; Retkoceri, Ragip; Vuciterna, Armend

2017-01-01

BACKGROUND: West Syndrome (WS) represents as a specific epileptic encephalopathy characterised with a unique type of attacks, called infantile spasms, severe forms of abnormalities in electroencephalographic (EEG) records as a hypsarythmias and delays in the psychomotoric development. The characteristics of the disease, mostly affecting male gender, are infantile spasms and typical findings in EEG as a hypsarythmia. Infantile spasms are a consequence of many factors in the undeveloped brain. ...

Persistent pseudomyopia following a whiplash injury in a previously emmetropic woman

OpenAIRE

Hughes, Fintan E.; Treacy, Maxwell P.; Duignan, Emma S.; Mullaney, Paul B.

2017-01-01

Purpose: Accommodative spasm, which manifests as ciliary muscle spasm, convergent strabismus or miosis, is a recognised consequence of head trauma. In whiplash cases, cervical spine hyperextension poses a risk of contra-coup injury and brainstem trauma, and is known to affect the visual system. However, to date, no cases of accommodative spasm due to whiplash injury have been reported. Observations: We present the case of a 34-year-old female who developed sudden onset blurred distance vis...

Clinical and angiographic characteristics of acute myocardial infarction caused by vasospastic angina without organic coronary heart disease

International Nuclear Information System (INIS)

Kim, Pum-Joon; Seung, Ki-Bae; Kim, Dong-Bin

2007-01-01

Vasospastic angina (VA) can occasionally cause acute myocardial infarction (AMI). From January 2003 to June 2005, coronary spastic angina was diagnosed in 292 patients by performing spasm provocation tests. Among the 292 patients, 21 (7.2% of all the VA patients) had an AMI. There were 20 patients who initially visited the emergency room for AMI without suffering prior VA. One patient with a history of VA had an AMI when he discontinued his medication. Among the 21 VA patients with AMI, 14 had experienced severe emotional stress before they visited the emergency room. The spasm provocation test showed that the VA patients with AMI had more multivessel and diffuse spasm than the VA patients without AMI (p<0.001). Clinically, the VA patients with AMI usually had their first symptom of VA as the severe chest pain of AMI. Two-thirds of the VA patients with AMI had experienced emotional stress before their AMI. Angiographically, the spasm provocation test for VA patients with AMI showed more multivessel and diffuse spasm than in VA patients without AMI. (author)

Predicting Nonauditory Adverse Radiation Effects Following Radiosurgery for Vestibular Schwannoma: A Volume and Dosimetric Analysis

International Nuclear Information System (INIS)

Hayhurst, Caroline; Monsalves, Eric; Bernstein, Mark; Gentili, Fred; Heydarian, Mostafa; Tsao, May; Schwartz, Michael; Prooijen, Monique van; Millar, Barbara-Ann; Ménard, Cynthia; Kulkarni, Abhaya V.; Laperriere, Norm; Zadeh, Gelareh

2012-01-01

Purpose: To define clinical and dosimetric predictors of nonauditory adverse radiation effects after radiosurgery for vestibular schwannoma treated with a 12 Gy prescription dose. Methods: We retrospectively reviewed our experience of vestibular schwannoma patients treated between September 2005 and December 2009. Two hundred patients were treated at a 12 Gy prescription dose; 80 had complete clinical and radiological follow-up for at least 24 months (median, 28.5 months). All treatment plans were reviewed for target volume and dosimetry characteristics; gradient index; homogeneity index, defined as the maximum dose in the treatment volume divided by the prescription dose; conformity index; brainstem; and trigeminal nerve dose. All adverse radiation effects (ARE) were recorded. Because the intent of our study was to focus on the nonauditory adverse effects, hearing outcome was not evaluated in this study. Results: Twenty-seven (33.8%) patients developed ARE, 5 (6%) developed hydrocephalus, 10 (12.5%) reported new ataxia, 17 (21%) developed trigeminal dysfunction, 3 (3.75%) had facial weakness, and 1 patient developed hemifacial spasm. The development of edema within the pons was significantly associated with ARE (p = 0.001). On multivariate analysis, only target volume is a significant predictor of ARE (p = 0.001). There is a target volume threshold of 5 cm3, above which ARE are more likely. The treatment plan dosimetric characteristics are not associated with ARE, although the maximum dose to the 5th nerve is a significant predictor of trigeminal dysfunction, with a threshold of 9 Gy. The overall 2-year tumor control rate was 96%. Conclusions: Target volume is the most important predictor of adverse radiation effects, and we identified the significant treatment volume threshold to be 5 cm3. We also established through our series that the maximum tolerable dose to the 5th nerve is 9 Gy.

Effects of parasagittal meningiomas on intracranial venous circulation assessed by the virtual reality technology.

Science.gov (United States)

Wang, Shousen; Ying, Jianbin; Wei, Liangfeng; Li, Shiqing; Jing, Junjie

2015-01-01

This study is to investigate the compensatory intracranial venous pathways in parasagittal meningiomas (PSM) patients by virtual reality technology. A total of 48 PSM patients (tumor group) and 20 patients with trigeminal neuralgia and hemifacial spasm but without intracranial venous diseases (control group) were enrolled. All patients underwent 3D CE-MRV examination. The 3D reconstructed images by virtual reality technology were used for assessment of diameter and number of intracranial veins, tumor location, venous sinus invasion degree and collateral circulation formation. Diameter of bridging veins in posterior 1/3 superior sagittal sinus (SSS) in tumor group was significantly smaller than that of the control group (P < 0.05). For tumors located in mid 1/3 SSS, diameter of bridging veins and vein of Labbé (VL) in posterior 1/3 SSS decreased significantly (P < 0.05). For tumors located in posterior 1/3 SSS, bridging vein number and transverse sinus (TS) diameter significantly decreased while superficial Sylvian vein (SSV) diameter increased significantly (P < 0.05). Compared with tumor in posterior 1/3 SSS subgroup, number of bridging veins in the tumor in mid 1/3 SSS subgroup increased significantly (P < 0.05). Compared with control group, only the bridging vein number in anterior 1/3 SSS segment in invasion Type 3-4 tumor subgroup decreased significantly (P < 0.05). Diameter of TS and bridging veins in posterior 1/3 SSS segment in sinus invasion Type 5-6 tumor subgroup decreased significantly (P < 0.05). Compared with control group, only the diameter of VL and TS of collateral circulation Grade 1 tumor subgroup decreased significantly (P < 0.05) while in Grade 3 tumor subgroup, TS diameter decreased and SSV diameter increased significantly (P < 0.05). The intracranial blood flow is mainly drained through SSV drainage after SSS occlusion by PSM.

Predicting Nonauditory Adverse Radiation Effects Following Radiosurgery for Vestibular Schwannoma: A Volume and Dosimetric Analysis

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Hayhurst, Caroline; Monsalves, Eric; Bernstein, Mark; Gentili, Fred [Gamma Knife Unit, Division of Neurosurgery, University Health Network, Toronto (Canada); Heydarian, Mostafa; Tsao, May [Radiation Medicine Program, Princess Margaret Hospital, Toronto (Canada); Schwartz, Michael [Radiation Oncology Program and Division of Neurosurgery, Sunnybrook Hospital, Toronto (Canada); Prooijen, Monique van [Radiation Medicine Program, Princess Margaret Hospital, Toronto (Canada); Millar, Barbara-Ann; Menard, Cynthia [Radiation Oncology Program, Princess Margaret Hospital, Toronto (Canada); Kulkarni, Abhaya V. [Division of Neurosurgery, Hospital for Sick Children, University of Toronto (Canada); Laperriere, Norm [Radiation Oncology Program, Princess Margaret Hospital, Toronto (Canada); Zadeh, Gelareh, E-mail: Gelareh.Zadeh@uhn.on.ca [Gamma Knife Unit, Division of Neurosurgery, University Health Network, Toronto (Canada)

2012-04-01

Purpose: To define clinical and dosimetric predictors of nonauditory adverse radiation effects after radiosurgery for vestibular schwannoma treated with a 12 Gy prescription dose. Methods: We retrospectively reviewed our experience of vestibular schwannoma patients treated between September 2005 and December 2009. Two hundred patients were treated at a 12 Gy prescription dose; 80 had complete clinical and radiological follow-up for at least 24 months (median, 28.5 months). All treatment plans were reviewed for target volume and dosimetry characteristics; gradient index; homogeneity index, defined as the maximum dose in the treatment volume divided by the prescription dose; conformity index; brainstem; and trigeminal nerve dose. All adverse radiation effects (ARE) were recorded. Because the intent of our study was to focus on the nonauditory adverse effects, hearing outcome was not evaluated in this study. Results: Twenty-seven (33.8%) patients developed ARE, 5 (6%) developed hydrocephalus, 10 (12.5%) reported new ataxia, 17 (21%) developed trigeminal dysfunction, 3 (3.75%) had facial weakness, and 1 patient developed hemifacial spasm. The development of edema within the pons was significantly associated with ARE (p = 0.001). On multivariate analysis, only target volume is a significant predictor of ARE (p = 0.001). There is a target volume threshold of 5 cm3, above which ARE are more likely. The treatment plan dosimetric characteristics are not associated with ARE, although the maximum dose to the 5th nerve is a significant predictor of trigeminal dysfunction, with a threshold of 9 Gy. The overall 2-year tumor control rate was 96%. Conclusions: Target volume is the most important predictor of adverse radiation effects, and we identified the significant treatment volume threshold to be 5 cm3. We also established through our series that the maximum tolerable dose to the 5th nerve is 9 Gy.

Baclofen Intoxication

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Canan Bor

2015-12-01

Full Text Available Baclofen is a β-(ρ-chlorophenyl derivative of the neurotransmitter gamma-aminobutyric acid (GABA and is usually prescribed for spasticity of spinal cord origin, intractable hiccup, trigeminal neuralgia, hemifacial spasm, and tardive dyskinesia. The usual recommended daily dose ranges from 40-80 mg, and the total dose should not exceed 80 mg per day. A 41 year old woman using baclofen for migraine therapy intended suicide after a bitter headache attack by taking 37 tablets, 10 mg in each. On arrival to emergency room, she was conscious and co-operable, but somnolent, her pupils were normoisocoric and light reflex was intact bilaterally. On her follow up, respiratory insufficiency and unconsciousness was observed so she was entubated orotracheally and transferred to intensive care unit (ICU for advanced tests and therapy. No pathology was determined on cranial CT. On ICU follow up, she was unconscious and mechanically ventilated, her Glasgow Coma Scale was 3/15 (E1M1VE and pupils were mid-dilated with no light reflex. Since she was again conscious, oriented and co-operable on 19th hour of arrival to ICU and 20th hour of arrival to emergency room, spontaneous breathing trials was started. Extubation was carried out on her 31th hour of arrival to ICU and 32th hour of arrival to emergency room. In conclusion; since baclofen overdose may cause deep coma, it should also be included in differential diagnosis. According to our opinion, performing diagnostic toxicological tests is not always possible that’s why history and physical examination is fundamental in case of baclofen intoxication and we can get good results by giving frequent neurological examination, supportive and extracorporeal therapy in such a case.

C-reactive protein for predicting prognosis and its gender-specific associations with diabetes mellitus and hypertension in the development of coronary artery spasm.

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Hung, Ming-Jui; Hsu, Kuang-Hung; Hu, Wei-Syun; Chang, Nen-Chung; Hung, Ming-Yow

2013-01-01

While hypertension is negatively associated with coronary artery spasm (CAS), scarce data are available on diabetes mellitus in relation to CAS. In addition, outcome prediction in patients with CAS is challenging due to the lack of appropriate biomarkers. Therefore, we sought to identify the roles that gender, high-sensitivity C-reactive protein (hs-CRP), diabetes mellitus and hypertension play in CAS development and prognosis. Patients (350 women and 547 men) undergoing diagnostic coronary angiography with or without proven CAS but without obstructive stenosis were evaluated at long-term follow-up (median 102 months). Diabetic women and diabetic men with low hs-CRP levels had a low and high risk of CAS (odds ratio [OR]: 0.16, 95% confidence interval [CI]: 0.01-1.88 and OR: 5.02, 95% CI: 1.03-24.54, respectively). The ORs of CAS in both women and men with the highest hs-CRP tertile (>3 mg/L) reduced from 4.41 to 1.45 and 2.98 to 1.52, respectively, if they had diabetes mellitus, and from 9.68 to 2.43 and 2.60 to 1.75, respectively, if they had hypertension. Hypertension had a more negative effect on CAS development in diabetic than non-diabetic women, which was not observed in men. The highest hs-CRP tertile was an independent predictor of adverse outcomes. Patients with the highest hs-CRP tertile had more coronary events than patients with the lowest hs-CRP tertitle (p = 0.021, log-rank test). Diabetes mellitus contributes to CAS development in men with low hs-CRP levels, but not in women. There are negative effects of diabetes mellitus and hypertension on CAS development in patients with high hs-CRP levels and especially in women. Elevated hs-CRP level independently predicts adverse outcomes.

Facial hemiatrophy: Review of literature and a case report

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Manisha S Tijare

2011-01-01

Full Text Available A case report of hemifacial atrophy is presented in this paper. It is also known as Parry-Romberg syndrome, is an uncommon degenerative and poorly understood condition. It is characterized by progressive atrophy of the skin, subcutaneous tissue, muscle, cartilage or bone; the condition can leave a marked deformity on one side of the face. The incidence and the cause of this alteration is debatable. The most common complications that appear in association to this health disorder are: trigeminal neuritis, facial paresthesia, severe headache and epilepsy. Now, plastic surgery with graft of autogenous fat can be performed, after stabilization of the disease, to correct the deformity. Orthodontic treatment can help in the correction of any associated malformation. This presentation gives a review of concern literature about the etiology, physiopathology, differential diagnosis and treatment of hemifacial atrophy.

Investigations in West Syndrome: Which, When and Why

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Richard E Appleton

2015-06-01

Full Text Available Investigators from the National Infantile Spasms Consortium (NISC in the USA studied the etiology of new-onset infantile spasms (IS in 251 infants (mean age at onset, 7.1, range, 0.1-22.7 months.

A Novel Animal Model for Investigating the Neural Basis of Focal Dystonia

Science.gov (United States)

2017-09-01

including the time for reviewing instructions, searching existing data sources, gathering and maintaining the data needed, and completing and...from an interaction between a predisposing condition and an environmental trigger, we proposed to use 7 Hz deep brain stimulation of the basal ganglia...benign essential blepharospasm, dry eye, motor plasticity, basal ganglia, deep brain stimulation , eyelids, blinking 16. SECURITY CLASSIFICATION OF: 17

Topical Botulinum Toxin

OpenAIRE

Collins, Ashley; Nasir, Adnan

2010-01-01

Nanotechnology is a rapidly growing discipline that capitalizes on the unique properties of matter engineered on the nanoscale. Vehicles incorporating nanotechnology have led to great strides in drug delivery, allowing for increased active ingredient stability, bioavailability, and site-specific targeting. Botulinum toxin has historically been used for the correction of neurological and neuromuscular disorders, such as torticollis, blepharospasm, and strabismus. Recent dermatological indicati...

Transverse facial cleft: A series of 17 cases

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L K Makhija

2011-01-01

Full Text Available Introduction: Transverse facial cleft (Tessier type 7 or congenital macrostomia is a rare congenital anomaly seldom occurring alone and is frequently associated with deformities of the structures developing from the first and second branchial arches. The reported incidence of No. 7 cleft varies from 1 in 60,000 to 1 in 300,000 live births. Material and Methods: Seventeen patients of transeverse facial cleft who presented to us in last 5 years were included in the study. Their history regarding familial and environmental predispositions was recorded. The cases were analysed on basis of sex, laterality, severity, associated anomalies and were graded according to severity. They were operated by z plasty technique and were followed up for 2 years to look for effectiveness of the technique and its complications. Result: Out of the seventeen patients of transverse cleft, none had familial predilection or any environmental etiology like antenatal radiological exposure or intake of drugs of teratogenic potential. Most of the patients (9/17 were associated with hemifacial microsomia and 1 patient was associated with Treacher Colin′s Syndrome. Out of the 6 cases of Grade I clefts, 4 were isolated transverse clefts and of the 10 patients of Grade II clefts, 7 were associated with hemifacial microsomia. We encountered only one case of Grade III Transverse Cleft which was not only associated with hemifacial microsomia but also had cardiac anomaly. Out of the17 cases, 15 were operated and in most of them the outcome was satisfactory.

Clinical-pathomorphological correlation in patients with symptomatic dystonias

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Ivanović Nataša

2002-01-01

Full Text Available Symptomatic dystonia can be the result of various metabolic, degenerative diseases, the consumption of certain medications or exposure to toxic agents. However, only symptomatic dystonia with focal structural lesion provides a significant "window" for, at least indirect, perception of aetiopa-thogenesis and pathomorphological substratum of idiopathic dystonia. Our study included 57 patients with symptomatic dystonia, which as a base had focal or multifocal lesions, of whom 7 patients had generalized dystonia, 18 hemidystonia, 6 segmental dystonia, 7 torticollis, 6 blepharospasm, 7 hand dystonia, 3 spasmodic dysphonia, and 3 had oromandibular dystonia. Stroke was highly statistically the most frequent cause of structural lesions (33/57 or 58%. Relevant pathomorphological changes were present in 50/57 (88% patients, of whom 25 (50% had lesion in the lenticular nucleus (including individual damage of the putamen and globus pallidus, 12/50 (24% had damage of the thalamus and 6/50 (12% had damage of the brainstem. Generalized dystonia was most frequently associated with bilateral lesion of the putamen, hemidystonia with lesion of contralateral putamen, torticollis with damage of the caudate nucleus, hand dystonia with lesion of the thalamus and blepharospasm with lesion of the upper brainstem.

Prepontine Schwannoma Presenting With Atypical Facial Symptoms - A Case Report

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Rishi Kumar Bali

2005-01-01

Full Text Available Face is an important landmark and any pathological condition affecting it has tremendous bearing on psychological make up of the patient. This report describes a rare case of a young female who presented with Hemifacial dysaesthesia complicated by ipsilateral masticatory complex dyskinesia.

Behandling af infantile spasmer

DEFF Research Database (Denmark)

Kjærsgård, Lars; Rasmussen, Niels

2006-01-01

Infantile spasms (IS) are characterised by neurodevelopmental regression, a unique type of seizures and a hypsarrhythmic EEG pattern. Studies recommend the medical treatment of IS as a positive short-term outcome with respect to the spasms and in the resolution of the hypsarrhythmia. However...

Myopathy

Science.gov (United States)

... alternating episodes of twitching and stiffness; and stiff-man syndrome: characterized by episodes of rigidity and reflex spasms common muscle cramps and stiffness, and tetany: characterized by prolonged spasms of the arms and legs × Definition The myopathies are neuromuscular disorders in which the ...

Trekkingen in het gelaat

NARCIS (Netherlands)

Potgieser, Adriaan R E; van Dijk, J.M.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

Wolf-Hirschhorn (4p-) syndrome with West syndrome.

Science.gov (United States)

Motoi, Hirotaka; Okanishi, Tohru; Kanai, Sotaro; Yokota, Takuya; Yamazoe, Tomohiro; Nishimura, Mitsuyo; Fujimoto, Ayataka; Yamamoto, Takamichi; Enoki, Hideo

2016-01-01

Wolf-Hirschhorn syndrome (WHS) is a chromosome disorder (4p-syndrome) which is characterized by craniofacial features and epileptic seizures. Here, we report a case of WHS with West syndrome, in whom the seizures were refractory to several antiepileptic drugs but were responsive to the addition of lamotrigine. The patient had epileptic spasms at age seven months. The interictal electroencephalogram was hypsarrhythmic. After adding lamotrigine, seizures decreased remarkably, and spasms disappeared. We have identified and described the very rare case of a girl with WHS who also developed West syndrome. In this case, adding lamotrigine to her medications effectively treated the spasms.

Clinical presentation and outcome of Sri-Lankan Ornamental Tarantula Poecilotheria fasciata spider bite: a case report

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NP Dinamithra

2013-10-01

Full Text Available We report on a 19-year-old boy with visible muscle spasms admitted to the hospitals 24 hours after spider bite. He was treated effectively with intravenous calcium gluconate followed by oral calcium supplements and made a full recovery 48 hours after the incident. Although no specific treatment exists in Srilanka, it has been suggested that calcium supplements may be beneficial to relieve the muscle spasms. Our patient made a full recovery with calcium supplements suggesting the treatment with calcium is beneficial in relieving the pain and muscle spasms caused by Sri-Lankan Ornamental Tarantula Poecilotheria fasciata.

Malassezia pachydermatis keratomycosis in a dog

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Eric C. Ledbetter

2015-12-01

Full Text Available A 13-year-old female Lhasa Apso was presented for blepharospasm and conjunctival hyperemia of the right eye. Ophthalmic examination revealed an anterior stromal ulcer associated with a raised yellow corneal plaque. In vivo confocal microscopy and cytology of the cornea identified neutrophilic inflammation and yeast cells. Malassezia pachydermatis was isolated from a corneal scraping. Treatment with topical voriconazole ophthalmic solution resolved the keratitis.

Sound-induced facial synkinesis following facial nerve paralysis.

Science.gov (United States)

Ma, Ming-San; van der Hoeven, Johannes H; Nicolai, Jean-Philippe A; Meek, Marcel F

2009-08-01

Facial synkinesis (or synkinesia) (FS) occurs frequently after paresis or paralysis of the facial nerve and is in most cases due to aberrant regeneration of (branches of) the facial nerve. Patients suffer from inappropriate and involuntary synchronous facial muscle contractions. Here we describe two cases of sound-induced facial synkinesis (SFS) after facial nerve injury. As far as we know, this phenomenon has not been described in the English literature before. Patient A presented with right hemifacial palsy after lesion of the facial nerve due to skull base fracture. He reported involuntary muscle activity at the right corner of the mouth, specifically on hearing ringing keys. Patient B suffered from left hemifacial palsy following otitis media and developed involuntary muscle contraction in the facial musculature specifically on hearing clapping hands or a trumpet sound. Both patients were evaluated by means of video, audio and EMG analysis. Possible mechanisms in the pathophysiology of SFS are postulated and therapeutic options are discussed.

Diagnostic value of 123I-betamethyl-p-iodophenyl-pentadecanoic acid (BMIPP) single photon emission computed tomography (SPECT) in patients with chest pain. Comparison with rest-stress 99mTc-tetrofosmin SPECT and coronary angiography

International Nuclear Information System (INIS)

Kawai, Yuko; Nozaki, Yoichi; Ohkusa, Takanori; Sakurai, Masayuki; Morita, Koichi; Tamaki, Nagara

2004-01-01

Basic and clinical studies have indicated that 15-(p-[ 123 I] iodophenyl)-3-(R, S) methylpentadecanoic acid (BMIPP) single photon emission computed tomography (SPECT) can identify ischemic myocardium without evidence of myocardial infarction by the regional decline of tracer uptake. The present study compared BMIPP SPECT with rest-stress myocardial perfusion imaging (MPI) findings and coronary angiography (CAG) in 150 patients with acute chest pain. Patients with acute chest pain who underwent all of the following tests were selected: MPI at rest-stress, BMIPP SPECT at rest and CAG. Organic coronary artery stenosis (≥75%) was observed in 46 patients, 27 patients had total or subtotal coronary occlusion by spasm in the spasm provocation test on CAG and the remaining 77 patients had no significant coronary artery stenosis or spasm. The sensitivity of BMIPP at rest to detect organic stenosis was significantly higher (54%) than that of rest-MPI (33%, p<0.005), but lower than that of stress-MPI (76%, p=0.05). The sensitivity of BMIPP at rest to detect spasm was significantly higher (63%) than that of both rest-MPI (15%; p<0.001) and stress-MPI (19%; p<0.001). Overall, the sensitivity of BMIPP at rest to detect both organic stenosis and spasm was significantly higher (58%) than that of rest-MPI (26%; p<0.001), despite having no significance with that of stress-MPI (55%). The specificity was not significantly different among the three imaging techniques. Resting BMIPP SPECT is an alternative method to stress MPI for identifying patients with not only organic stenosis but also spasm without the need for a stress examination. (author)

A Novel Animal Model for Investigating the Neural Basis of Focal Dystonia

Science.gov (United States)

2016-09-01

plasticity of the human motor cortex in writer’s cramp. Brain. 2003;126:2586–2596. 46. Quartarone A, Morgante F, Sant’angelo A, Rizzo V, Bagnato S...cerebellar output in the genetically dystonic rat . Adv Neurol. 1998;78:63–78. 61. LeDoux MS, Lorden JF, Ervin JM. Cerebellectomy eliminates the motor ...experiments and preliminary recordings from the superior colliculus. 15. SUBJECT TERMS Dystonia, benign essential blepharospasm, dry eye, motor

Effects of a child with a craniofacial anomaly on stability of the parental relationship.

Science.gov (United States)

St John, Dane; Pai, Lori; Belfer, Myron L; Mulliken, John B

2003-09-01

The purpose of this study was to determine rates of divorce in parents of children with various types of craniofacial anomalies and to analyze possible confounding factors. A 29-question survey was sent to parents of all children evaluated in the Craniofacial Centre between 1992 and 1997. Parents were questioned regarding pre- and postnatal marital stability, whether the child's facial anomaly contributed to divorce, and involvement in the child's welfare. Using deformational posterior plagiocephaly as a control group, rates of divorce vs. non-divorce were compared for craniofacial anomalies, categorized as asymmetric (hemifacial microsomia, unilateral coronal synostosis, cleft lip, cleft lip/palate) or symmetric (syndromic-craniosynostosis, orbital hypertelorism, Treacher Collins syndrome). Major anomalies (hemifacial microsomia, craniosynostosis, orbital hypertelorism, Treacher Collins syndrome) were also compared to minor anomalies (cleft lip, cleft lip/palate). Surveys were sent to both parents in 412 families; 403 surveys were returned; and the results were evaluated in 275 families (67%). Frequency analysis demonstrated an overall divorce rate of 6.8% and 4.9% separation. Anomalies associated with the highest rate of divorce were hemifacial microsomia (24.0%), syndromic craniosynostosis (12.2%), and cleft lip/palate (6.8%). 79% of non-divorced couples reported a strong prenatal relationship, whereas 59% of divorced couples reported a problematic relationship. Following birth of the affected child, 47% of non-divorced couples responded that the bonds became stronger and 41% of divorced couples thought the relationship worsened. Two-sided Fisher exact test comparing control vs. all other anomalies showed significance (p=.030) for rates of divorce. Separation of anomalies into asymmetric vs. symmetric and major vs. minor categories demonstrated no significant difference in divorce rate (p>.05). The mother was more likely to become a child's primary caregiver

Documentation for the Computer Assisted Diagnostic Program for Dental Pain

Science.gov (United States)

1989-04-10

FistulCI.IS tract from periapical abscessjpanllis *~thyroidism (brown tumor) *IDeal malignancy *Pericoronitis Epllis granulomatosum Antral polyp...drug-imuced (ex. Dilantin) Amyloidosis Hemifacial hypertrophy C-3 7. Cystic lesions of gingiva Eruption cyst Gingival cyst Parulis...Nasoalveolar cyst Nasopalatine duct cyst 8. Keratotic non-sloughing, non-ulcerated, non-eroded, non-papillazy, lesions Linea alba HYPerkeratosis

Spasticity-assessment: a review

DEFF Research Database (Denmark)

Biering-Sørensen, F.; Nielsen, Jens Bo; Klinge, Klaus Peter

2006-01-01

Study design:Review of the literature on the validity and reliability of assessment of spasticity and spasms.Objectives:Evaluate the most frequently used methods for assessment of spasticity and spasms, with particular focus on individuals with spinal cord lesions.Setting:Clinic for Spinal Cord...... Injuries, Rigshospitalet, University Hospital of Copenhagen, and Department of Medical Physiology, University of Copenhagen, Denmark.Methods:The assessment methods are grouped into clinical, biomechanical and electrophysiological, and the correlation between these is evaluated.Results:Clinical methods......: For assessment of spasticity, the Ashworth and the modified Ashworth scales are commonly used. They provide a semiquantitative measure of the resistance to passive movement, but have limited interrater reliability. Guidelines for the testing procedures should be adhered to. Spasm frequency scales seem...

Linear accelerator stereotactic radiosurgery for vestibular schwannomas: a UK series.

Science.gov (United States)

Benghiat, H; Heyes, G; Nightingale, P; Hartley, A; Tiffany, M; Spooner, D; Geh, J I; Cruickshank, G; Irving, R M; Sanghera, P

2014-06-01

To evaluate non-auditory toxicity and local control after linear accelerator stereotactic radiosurgery (SRS) for the treatment of vestibular schwannomas. The institutional policy was to use SRS for radiologically progressing vestibular schwannomas. Case notes and plans were retrospectively reviewed for all patients undergoing SRS for vestibular schwannomas between September 2002 and June 2012. All patients were surgically immobilised using a BrainLab stereotactic head frame. The treatment plan was generated using BrainLab software (BrainScan 5.03). The aim was to deliver 12 Gy to the surface of the target with no margin. Patients with a minimum of 12 months of follow-up were included for toxicity and local control assessment. Radiological progression was defined as growth on imaging beyond 2 years of follow-up. Overall local control was defined in line with other series as absence of surgical salvage. Ninety-nine patients were identified. Two patients were lost to follow-up. After a median follow-up interval of 2.4 years, the actuarial radiological progression-free survival at 3 years was 100% and overall local control was also 100%. However, two patients progressed radiologically at 3.3 and 4.5 years, respectively. Twenty-one of 97 (22%) evaluable patients suffered trigeminal toxicity and this was persistent in 8/97 (8%). Two of 97 (2%) suffered long-term facial nerve toxicity (one with associated radiological progression causing hemi-facial spasm alone). One of 97 (1%) required intervention for obstructive hydrocephalus. No statistically significant dosimetric relationship could be shown to cause trigeminal or facial nerve toxicity. However, 7/8 patients with persistent trigeminal nerve toxicity had tumours in contact with the trigeminal nerve. SRS delivering 12 Gy using a linear accelerator leads to high local control rates, but only prospective evaluation will fully establish short-term toxicity. In this study, persistent trigeminal toxicity occurred almost

Persistent pseudomyopia following a whiplash injury in a previously emmetropic woman.

Science.gov (United States)

Hughes, Fintan E; Treacy, Maxwell P; Duignan, Emma S; Mullaney, Paul B

2017-12-01

Accommodative spasm, which manifests as ciliary muscle spasm, convergent strabismus or miosis, is a recognised consequence of head trauma. In whiplash cases, cervical spine hyperextension poses a risk of contra-coup injury and brainstem trauma, and is known to affect the visual system. However, to date, no cases of accommodative spasm due to whiplash injury have been reported. We present the case of a 34-year-old female who developed sudden onset blurred distance vision after a rear impact car crash, having previously been emmetropic. Her unaided distance visual acuity was 20/70 in the right eye and 20/20 in the left. Best-corrected visual acuity in the right eye was 20/20 with a correction that progressed from -1.75 to -3.50 DS over the 12 months following the accident.This patient's sudden unilateral myopia, with unilaterally increased amplitude of accommodation suggests pseudomyopia due to accommodative spasm. Magnetic resonance imaging showed no evidence of injury to her brain stem, frontal lobes or oculomotor nerve. The patient is now well adjusted with a -3.50DS corrective lens for the right eye. The accommodation reflex is susceptible to injury at the occipital lobe, frontal eye fields, Edinger-Westphal nuclei and oculomotor nerves. As such it should be examined in patients who present with visual disturbances following whiplash injury.It is important that such cases are identified at presentation, as early intervention can improve outcomes in accommodative spasm and reduce the long term psychological effects often associated with whiplash injuries.

Assessment of pelvic floor muscles in women with deep endometriosis.

Science.gov (United States)

Dos Bispo, Ana Paula Santos; Ploger, Christine; Loureiro, Alessandra Fernandes; Sato, Hélio; Kolpeman, Alexander; Girão, Manoel João Batista Castello; Schor, Eduardo

2016-09-01

To assess function and prevalence of spasms and trigger points of the pelvic floor muscles in women with deep endometriosis. One hundred and four (104) patients were assessed. Group 1 (G1) was composed of 52 subjects diagnosed with deep endometriosis proven by magnetic resonance imaging (MRI); Group 2 (G2) was composed of 52 women with no signs of endometriosis. Subjects from both G1 and G2 were seen at the Division of Pelvic Pain and Endometriosis and at Center for Prevention of Sexually Transmitted Diseases, both at Federal University of São Paulo (UNIFESP), respectively. A full physical therapy evaluation was carried out, including medical history, presence of dyspareunia and physical examination, which included detailed evaluation of pelvic floor muscles and occurrence of muscle spasm, trigger point and muscle function. The average age of the subjects in the study group was 36.4 and 30.9 years in the control group (p = 0.002). A greater prevalence of deep dyspareunia was found in the subjects in the endometriosis group when compared to the control group (p = 0.010). Women in G1 had higher prevalence of muscle spasms. In this group, 53.9 % had spasms-compared to only 17.3 % of women in G2 (p < 0.001). On the other hand, no significant difference between the groups (p = 0.153) was found while searching for the presence of trigger points. Women with deep endometriosis have increased prevalence of pelvic floor muscle spasms when compared to the control group.

Development of a clinical spasticity scale for evaluation of dogs with chronic thoracolumbar spinal cord injury.

Science.gov (United States)

Lewis, Melissa J; Olby, Natasha J

2017-07-01

OBJECTIVE To develop a spasticity scale for dogs with chronic deficits following severe spinal cord injury (SCI) for use in clinical assessment and outcome measurement in clinical trials. ANIMALS 20 chronically paralyzed dogs with a persistent lack of hind limb pain perception caused by an acute SCI at least 3 months previously. PROCEDURES Spasticity was assessed in both hind limbs via tests of muscle tone, clonus, and flexor and extensor spasms adapted from human scales. Measurement of patellar clonus duration and flexor spasm duration and degree was feasible. These components were used to create a canine spasticity scale (CSS; overall score range, 0 to 18). Temporal variation for individual dogs and interrater reliability were evaluated. Gait was quantified with published gait scales, and CSS scores were compared with gait scores and clinical variables. Owners were questioned regarding spasticity observed at home. RESULTS 20 dogs were enrolled: 18 with no apparent hind limb pain perception and 2 with blunted responses; 5 were ambulatory. Testing was well tolerated, and scores were repeatable between raters. Median overall CSS score was 7 (range, 3 to 11), and flexor spasms were the most prominent finding. Overall CSS score was not associated with age, SCI duration, lesion location, or owner-reported spasticity. Overall CSS score and flexor spasm duration were associated with gait scores. CONCLUSIONS AND CLINICAL RELEVANCE The CSS could be used to quantify hind limb spasticity in dogs with chronic thoracolumbar SCI and might be a useful outcome measure. Flexor spasms may represent an integral part of stepping in dogs with severe SCI.

CT findings in patients with infantile epilepsy on ACTH therapy

International Nuclear Information System (INIS)

Watanabe, Kazunari; Hara, Kimiko; Hakamada, Akira; Miyazaki, Shuji.

1981-01-01

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. (Ueda, J.)

Venom ophthalmia caused by venoms of spitting elapid and other snakes: Report of ten cases with review of epidemiology, clinical features, pathophysiology and management.

Science.gov (United States)

Chu, Edward R; Weinstein, Scott A; White, Julian; Warrell, David A

2010-09-01

Venom ophthalmia caused by venoms of spitting elapid and other snakes: report of ten cases with review of epidemiology, clinical features, pathophysiology and management. Chu, ER, Weinstein, SA, White, J and Warrell, DA. Toxicon XX:xxx-xxx. We present ten cases of ocular injury following instillation into the eye of snake venoms or toxins by spitting elapids and other snakes. The natural history of spitting elapids and the toxinology of their venoms are reviewed together with the medical effects and management of venom ophthalmia in humans and domestic animals including both direct and allergic effects of venoms. Although the clinical features and management of envenoming following bites by spitting elapids (genera Naja and Hemachatus) are well documented, these snakes are also capable of "spraying" venom towards the eyes of predators, a defensive strategy that causes painful and potentially blinding ocular envenoming (venom ophthalmia). Little attention has been given to the detailed clinical description, clinical evolution and efficacy of treatment of venom ophthalmia and no clear management guidelines have been formulated. Knowledge of the pathophysiology of ocular envenoming is based largely on animal studies and a limited body of clinical information. A few cases of ocular exposure to venoms from crotaline viperids have also been described. Venom ophthalmia often presents with pain, hyperemia, blepharitis, blepharospasm and corneal erosions. Delay or lack of treatment may result in corneal opacity, hypopyon and/or blindness. When venom is "spat" into the eye, cranial nerve VII may be affected by local spread of venom but systemic envenoming has not been documented in human patients. Management of venom ophthalmia consists of: 1) urgent decontamination by copious irrigation 2) analgesia by vasoconstrictors with weak mydriatic activity (e.g. epinephrine) and limited topical administration of local anesthetics (e.g. tetracaine) 3) exclusion of corneal abrasions

Epilepsy and the Wnt Signaling Pathway

Science.gov (United States)

2015-06-01

forebrain development. The primary target is Wnt 8b, which is elevated in this period 4. Fox G1 is also genetically associated with infantile spasms 8...the Warburg effect’s role in non- cancerous tissues is largely unexplored. Second, in other diseases such as diabetes , Wnt signaling has emerged as...epilepsy and infantile spasms, we found that both mechanisms appeared to contribute. Two of the three genes came from our observation that several genes

Clinical presentation and outcome of Sri-Lankan Ornamental Tarantula Poecilotheria fasciata spider bite: a case report

OpenAIRE

NP Dinamithra; S Sivansuthan; P Johnson; JGP Nishshanka

2013-01-01

We report on a 19-year-old boy with visible muscle spasms admitted to the hospitals 24 hours after spider bite. He was treated effectively with intravenous calcium gluconate followed by oral calcium supplements and made a full recovery 48 hours after the incident. Although no specific treatment exists in Srilanka, it has been suggested that calcium supplements may be beneficial to relieve the muscle spasms. Our patient made a full recovery with calcium supplements suggesting the treatment wit...

Acute Cervical Dystonia Induced by Clebopride

OpenAIRE

Choi, Jin Kyo; Hong, Jin Yong

2017-01-01

Antidopaminergic drugs are known to induce extrapyramidal symptoms. Clebopride, a dopamine antagonist, also can produce parkinsonism, tardive dyskinesia, tardive dystonia, hemifacial dystonia, or oculogyric crisis; however, acute dystonic reaction caused by clebopride has not been reported in adults. We report two young men who experienced acute cervical dystonia within a few days of taking clebopride. The patients recovered after discontinuation of the drug. Physicians prescribing clebopride...

Pseudomyopia in a patient with blocked ventriculo-peritoneal shunt--a case report.

Science.gov (United States)

Voon, L W; Goh, K Y; Lim, T H; Tan, K K; Yong, V S

1997-03-01

Accommodative spasm usually encompasses a classical triad of pseudomyopia, esodeviation and pupillary constriction. Accommodative spasm is most often psychogenic in nature; however, it may be associated with other organic diseases of which a rare cause is that of intracranial catheter complications. We report a case of dorsal midbrain syndrome with pseudomyopia in a patient with a blocked ventriculo-peritoneal shunt inserted for aqueductal stenosis. Clinical presentation was unusual in this patient as pseudomyopia occurred with exodeviation and without pupillary constriction.

Cost analysis of the use of botulinum toxin type A in Spain

Directory of Open Access Journals (Sweden)

F. de Andrés-Nogales

2014-05-01

Full Text Available Objective: To estimate treatment costs of blepharospasm, cervical dystonia(CD, upper limb spasticity (ULS and spasticity in children withcerebral palsy (SCCP with botulinum neurotoxin type A (BoNT-A inSpain. Method: Annual BoNT-A treatment costs were calculated (2013 ex-factoryprice ( applying RDL 8/2010 and RDL 9/2011 deductions, basedon initial dose (id, average dose (ad and maximum dose (md accordingto Summary of Product Characteristics of Botox® (100U/50U, Dysport®(500U and Xeomin® (100U and considering the use of complete vials.In addition, annual treatment costs were calculated considering the useof vials in more than one patient and also patient population annualtreatment costs based on diseases’ prevalence. Results: Annual BoNT-A treatment costs per patient were estimated atbetween 265 and 2,120 with savings from 10% to 55% accordingto the selected BoNT-A. CD and ULS treatment provided the greatestcost per patient. Botox® provided greater savings in ULS (id/ad, CD(id, and in blepharospasm and SCCP (id/ad/md. Dysport® treatmentwas less costly in CD (md and ULS (md, while Xeomin® was in CD(ad. Based on the estimated treated population in Spain, the annualtreatment costs ranged from 368,392 to 13,958,836 dependingon indication, dose and BoNT-A considered. Conclusions: The appropriate BoNT-A choice would lead to considerablesavings

Parry-Romberg syndrome: findings in advanced magnetic resonance imaging sequences - case report

Energy Technology Data Exchange (ETDEWEB)

Paula, Rafael Alfenas de; Ribeiro, Bruno Niemeyer de Freitas, E-mail: alfenas85@gmail.com [Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ (Brazil). Hospital Universitario Clementino Fraga Filho; Bahia, Paulo Roberto Valle [Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ (Brazil). Dept. de radiologia; Ribeiro, Renato Niemeyer de Freitas [Hospital de Clinica de Jacarepagua, Rio de Janeiro, RJ (Brazil); Carvalho, Lais Balbi de [Universidade Presidente Antonio Carlos (Unipac), Juiz de Fora, MG (Brazil)

2014-05-15

Parry-Romberg syndrome is a rare disease characterized by progressive hemifacial atrophy associated with other systemic changes, including neurological symptoms. Currently, there are few studies exploring the utilization of advanced magnetic resonance sequences in the investigation of this disease. The authors report the case of a 45-year-old patient and describe the findings at structural magnetic resonance imaging and at advanced sequences, correlating them with pathophysiological data. (author)

A case report of Parry Romberg Syndrome initially presenting as periodontitis

OpenAIRE

Taylor, Greig; Culshaw, Shauna; Armas, Jose; Savarrio, Lee; Goodall, Christine

2017-01-01

Parry Romberg Syndrome (PRS) is a rare disorder of progressive hemifacial atrophy, involving soft tissues, fat and occasionally bone. It can co-exist with presentations of Morphea. We describe an unusual case of persistent periodontal and alveolar destruction associated with PRS. A 56-year-old African female initially presented with persistent periodontal destruction, which showed minimal response to conventional periodontal treatment. After non-surgical treatment, surgical debridement follow...

Clinical usefulness of hyperventilation-loaded SPECT in coronary artery disease

International Nuclear Information System (INIS)

Taya, Makoto

1994-01-01

The purpose of this study was twofold: (1) to assess the clinical usefulness of hyperventilation-loaded Tl-201 myocardial single photon emission computed tomography (HV-SPECT) in detecting coronary spasm; and (2) to correlate coronary spasm with ischemic heart diseases. A total of 138 patients were examined. They were divided into Group 1 of a total of 69 patients with chest pain at rest, consisting of 25 with vasospastic angina and 44 with atypical chest pain; Group 2 of 37 patients with angina pectoris; and Group 3 of 32 patients with myocardial infarction. In Group I, HV-SPECT was positive in 96% (24/25) for vasospastic angina and 50% (22/44) for atypical angina pectoris. When thallium defect scores 2 or more were interpreted as a decreased thallium uptake, Tl-201 HV-SPECT had a sensitivity of 72%, a specificity of 93%, and an accuracy of 85.6% in diagnosing vasospastic angina. In Group 2, HV-SPECT showed a significantly higher positive rate in patients with resting angina pectoris than those with effort angina pectoris (69% vs 22%). In Group 3, HV-SPECT was a positive rate of 53%. When Nifedipine was administered, the positive cases turned to be negative in 47%. HV-SPECT proved to be useful as a noninvasive method for diagnosing coronary spasm. Coronary spasm was suggested to be present in ischemic heart disease other than vasospastic angina and atypical chest pain. (N.K.)

Prefabricated neck expanded skin flap with the superficial temporal vessels for facial resurfacing.

Science.gov (United States)

Lazzeri, Davide; Su, Weijie; Qian, Yunliang; Messmer, Caroline; Agostini, Tommaso; Spinelli, Giuseppe; Marcus, Jeffrey R; Levin, L Scott; Zenn, Micheal R; Zhang, Yi Xin

2013-05-01

The achievement of a normal-appearing face after surgical resurfacing remains an elusive goal. This is due in part to insufficient color matching, restoration of contours, and the persistence of visible scars. Flap prefabrication is a staged procedure that provides an independent axial blood supply to local expanded tissues. We describe a new reconstructive alternative with superior reconstructive surgical options for facial resurfacing that better matches damaged or discarded facial tissues. A superficial temporal fascial flap was harvested as the vascular supply of the prefabricated neck flap and located in a subcutaneous neck pocket over a tissue expander. After a 5-month period for expansion and maturation, the prefabricated skin flap was raised, islanded, and rotated to resurface the facial defect. Four patients with hemifacial postburn contracture and two patients affected by hemifacial vascular malformations aged 17 to 42 years (mean 29 years) were successfully treated with no major complication after a mean period of 15 months. Prefabricated neck-expanded skin flap demonstrated an excellent color and texture match with facial skin that surrounded the repair sites, and optimal aesthetic results were obtained. Importantly, facial expression was completely maintained due to thinness and pliability of the rotated skin. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Neck pain or spasms - self care

Science.gov (United States)

Pain - neck - self-care; Neck stiffness - self-care; Cervicalgia - self-care; Whiplash - self-care ... some pharmacies or retail stores. Ask your health care provider about using a soft neck collar to ...

What Is a Coronary Artery Spasm?

Science.gov (United States)

... medications such as: Nitrates, which can relieve chest pain Calcium channel blockers, which can help reduce muscle tightening in your chest Statin medications, which not only lower cholesterol, but have ...

Caring for muscle spasticity or spasms

Science.gov (United States)

... provider tells you to. Be careful about getting pressure sores from exercise or being in the same position ... Instructions Brain surgery - discharge Multiple sclerosis - discharge Preventing pressure ulcers Stroke - discharge Review Date 5/21/2016 Updated ...

Botulinum toxin for treatment of Frey's syndrome: report of two cases

Directory of Open Access Journals (Sweden)

Teive Hélio A.G.

2003-01-01

Full Text Available Frey's syndrome is a phenomenon of hemifacial flushing and sweating after gustatory stimulus, usually secondary to surgical trauma over the parotid gland, although other injury mechanisms may be seen. It is accepted as a result of aberrant regeneration of facial autonomic nerve fibers. Treatment evolved from ineffective medical and surgical approaches to botulinum toxin. We evaluate the effectiveness and safety of botulinum toxin in the treatment of this complication in two patients.

Single-fraction Radiosurgery for Presumed Intracranial Meningiomas: Efficacy and Complications From a 22-Year Experience

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Pollock, Bruce E., E-mail: pollock.bruce@mayo.edu [Department of Neurological Surgery, Mayo Clinic College of Medicine, Rochester, MN (United States); Department of Radiation Oncology, Mayo Clinic College of Medicine, Rochester, MN (United States); Stafford, Scott L. [Department of Radiation Oncology, Mayo Clinic College of Medicine, Rochester, MN (United States); Link, Michael J. [Department of Neurological Surgery, Mayo Clinic College of Medicine, Rochester, MN (United States); Garces, Yolanda I.; Foote, Robert L. [Department of Radiation Oncology, Mayo Clinic College of Medicine, Rochester, MN (United States)

2012-08-01

Purpose: To define the rate of tumor control and factors associated with radiation-related complications after single-fraction radiosurgery (SRS) for patients with imaging defined intracranial meningiomas. Materials and Methods: Retrospective review of 251 patients (192 women, 59 men) having SRS for imaging-defined intracranial meningiomas between 1990 and 2008. Excluded were patients with radiation-induced tumors, meningiomatosis, or neurofibromatosis. The mean patient age was 58.6 {+-} 13.4 years. The majority of tumors involved the skull base/tentorium (n = 210, 83.7%). The mean treatment volume was 7.7 {+-} 6.2 cm{sup 3}; the mean tumor margin dose was 15.8 {+-} 2.0 Gy. Follow-up (mean, 62.9 {+-} 43.9 months) was censored at last evaluation (n = 224), death (n = 22), or tumor resection (n = 5). Results: No patient died from tumor progression or radiation-related complications. Tumor size decreased in 181 patients (72.1%) and was unchanged in 67 patients (26.7%). Three patients (1.2%) had in-field tumor progression noted at 28, 145, and 150 months, respectively. No patient had a marginal tumor progression. The 3- and 10-year local control rate was 99.4%. One patient had distant tumor progression at 105 months and underwent repeat SRS. Thirty-one patients (12.4%) had either temporary (n = 8, 3.2%) or permanent (n = 23, 9.2%) symptomatic radiation-related complications including cranial nerve deficits (n = 14), headaches (n = 5), hemiparesis (n = 5), new/worsened seizure (n = 4), cyst-formation (n = 1), hemifacial spasm (n = 1), and stroke (n = 1). The 1- and 5-year complication rates were 8.3% and 11.5%, respectively. Radiation-related complications were associated with convexity/falx tumors (HR = 2.8, 95% CI 1.3-6.1, p = 0.009) and increasing tumor volume (HR = 1.05, 95% CI 1.0-1.1, p = 0.04) on multivariate analysis. No patient developed a radiation-induced tumor. Conclusions: Single-fraction SRS at the used dose range provides a high rate of tumor control for

Single-fraction Radiosurgery for Presumed Intracranial Meningiomas: Efficacy and Complications From a 22-Year Experience

International Nuclear Information System (INIS)

Pollock, Bruce E.; Stafford, Scott L.; Link, Michael J.; Garces, Yolanda I.; Foote, Robert L.

2012-01-01

Purpose: To define the rate of tumor control and factors associated with radiation-related complications after single-fraction radiosurgery (SRS) for patients with imaging defined intracranial meningiomas. Materials and Methods: Retrospective review of 251 patients (192 women, 59 men) having SRS for imaging-defined intracranial meningiomas between 1990 and 2008. Excluded were patients with radiation-induced tumors, meningiomatosis, or neurofibromatosis. The mean patient age was 58.6 ± 13.4 years. The majority of tumors involved the skull base/tentorium (n = 210, 83.7%). The mean treatment volume was 7.7 ± 6.2 cm 3 ; the mean tumor margin dose was 15.8 ± 2.0 Gy. Follow-up (mean, 62.9 ± 43.9 months) was censored at last evaluation (n = 224), death (n = 22), or tumor resection (n = 5). Results: No patient died from tumor progression or radiation-related complications. Tumor size decreased in 181 patients (72.1%) and was unchanged in 67 patients (26.7%). Three patients (1.2%) had in-field tumor progression noted at 28, 145, and 150 months, respectively. No patient had a marginal tumor progression. The 3- and 10-year local control rate was 99.4%. One patient had distant tumor progression at 105 months and underwent repeat SRS. Thirty-one patients (12.4%) had either temporary (n = 8, 3.2%) or permanent (n = 23, 9.2%) symptomatic radiation-related complications including cranial nerve deficits (n = 14), headaches (n = 5), hemiparesis (n = 5), new/worsened seizure (n = 4), cyst-formation (n = 1), hemifacial spasm (n = 1), and stroke (n = 1). The 1- and 5-year complication rates were 8.3% and 11.5%, respectively. Radiation-related complications were associated with convexity/falx tumors (HR = 2.8, 95% CI 1.3–6.1, p = 0.009) and increasing tumor volume (HR = 1.05, 95% CI 1.0–1.1, p = 0.04) on multivariate analysis. No patient developed a radiation-induced tumor. Conclusions: Single-fraction SRS at the used dose range provides a high rate of tumor control for patients

Detection of brain atrophy due to ACTH or corticosteroid therapy with computed tomography

International Nuclear Information System (INIS)

Tamai, Isamu; Takei, Tadao; Oota, Hideomi; Maekawa, Kihei.

1981-01-01

Adrenocorticotropic hormone (ACTH) or corticosteroids seemed to cause brain atrophy in intants. We studied the atrophy which was caused by these drugs with computed tomography (CT). 1) Nine cases of infantile spasms examined before, during and after ACTH therepy with CT. Brain atrophy on CT was observed immediately after the completion of ACTH therapy. The brain atrophy receded slightly after several months. It was more marked in younger patients, in cases treated by hight doses of ACTH and in cases where brain atrophy had already been obserbed before ACTH therapy. 2) Twenty cases of infantile spasms or Lennox Gastaut syndrome were examined after ACTH therapy with CT. Brain atrophy was observed in twelve cases. Main features of brain atrophy were the enlargement of sylvian fissure and the widening of subarachnoid space at the frontal or temporal region. Mental retardation was observed in eighteen cases. 3) Two cases of nephrotic syndrome were treated with pulse therapy of prednisolone. CT was carried out before and after treatment. Atrophy of cerebrum was observed in these cases. 4) A case of infantile spasms treated with anticonvulsants without ACTH was studied by electroencephalography (EEG) and CT. The abnormal pattern of EEG was markedly corrected, while brain atrophy on CT was not observed after the therapy. Because of these observations the use of ACTH has to be reconsidered. ACTH should be the drug of second choice for the therapy of infantile spasms and should be used in case other anticonvulsants have no effect. ACTH should be used at lower dosages and for shorter periods of time. (author)

Detection of brain atrophy due to ACTH or corticosteroid therapy with computed tomography

Energy Technology Data Exchange (ETDEWEB)

Tamai, I.; Takei, T. (National Sagamihara Hospital, Kanagawa (Japan)); Oota, H.; Maekawa, K.

1981-07-01

Adrenocorticotropic hormone (ACTH) or corticosteroids seemed to cause brain atrophy in infants. We studied the atrophy which was caused by these drugs with computed tomography (CT). 1) Nine cases of infantile spasms examined before, during and after ACTH therapy with CT. Brain atrophy on CT was observed immediately after the completion of ACTH therapy. The brain atrophy receded slightly after several months. It was more marked in younger patients, in cases treated by high doses of ACTH and in cases where brain atrophy had already been observed before ACTH therapy. 2) Twenty cases of infantile spasms or Lennox Gastaut syndrome were examined after ACTH therapy with CT. Brain atrophy was observed in twelve cases. Main features of brain atrophy were the enlargement of sylvian fissure and the widening of subarachnoid space at the frontal or temporal region. Mental retardation was observed in eighteen cases. 3) Two cases of nephrotic syndrome were treated with pulse therapy of prednisolone. CT was carried out before and after treatment. Atrophy of cerebrum was observed in these cases. 4) A case of infantile spasms treated with anticonvulsants without ACTH was studied by electroencephalography (EEG) and CT. The abnormal pattern of EEG was markedly corrected, while brain atrophy on CT was not observed after the therapy. Because of these observations the use of ACTH has to be reconsidered. ACTH should be the drug of second choice for the therapy of infantile spasms and should be used in case other anticonvulsants have no effect. ACTH should be used at lower dosages and for shorter periods of time.

Transarterial lidocaine-lipiodol emulsion administration for relief of pain during transarterial chemoembolization of malignant tumor

International Nuclear Information System (INIS)

Wu Anle; Yan Zhiping; Zhou Kangrong; Wang Jianhua; Cheng Jiemin; Qian Sheng; Luo Jianjun; Chen Yi

2004-01-01

Objective: To assess the feasibility and efficacy of transarterial lidocaine-lipiodol emulsion administration for controlling abdominal pain and preventing the arterial spasm resulting from TACE, and to evaluate the optimal amount of lidocaine administration. Methods: In a prospective trial of 120 consecutive patients with malignant tumor who underwent TACE were divided into three groups, those who received lidocaine-lipiodol emulsion administration (group A, n=40), those who received lidocaine bolus intraarterial infusion immediately before TACE (group B, n=40) and those who received no lidocaine injection before TACE, (group C, n=40). The degree of post-procedure pain was evaluated by a subjective method (using visual analogue scales from 0 to 10), and an objective method (amount of post-procedure analgesics). Incidence and degree of arterial spasm were assessed by DSA. Results: The correlative pain incidences between the three groups showed significant difference (P 0.05). Mean dose of intramuscular analgesics for controlling intolerable pain in group A and B was significantly lower than that of group C (P<0.05). There was no difference in the incidence of arterial spasm between group A and B but it was much lower in group C. Lipiodol deposit in malignant mass was densest in group A, especially in the metastatic nodules of the liver. Conclusions: Transarterial administration of lidocaine-lipiodol emulsion can not only reduce the incidence of pain during TACE, but also prevent the arterial spasm. It is much more effective than pre-TACE administration of pethidine and intraarterial infusion of lidocaine. The authors recommond routinely for the administration of lidocaine-lipiodol emulsion. (authors)

Acute Cervical Dystonia Induced by Clebopride.

Science.gov (United States)

Choi, Jin Kyo; Hong, Jin Yong

2017-01-01

Antidopaminergic drugs are known to induce extrapyramidal symptoms. Clebopride, a dopamine antagonist, also can produce parkinsonism, tardive dyskinesia, tardive dystonia, hemifacial dystonia, or oculogyric crisis; however, acute dystonic reaction caused by clebopride has not been reported in adults. We report two young men who experienced acute cervical dystonia within a few days of taking clebopride. The patients recovered after discontinuation of the drug. Physicians prescribing clebopride should be aware of the adverse effects of this drug.

Acute Cervical Dystonia Induced by Clebopride

Directory of Open Access Journals (Sweden)

Jin Kyo Choi

2017-01-01

Full Text Available Antidopaminergic drugs are known to induce extrapyramidal symptoms. Clebopride, a dopamine antagonist, also can produce parkinsonism, tardive dyskinesia, tardive dystonia, hemifacial dystonia, or oculogyric crisis; however, acute dystonic reaction caused by clebopride has not been reported in adults. We report two young men who experienced acute cervical dystonia within a few days of taking clebopride. The patients recovered after discontinuation of the drug. Physicians prescribing clebopride should be aware of the adverse effects of this drug.

Warm-needle moxibustion for spasticity after stroke: A systematic review of randomized controlled trials.

Science.gov (United States)

Yang, Liu; Tan, Jing-Yu; Ma, Haili; Zhao, Hongjia; Lai, Jinghui; Chen, Jin-Xiu; Suen, Lorna K P

2018-03-22

Spasticity is a common post-stroke complication, and it results in substantial deterioration in the quality of life of patients. Although potential positive effects of warm-needle moxibustion on spasticity after stroke have been observed, evidence on its definitive effect remains uncertain. This study aimed to summarize clinical evidence pertaining to therapeutic effects and safety of warm-needle moxibustion for treating spasticity after stroke. Randomized controlled trials were reviewed systematically on the basis of the Cochrane Handbook for Systematic Reviews of Interventions. The report follows the PRISMA statement. Ten electronic databases (PubMed, CENTRAL, EMBASE, AMED, CINAHL, Web of Science, CBM, CNKI, WanFang, and VIP) were explored, and articles were retrieved manually from two Chinese journals (The Journal of Traditional Chinese Medicine and Zhong Guo Zhen Jiu) through retrospective search. Randomized controlled trials with warm-needle moxibustion as treatment intervention for patients with limb spasm after stroke were included in this review. The risk of bias assessment tool was utilized in accordance with Cochrane Handbook 5.1.0. All included studies reported spasm effect as primary outcome. Effect size was estimated using relative risk, standardized mean difference, or mean difference with a corresponding 95% confidence interval. Review Manager 5.3 was utilized for meta-analysis. Twelve randomized controlled trials with certain methodological flaws and risk of bias were included, and they involved a total of 878 participants. Warm-needle moxibustion was found to be superior to electroacupuncture or acupuncture in reducing spasm and in promoting motor function and daily living activities. Pooled results for spasm effect and motor function were significant when warm-needle moxibustion was compared with electroacupuncture or acupuncture. A comparison of daily living activities indicated significant differences between warm-needle moxibustion and

A rare case of gestational thyrotoxicosis as a cause of acute myocardial infarction

Directory of Open Access Journals (Sweden)

Varalaxmi Bhavani Nannaka

2016-11-01

Full Text Available Angina pectoris in pregnancy is unusual and Prinzmetal’s angina is much rarer. It accounts for 2% of all cases of angina. It is caused by vasospasm, but the mechanism of spasm is unknown but has been linked with hyperthyroidism in some studies. Patients with thyrotoxicosis-induced acute myocardial infarction are unusual and almost all reported cases have been associated with Graves’ disease. Human chorionic gonadotropin hormone-induced hyperthyroidism occurs in about 1.4% of pregnant women, mostly when hCG levels are above 70–80 000 IU/L. Gestational transient thyrotoxicosis is transient and generally resolves spontaneously in the latter half of pregnancy, and specific antithyroid treatment is not required. Treatment with calcium channel blockers or nitrates reduces spasm in most of these patients. Overall, the prognosis for hyperthyroidism-associated coronary vasospasm is good. We describe a very rare case of an acute myocardial infarction in a 27-year-old female, at 9 weeks of gestation due to right coronary artery spasm secondary to gestational hyperthyroidism with free thyroxine of 7.7 ng/dL and TSH <0.07 IU/L.

Comparative study of 3D TOF-SPGR and 3D FASE on the display of the relationship between cranial nerves and peripheral vessels

International Nuclear Information System (INIS)

Sun Wenge; Li Yanliang; Zhang Lina; Qi Xixun; Jin Anyu; Xu Ke; Tong Zhiyong; Liu Jing

2004-01-01

Objective: To select a better sequence which can supply reliable radiological information for vessel compression on cranial nerves in patients with facial spasm or trigeminal neuralgia. Methods: 3D TOF-SPGR and 3D FASE were used in 40 patients with facial spasm or trigeminal neuralgia to display the relationship between cranial nerves (facial nerve and trigeminal nerve) and peripheral vessels. Results: 38 patients got surgical results. 33 unilateral vessel compression or contact on facial nerves or trigeminal nerves was found on 3D TOF-SPGR, while no contact was found in 5. 26 unilateral vessel compression or contact on facial nerves or trigeminal nerves was found on 3D FASE, while no contact was found in 12. Significant difference between the two sequences on the display of vessel compression on facial nerves or trigeminal nerves was found by statistical analysis (χ 2 =5.14, P=0.016). Conclusion: 3D TOF-SPGR is better than 3D FASE on display of the relationship between nerves and peripheral vessels, and it should be the primary MRI sequence for patients with facial spasm or trigeminal neuralgia clinically. (author)

The use of three-dimensional imaging to evaluate the effect of conventional orthodontic approach in treating a subject with facial asymmetry

OpenAIRE

Kheir, Nadia Abou; Kau, Chung How

2016-01-01

The growth of the craniofacial skeleton takes place from the 3rd week of intra-uterine life until 18 years of age. During this period, the craniofacial complex is affected by extrinsic and intrinsic factors which guide or alter the pattern of growth. Asymmetry can be encountered due to these multifactorial effects or as the normal divergence of the hemifacial counterpart occurs. At present, an orthodontist plays a major role not only in diagnosing dental asymmetry but also facial asymmetry. H...

Radiological and orthopedic abnormalities in Satoyoshi syndrome

Energy Technology Data Exchange (ETDEWEB)

Haymon, M.L. [Children`s Hospital, New Orleans, LA (United States). Dept. of Radiology; Willis, R.B. [Children`s Hospital, New Orleans, LA (United States). Dept. of Orthopedics; Ehlayel, M.S. [Div. of Genetics, Dept. of Pediatrics, Louisiana State Univ. Medical Center, Orleans, LA (United States)]|[Louisiana State Medical Center, New Orleans, LA (United States). Center for Molecular and Human Genetics; Lacassie, Y. [Div. of Genetics, Dept. of Pediatrics, Louisiana State Univ. Medical Center, Orleans, LA (United States)]|[Louisiana State Medical Center, New Orleans, LA (United States). Center for Molecular and Human Genetics]|[Children`s Hospital, New Orleans, LA (United States). Dept. of Pediatrics

1997-05-01

Satoyoshi syndrome is a are disorder on unknown etiology characterized by progressive, painful intermittent muscle spasms, serve skeletal abnormalities mimicking a skeletal dyplasia, malabsorption, alopecia, and amenorrhea. We further report on a 20{sup 1}/{sub 2}-year-old Caucasian woman whith characteristic manifestation of the syndrome. Since the establishment of the diagnostic 1 year ago, she has been treated with prednisone with good response. However, treatment of the multiple deformities and fractures has been difficult and challenging. The early recognition and treatment of this disorder is of utmost importance, as the skeletal deformities and fractures seem to be secondary to the muscular spasms, as suggested by Satoyoshi.

Radiological and orthopedic abnormalities in Satoyoshi syndrome

International Nuclear Information System (INIS)

Haymon, M.L.; Willis, R.B.; Ehlayel, M.S.; Louisiana State Medical Center, New Orleans, LA; Lacassie, Y.; Louisiana State Medical Center, New Orleans, LA; Children's Hospital, New Orleans, LA

1997-01-01

Satoyoshi syndrome is a are disorder on unknown etiology characterized by progressive, painful intermittent muscle spasms, serve skeletal abnormalities mimicking a skeletal dyplasia, malabsorption, alopecia, and amenorrhea. We further report on a 20 1 / 2 -year-old Caucasian woman whith characteristic manifestation of the syndrome. Since the establishment of the diagnostic 1 year ago, she has been treated with prednisone with good response. However, treatment of the multiple deformities and fractures has been difficult and challenging. The early recognition and treatment of this disorder is of utmost importance, as the skeletal deformities and fractures seem to be secondary to the muscular spasms, as suggested by Satoyoshi

Temporal discrimination thresholds in adult-onset primary torsion dystonia: an analysis by task type and by dystonia phenotype.

LENUS (Irish Health Repository)

Bradley, D

2012-01-01

Adult-onset primary torsion dystonia (AOPTD) is an autosomal dominant disorder with markedly reduced penetrance. Sensory abnormalities are present in AOPTD and also in unaffected relatives, possibly indicating non-manifesting gene carriage (acting as an endophenotype). The temporal discrimination threshold (TDT) is the shortest time interval at which two stimuli are detected to be asynchronous. We aimed to compare the sensitivity and specificity of three different TDT tasks (visual, tactile and mixed\\/visual-tactile). We also aimed to examine the sensitivity of TDTs in different AOPTD phenotypes. To examine tasks, we tested TDT in 41 patients and 51 controls using visual (2 lights), tactile (non-painful electrical stimulation) and mixed (1 light, 1 electrical) stimuli. To investigate phenotypes, we examined 71 AOPTD patients (37 cervical dystonia, 14 writer\\'s cramp, 9 blepharospasm, 11 spasmodic dysphonia) and 8 musician\\'s dystonia patients. The upper limit of normal was defined as control mean +2.5 SD. In dystonia patients, the visual task detected abnormalities in 35\\/41 (85%), the tactile task in 35\\/41 (85%) and the mixed task in 26\\/41 (63%); the mixed task was less sensitive than the other two (p = 0.04). Specificity was 100% for the visual and tactile tasks. Abnormal TDTs were found in 36 of 37 (97.3%) cervical dystonia, 12 of 14 (85.7%) writer\\'s cramp, 8 of 9 (88.8%) blepharospasm, 10 of 11 (90.1%) spasmodic dysphonia patients and 5 of 8 (62.5%) musicians. The visual and tactile tasks were found to be more sensitive than the mixed task. Temporal discrimination threshold results were comparable across common adult-onset primary torsion dystonia phenotypes, with lower sensitivity in the musicians.

Facial nerve problems and Bell's palsy

OpenAIRE

Sala, DV; Venter, C; Valenas, O

2015-01-01

Bell's palsy is paralysis or weakness of muscle at the hemifacial level, a form of temporary facial paralysis, probable a virus infection or trauma, to one or two facial nerves. Damage to the facial nerve innervating the muscles on one side of the face result in a flabby appearance, fell the respective hemiface. Nerve damage can also affect the sense of taste and salivary and lacrimal secretion. This condition begins suddenly, often overnight, and usually gets better on its own within a few w...

A PATIENT DENGUE HEMORRHAGIC FEVER WITH SPASMS

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Ulfa Kholili

2014-09-01

Full Text Available Indonesia is one of the countries with the high endemic of Dengue viral infection followed by Thailand, Myanmar, India and Srilanka. For more 10-15 years, Dengue Viral Infection/DHF has become a cause of patient who should be hospitalized and was the first cause of death children in south easthern Asia.1,2 Batavia was the first city of Indonesia found Dengue Viral infection which had been written in journal by David Bylon in the 1779. Encephalopathy of dengue (ED is one unusually complication of dengue viral infection which had been characterized by aberration the arrangement of nerves central (CNS. This paper want to describe of a young teenage with suffer from DHF and seizure. Beside it, pleural effusion and cerebral edema had been found. Seizure most likely due to dengue encephalopathy associated with cerebral edema and was supported by positive IgG and IgM anti dengue. Corticosteroid was given to improve cerebral edema. By good management as long as admission, she was discharged from hospital with a good condition.

Genetics of Severe Early Onset Epilepsies

Science.gov (United States)

2017-08-24

Epilepsy; Epileptic Encephalopathy; Ohtahara Syndrome; Infantile Spasms; Dravet Syndrome; Malignant Migrating Partial Epilepsy of Infancy; Early Myoclonic Epileptic Encephalopathy; PCDH19-related Epilepsy and Related Conditions

Effects of Milrinone continuous intravenous infusion on global cerebral oxygenation and cerebral vasospasm after cerebral aneurysm surgical clipping

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Mohamed A. Ghanem

2014-01-01

Conclusions: Milrinone improved significantly the global cerebral oxygenation and reduced the incidence of cerebral vasospasm during the dangerous period of cerebral spasm after cerebral aneurysm clipping.

Belladonna

Science.gov (United States)

... asthma. Belladonna is also used as suppositories for hemorrhoids. ... condition. Arthritis-like pain. Asthma. Colds. Hay fever. Hemorrhoids. Motion sickness. Nerve problems. Parkinson's disease. Spasms and ...

Hydrotherapy for the Treatment of Pain in People with Multiple Sclerosis: A Randomized Controlled Trial

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Adelaida María Castro-Sánchez

2012-01-01

Full Text Available Background. Multiple sclerosis (MS is a chronic demyelinating neurological disease. Several studies have reported that complementary and alternative therapies can have positive effects against pain in these patients. Objective. The objective was to investigate the effectiveness of an Ai-Chi aquatic exercise program against pain and other symptoms in MS patients. Methods. In this randomized controlled trial, 73 MS patients were randomly assigned to an experimental or control group for a 20-week treatment program. The experimental group underwent 40 sessions of Ai-Chi exercise in swimming pool and the control group 40 sessions of abdominal breathing and contraction-relaxation exercises in therapy room. Outcome variables were pain, disability, spasm, depression, fatigue, and autonomy, which were assessed before the intervention and immediately and at 4 and 10 weeks after the last treatment session. Results. The experimental group showed a significant (P<0.028 and clinically relevant decrease in pain intensity versus baseline, with an immediate posttreatment reduction in median visual analogue scale scores of 50% that was maintained for up to 10 weeks. Significant improvements were also observed in spasm, fatigue, disability, and autonomy. Conclusion. According to these findings, an Ai-Chi aquatic exercise program improves pain, spasms, disability, fatigue, depression, and autonomy in MS patients.

Hydrotherapy for the treatment of pain in people with multiple sclerosis: a randomized controlled trial.

Science.gov (United States)

Castro-Sánchez, Adelaida María; Matarán-Peñarrocha, Guillermo A; Lara-Palomo, Inmaculada; Saavedra-Hernández, Manuel; Arroyo-Morales, Manuel; Moreno-Lorenzo, Carmen

2012-01-01

Background. Multiple sclerosis (MS) is a chronic demyelinating neurological disease. Several studies have reported that complementary and alternative therapies can have positive effects against pain in these patients. Objective. The objective was to investigate the effectiveness of an Ai-Chi aquatic exercise program against pain and other symptoms in MS patients. Methods. In this randomized controlled trial, 73 MS patients were randomly assigned to an experimental or control group for a 20-week treatment program. The experimental group underwent 40 sessions of Ai-Chi exercise in swimming pool and the control group 40 sessions of abdominal breathing and contraction-relaxation exercises in therapy room. Outcome variables were pain, disability, spasm, depression, fatigue, and autonomy, which were assessed before the intervention and immediately and at 4 and 10 weeks after the last treatment session. Results. The experimental group showed a significant (P < 0.028) and clinically relevant decrease in pain intensity versus baseline, with an immediate posttreatment reduction in median visual analogue scale scores of 50% that was maintained for up to 10 weeks. Significant improvements were also observed in spasm, fatigue, disability, and autonomy. Conclusion. According to these findings, an Ai-Chi aquatic exercise program improves pain, spasms, disability, fatigue, depression, and autonomy in MS patients.

Imaging of cocaine-induced global and regional myocardial ischemia

International Nuclear Information System (INIS)

Oster, Z.H.; Som, P.; Wang, G.J.; Weber, D.A.

1991-01-01

Severe and often fatal cardiac complications have been reported in cocaine users with narrowed coronary arteries caused by atherosclerosis as well as in young adults with normal coronaries. The authors have found that in normal dogs cocaine induces severe temporary hypoperfusion of the left ventricle as indicated by a significantly lower 201Tl concentration compared to the baseline state. The most significant decrease in uptake occurred 5 min after injection and was more pronounced in the septal and apical segments. Following intravenous administration of cocaine, instead of gradual disappearance of 201Tl from the left ventricle, there was continuous increase in 201Tl concentration in the left ventricle. These imaging experiments indicate that the deleterious effects of cocaine on the heart are probably due to spasm of the coronaries and decreased myocardial perfusion. Since spasm of the large subpericardial vessels does not seem to explain the magnitude of the increased coronary resistance and decreased coronary flow after cocaine as described in the literature, it is suggested that microvascular spasm of smaller vessels plays a major role in the temporary decrease in perfusion. The data may also suggest that severe temporary myocardial ischemia is probably the initiating factor for the cardiac complications induced by cocaine

Barium enema findings of milk allergy in infants

International Nuclear Information System (INIS)

Kim, Gyoung Ju; Kim, Mi Jeong; Lee, Hee Jung

2006-01-01

We wanted to evaluate the barium enema findings of milk allergy in infants. Retrospective evaluation of the plain abdominal radiography and barium enema findings was performed in fifteen young infants suffering with milk allergy. The presence of gaseous distension, rectal gas, paralytic ileus and mechanical obstruction was evaluated on the plain radiography. The presence of spasm, a transitional zone, a reversed rectosigmoid index and mucosal irregularity was analyzed on the barium enema; the presence of barium retention was also evaluated on 24-hour-delayed plain radiography. Paralytic ileus was the most common finding on the plain radiography (93%). On the barium enema, continuous spasm of the colon, ranging from the rectum to the descending colon, was revealed in ten infants (67%). A transitional zone was observed in one infant and a reversed rectosigmoid index was revealed in four. Mucosal irregularity was observed in two infants. Barium retention was demonstrated in 11 of fifteen cases: throughout the entire colon (n = 3), from the rectum to the descending colon (n = 7), and up to the transverse colon (n = 1). The most common barium enema finding of milk allergy in infants was spasm of the distal colon. The other findings were a transitional zone, a reversed rectosigmoid index, mucosal irregularity and barium retention

Intrathecal Versus Oral Baclofen: A Matched Cohort Study of Spasticity, Pain, Sleep, Fatigue, and Quality of Life.

Science.gov (United States)

McCormick, Zachary L; Chu, Samuel K; Binler, Danielle; Neudorf, Daniel; Mathur, Sunjay N; Lee, Jungwha; Marciniak, Christina

2016-06-01

Baclofen commonly is used to manage spasticity caused by central nervous system lesions or dysfunction. Although both intrathecal and oral delivery routes are possible, no study has directly compared clinical outcomes associated with these 2 routes of treatment. To compare spasticity levels, pain, sleep, fatigue, and quality of life between individuals receiving treatment with intrathecal versus oral baclofen. Cross-sectional matched cohort survey study. Urban academic rehabilitation outpatient clinics. Adult patients with spasticity, treated with intrathecal or oral baclofen for at least 1 year, matched 1:1 for age, gender, and diagnosis. Standardized surveys were administered during clinic appointments or by telephone. Surveys included the Penn Spasm Frequency Scale, Brief Pain Inventory, Epworth Sleepiness Scale, Fatigue Severity Scale, Life Satisfaction Questionnaire, and Diener Satisfaction with Life Scale. A total of 62 matched subjects were enrolled. The mean (standard deviation [SD]) age was 46 (11) years with a mean duration of intrathecal baclofen or oral baclofen treatment of 11 (6) and 13 (11) years, respectively. There were 40 (64%) male and 22 (36%) female subjects. Primary diagnoses included spinal cord injury (n = 38), cerebral palsy (n = 10), stroke (n = 10), and multiple sclerosis (n = 4). The mean (SD) dose of intrathecal and oral baclofen at the time of survey were 577 (1429) μg/day and 86 (50) mg/day, respectively. Patients receiving intrathecal compared with oral baclofen experienced significantly fewer (1.44 [0.92] versus 2.37 [1.12]) and less severe (1.44 [0.92] versus 2.16 [0.83]) spasms, respectively as measured by the Penn Spasm Frequency Scale (P life between groups. Subanalysis of patients with SCI mirrored results of the entire study sample, with significant decreases in spasm frequency and severity associated with intrathecal compared to oral baclofen (P < .01; P < .01), but no other between group differences. The mean (SD) percent

Ectopic external auditory canal and ossicular formation in the oculo-auriculo-vertebral spectrum

Energy Technology Data Exchange (ETDEWEB)

Supakul, Nucharin [Indiana University School of Medicine, Department of Radiology, Indianapolis, IN (United States); Ramathibodi Hospital, Mahidol University, Department of Diagnostic and Therapeutic Radiology, Bangkok (Thailand); Kralik, Stephen F. [Indiana University School of Medicine, Department of Radiology, Indianapolis, IN (United States); Ho, Chang Y. [Indiana University School of Medicine, Department of Radiology, Indianapolis, IN (United States); Riley Children' s Hospital, MRI Department, Indianapolis, IN (United States)

2015-07-15

Ear abnormalities in oculo-auricular-vertebral spectrum commonly present with varying degrees of external and middle ear atresias, usually in the expected locations of the temporal bone and associated soft tissues, without ectopia of the external auditory canal. We present the unique imaging of a 4-year-old girl with right hemifacial microsomia and ectopic location of an atretic external auditory canal, terminating in a hypoplastic temporomandibular joint containing bony structures with the appearance of auditory ossicles. This finding suggests an early embryological dysfunction involving Meckel's cartilage of the first branchial arch. (orig.)

Determinación de HLA en pacientes con Síndrome de Parry Romberg atendidos en el Servicio de Cirugía Plástica y Reconstructiva del Hospital General "Dr. Manuel Gea González"

OpenAIRE

Palafox, Damián; Gutiérrez-Gómez, Claudia; Granados, Julio; Barquera, Rodrigo; Ortega-Hernández, Esteban; Hernández-Zaragoza, Diana I.; Pérez-Dosal, Marcia; Pacheco-Ubaldo, Hanna R.; Lona-Sánchez, Abraham

2016-01-01

Introducción y Objetivo. El síndrome de Parry Romberg se caracteriza por atrofia hemifacial progresiva; afecta piel y tejidos blandos y en ocasiones provoca también atrofia de músculos, cartílago y estructuras óseas subyacentes. Su diagnóstico diferencial incluye: esclerodermia generalizada, morfea y síndrome de CREST. Las alteraciones en piel se asocian con variantes alélicas (polimórficas) del sistema HLA (antígeno leucocitario humano). La esclerodermia se ha asociado con HLA DR11, el CREST...

Cranial nerve vascular compression syndromes of the trigeminal, facial and vago-glossopharyngeal nerves: comparative anatomical study of the central myelin portion and transitional zone; correlations with incidences of corresponding hyperactive dysfunctional syndromes.

Science.gov (United States)

Guclu, Bulent; Sindou, Marc; Meyronet, David; Streichenberger, Nathalie; Simon, Emile; Mertens, Patrick

2011-12-01

The aim of this study was to evaluate the anatomy of the central myelin portion and the central myelin-peripheral myelin transitional zone of the trigeminal, facial, glossopharyngeal and vagus nerves from fresh cadavers. The aim was also to investigate the relationship between the length and volume of the central myelin portion of these nerves with the incidences of the corresponding cranial dysfunctional syndromes caused by their compression to provide some more insights for a better understanding of mechanisms. The trigeminal, facial, glossopharyngeal and vagus nerves from six fresh cadavers were examined. The length of these nerves from the brainstem to the foramen that they exit were measured. Longitudinal sections were stained and photographed to make measurements. The diameters of the nerves where they exit/enter from/to brainstem, the diameters where the transitional zone begins, the distances to the most distal part of transitional zone from brainstem and depths of the transitional zones were measured. Most importantly, the volume of the central myelin portion of the nerves was calculated. Correlation between length and volume of the central myelin portion of these nerves and the incidences of the corresponding hyperactive dysfunctional syndromes as reported in the literature were studied. The distance of the most distal part of the transitional zone from the brainstem was 4.19  ±  0.81 mm for the trigeminal nerve, 2.86  ±  1.19 mm for the facial nerve, 1.51  ±  0.39 mm for the glossopharyngeal nerve, and 1.63  ±  1.15 mm for the vagus nerve. The volume of central myelin portion was 24.54  ±  9.82 mm(3) in trigeminal nerve; 4.43  ±  2.55 mm(3) in facial nerve; 1.55  ±  1.08 mm(3) in glossopharyngeal nerve; 2.56  ±  1.32 mm(3) in vagus nerve. Correlations (p  nerves and incidences of the corresponding diseases. At present it is rather well-established that primary trigeminal neuralgia, hemifacial spasm and vago

Multifocal dystonia, Clinical feature of Hallervorden-Spatz

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Ghelichkhani H

1998-09-01

Full Text Available Hallervorden-spatz disease is an inherited metabolic disorder with autosomal recessive trait. Onset is in late childhood or early adolescence. Clinical manifestation is variable but pyramidal and extrapyramidal signs are often prominent. Many of patients show progressive dementia and extrapyramidal symptoms. Ataxia or myoclonus is reported in the course of the disease in individual cases. Focal dystonias including tongue, eyelids (blepharospasm and optic atrophy, retinitis pigmentosa, rarely familial parkinsonism are also reported. Pathologically pigmentary degeneration of globus pallidus, substantia nigra (pars reticular and red nucleus is characteristic. In our case the main clinical feature was multifocal dystonia without obvious pyramidal or other extrapyramidal symptoms, and diagnosis was based on clinical and MRI findings.

Persistent pseudomyopia following a whiplash injury in a previously emmetropic woman

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Fintan E. Hughes

2017-12-01

It is important that such cases are identified at presentation, as early intervention can improve outcomes in accommodative spasm and reduce the long term psychological effects often associated with whiplash injuries.

21 CFR 890.5290 - Shortwave diathermy.

Science.gov (United States)

2010-04-01

... within body tissues for the treatment of selected medical conditions such as relief of pain, muscle spasms, and joint contractures, but not for the treatment of malignancies. (2) Classification. Class II...

21 CFR 890.5275 - Microwave diathermy.

Science.gov (United States)

2010-04-01

... within body tissues for the treatment of selected medical conditions such as relief of pain, muscle spasms, and joint contractures, but not for the treatment of malignancies. (2) Classification. Class II...

Paralisia facial periférica idiopática de Bell: a propósito de 180 pacientes Idiopathic facial paralysis (Bell´s palsy: a study of 180 patients

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Marcelo Moraes Valença

2001-09-01

with Bell´s palsy. In the study population there was a predominance of female (66.7%. Two peaks of incidence in the age distribution were identified: third-fourth and sixth decades of life. In the group of 180 patients there were 198 events of facial paralysis, 17 recurrences and in one patient the paralysis was bilateral at the onset. In 15 patients (8.3% there were recurrences of the facial paralysis, in 12 cases (70.6% the recurrences were ipsilateral. The left side of the face was involved in 55.6% of the cases. In eight patients the paralysis ocurred during pregnancy (n=5 or puerperium (n=3. As associated conditions we found: arterial hypertension (11.7%, diabetes mellitus (11.1%, pregnancy or puerperium (4.4%; 6.7% in the women, and neurocysticercosis (1.1%. In 72.8% of the cases no association with such conditions was found. In 22.8% of the patients some kind of sequelae were identified: hemifacial spasm (12.8%, partial recovery of the motor deficit (10.6%, syndrome of the crocodile tears (3.3%, sincinetic contration (2.8%, and the Marcus Gunn inverse phenomenon (1.1%. In conclusion, this study shows that the idiopathic facial paralysis may lead to important sequelae in more than 20% of the patients.

Antispasmodic and anti-inflammatory activity of Carrageenan from Hypnea musciformis Wulfen

Digital Repository Service at National Institute of Oceanography (India)

Solimabi; Das, B.

Pharmacological studies on K-carrageenan extracted from Hypnea musciformis have shown that it antagonizes histamine-induced spasm in guineapig ielum and possesses anti-inflammatory activity against rat hind paw oedema induced by commercial...

Middle cerebral artery vasospasm: transcranial color-coded duplex sonography versus conventional nonimaging transcranial Doppler sonography.

Science.gov (United States)

Swiat, Maciej; Weigele, John; Hurst, Robert W; Kasner, Scott E; Pawlak, Mikolaj; Arkuszewski, Michal; Al-Okaili, Riyadh N; Swiercz, Miroslaw; Ustymowicz, Andrzej; Opala, Grzegorz; Melhem, Elias R; Krejza, Jaroslaw

2009-03-01

To prospectively compare accuracies of transcranial color-coded duplex sonography (TCCS) and transcranial Doppler sonography (TCD) in the diagnosis of middle cerebral artery (MCA) vasospasm. Prospective blinded head-to-head comparison TCD and TCCS methods using digital subtraction angiography (DSA) as the reference standard. Department of Radiology in a tertiary university health center in a metropolitan area. Eighty-one consecutive patients (mean age, 53.9 +/- 13.9 years; 48 women). The indication for DSA was subarachnoid hemorrhage in 71 patients (87.6%), stroke or transient ischemic attack in five patients (6.2%), and other reasons in five patients (6.2%). The MCA was graded as normal, narrowed 50% using DSA. The accuracy of ultrasound methods was estimated by total area (Az) under receiver operator characteristic curve. To compare sensitivities of ultrasound methods, McNemar's test was used with mean velocity thresholds of 120 cm/sec for the detection of less advanced, and 200 cm/sec for the more advanced MCA narrowing. Angiographic MCA narrowing 50% in 10 of 135 arteries. Accuracy of TCCS was insignificantly higher than that of TCD in the detection of 50% narrowing, total Az for mean velocity being 0.83 +/- 0.05, 0.77 +/- 0.05, and 0.95 +/- 0.02, 0.86 +/- 0.08, respectively. Sensitivity of TCCS at commonly used threshold of 120 cm/sec for less advanced MCA spasm was significantly better than that of TCD at similar specificity, 55% vs. 39%, p = 0.038, whereas at a threshold of 200 cm/sec used for more advanced spasm, sensitivities and specificities of both methods were not different. The accuracy of TCCS and TCD is similar, but TCCS is more sensitive than TCD in the detection of MCA spasm. Sensitivity of both techniques in the detection of mild and more advanced spasm using 120 cm/sec and 200 cm/sec thresholds, respectively, is poor; however, a larger sample is required to increase precision of our sensitivity estimates.

Achieving aesthetic results in facial reconstructive microsurgery: planning and executing secondary refinements.

Science.gov (United States)

Haddock, Nicholas T; Saadeh, Pierre B; Siebert, John W

2012-12-01

Free tissue transfer to improve bulk and contour in facial deformities has been proven useful, yet refinements that turn an acceptable result into an excellent result are essential to reconstruction. The authors reviewed their experience and described these refinements. The charts of 371 free tissue transfer cases (1989 to 2010) performed by the senior author (J.W.S.) were reviewed. Free tissue transfer of a circumflex scapular variant flap or superficial inferior epigastric was performed to treat deformities arising from hemifacial atrophy (n = 126), hemifacial microsomia (n = 89), radiation therapy (n = 40), bilateral malformations including lupus and polymyositis (n = 50), other congenital anomalies (n = 25), facial palsy (n = 17), and burns and trauma (n = 24). Revision surgery planning began at initial flap operation where the flap was stretched maximally and interdigitated with recipient tissue. More tissue was required in the malar region. Revision refinement was indicated in all cases (after 6 months). Flap revision involved liposuction, debulking, reelevation, and release of tethering, followed by tissue rearrangement by means of advancement, rotation, transposition, and/or turnover flaps of subcutaneous tissues from the previous free flap. The jawline frequently required more debulking. Periorbital reconstruction was combined with lower lid support with or without canthal repositioning. Conventional face-lift techniques with the flap as superficial musculoaponeurotic system augmented the result. Autologous fat injection to the alar rim, medial canthus, upper eyelid, and lip was a useful adjunct. Severe lip deficiencies were addressed with local flaps. The keys to improving results were continual critical reassessment, open-mindedness to new approaches, and maintaining high expectations. Therapeutic, V.

Painful swallowing

Science.gov (United States)

... to a problem with the esophagus, such as: Achalasia Esophageal spasms Gastroesophageal reflux disease Inflammation of the ... any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should ...

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 ...

Traumatic Brain Injury: Hope Through Research

Science.gov (United States)

... fatigue or drowsiness; a lack of energy or motivation changes in sleep patterns (e.g., sleeping a ... nerve cells in the brain causing strange sensations, emotions, and behavior, or sometimes convulsions, muscle spasms, and ...

Calendula

Science.gov (United States)

... the skin (used topically) for nosebleeds, varicose veins, hemorrhoids, inflammation of the rectum (proctitis), and inflammation of ... circulation. Muscle spasms. Fever. Cancer. Nosebleeds. Varicose veins. Hemorrhoids. Promoting menstruation. Treating mouth and throat soreness. Wounds. ...

Subarachnoid hemorrhage

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... control blood pressure Medicine to prevent artery spasms Painkillers and anti-anxiety medicines to relieve headache and ... Other complications include: Complications of surgery Medicine side effects Seizures Stroke When to Contact a Medical Professional ...

Epilepsy

Science.gov (United States)

Epilepsy is a brain disorder that causes people to have recurring seizures. The seizures happen when clusters ... may have violent muscle spasms or lose consciousness. Epilepsy has many possible causes, including illness, brain injury, ...

Autonomic dysreflexia

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... pulse Muscle spasms, especially in the jaw Nasal congestion Throbbing headache Sometimes there are no symptoms, even ... of the small intestine, colon, appendix, and bronchial tubes in the lungs) Neuroleptic malignant syndrome (a condition ...

Prevention: Exercise

Medline Plus

Full Text Available ... Back Pain SI Joint Pain Other Scoliosis Back Pain and Emotional Distress Muscle Spasms Pinched Nerve Discitis Degenerative Conditions Bulge vs Herniation Cervical Stenosis, Myelopathy, and Radiculopathy Herniated Lumbar Disc Herniated ...

[Involvement of thrombophilia in coronary thrombosis].

Science.gov (United States)

Bal Dit Sollier, C; Drouet, L

2017-12-01

This review of thrombophilia and coronary thrombosis takes into account the "classical" thrombophilia commonly found in venous pathology and the conditions under which their research may be useful in certain forms of arterial thrombosis especially coronary thrombosis. In addition to the classical thrombophilia, exceptional thrombophilia are evoked, which are both factors of venous thrombosis but also arterial thrombosis. There are also thrombophilia that are more specific to the arterial system such as - homocystein which is potentially both a thrombosis factor but also an agent of arterial parietal lesion or - serotonin which is a factor of arterial spasm and especially coronary spasm. Finally, under the term thrombophilia, it is possible to include thrombophilic conditions, in particular cancers and inflammatory conditions. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Quantitative variation of melanins in alpaca (Lama pacos L.

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Marco Antonini

2011-07-01

Full Text Available The amount of melanin pigments was investigated in 95 Peruvian alpaca, representative of six different fleece colours, by means of spectrophotometric assays: SpEM (Spectrophoto­metric Eumelanin, SpPM (Spectrophotometric Pheomelanin, SpASM (Spectrophotometric Alkali Soluble Melanin, and SpTM (Spectro­photometric Total Melanin. It was found that these melanin pigments were suitable for identifying three homogeneous groups, each consisting of two closely related colours. A low, an intermediate, and a high amount of SpASM, SpTM, and SpPM characterize pinkish grey and light reddish brown, brown and reddish brown, dark reddish brown and black fleeces, respectively. SpEM and SpTM provide a further split within this latter group; higher concentrations of these pigments distinguish black fleece from dark reddish brown.

Acute hemifacial ischemia as a late complication of carotid stenting

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Maurizio Domanin, MD

2017-06-01

Full Text Available Concerns about carotid artery stenting (CAS center primarily on procedural complications like acute occlusion, stroke, and long-term intrastent restenosis. External carotid artery (ECA thrombosis is observed during CAS follow-up, but it often remains asymptomatic or, at worst, results in jaw claudication. We report here a case of late occlusion of the ECA after CAS with symptoms of acute homolateral facial ischemia as well as pain, cyanosis, tongue numbness, and skin coldness. The patient was submitted to local thrombolysis and balloon angioplasty with regression of symptoms after recanalization. With this report, we add a caveat about blockage of the ECA ostium during CAS.

Distracción ósea y cirugía ortognática en paciente previamente radiado

OpenAIRE

Martínez-Pérez, Fátima; Arribas-García, Ignacio; Álvarez-Flórez, Modesto; Gómez-Oliveira, Guillermo; Serrano Álvarez-Buylla, Adriana; Martínez-Gimeno, Carlos

2016-01-01

Introducción: Presentamos a un paciente que recibió tratamiento radioterápico en la infancia por un retinoblastoma. Como principal secuela presenta una severa hipoplasia ósea hemifacial, alteración de la dentición y atrofia muscular. Se realiza cirugía correctora facial mediante distracción ósea, cirugía ortognática y lipoescultura facial con un resultado estético y funcional excelente y estable hasta el momento actual. Caso clínico: Varón de 15 años con hipoplasia témporo-parieto-frontal,...

Spasm of the near reflex: A case report

Directory of Open Access Journals (Sweden)

Maedbh Rhatigan

2017-06-01

Conclusion and importance: SNR is predominantly a clinical diagnosis, and often leads to patients undergoing unnecessary investigations and sometimes treatment. Recognising the salient features that differentiate it could potentially avoid this.

Short term effect of vigabatrin in infantile spasms

OpenAIRE

Pozo Alonso, Albia J.; Pozo Lauzán, Desiderio; Cordero López, Girelda

2007-01-01

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 ...

Botulinum Toxin Injections: A Treatment for Muscle Spasms

Science.gov (United States)

... sites. To treat soreness, you can take acetaminophen (brand name: Tylenol) or ibuprofen (brand names: Advil, Motrin). You can also put an ... Childbirth Women Men Seniors Your Health Resources Healthcare Management End-of-Life Issues Insurance & Bills Self Care ...

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 ...

Genetics Home Reference: task-specific focal dystonia

Science.gov (United States)

... of particular tasks, such as writing, playing a musical instrument, or participating in a sport. Dystonias are a ... cramps and spasms that occur while playing a musical instrument. This condition can affect amateur or professional musicians, ...

Types of Seizures Affecting Individuals with TSC

Science.gov (United States)

... Policy Sitemap Learn Engage Donate About TSC Epilepsy/Seizure Disorders Seizures remain one of the most common neurological ... TSC Brain and Neurological Function Brain Abnormalities Epilepsy/Seizure Disorders Infantile Spasms Epilepsy in Adults with TSC Epilepsy ...

[Contribution of botulinum toxin to maxillo-facial surgery].

Science.gov (United States)

Batifol, D; de Boutray, M; Goudot, P; Lorenzo, S

2013-04-01

Botulinum toxin has a wide range of use in maxillo-facial surgery due to its action on muscles, on the glandular system, and against pain. It already has been given several market authorizations as indicated for: blepharospasm, spasmodic stiff neck, and glabellar lines. Furthermore, several studies are ongoing to prove its effectiveness and usefulness for many other pathologies: treatment of pain following cervical spine surgery; action on salivary glands after trauma, hypertrophy, or hyper-salivation; analgesic action (acknowledged but still being experimented) on neuralgia, articular pain, and keloids scars due to its anti-inflammatory properties. Botulinum toxin injections in the cervico-facial area are more and more used and should be to be correctly assessed. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Prevention: Exercise

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Full Text Available ... SI Joint Pain Other Scoliosis Back Pain and Emotional Distress Muscle Spasms Pinched Nerve Discitis Degenerative Conditions ... Physical Therapy Postural Training Traction Watchful Waiting and Education Injection Treatments for Spinal Pain Epidural Steroid Injections ...

Prevention: Exercise

Medline Plus

Full Text Available ... Back Pain and Emotional Distress Muscle Spasms Pinched Nerve Discitis Degenerative Conditions Bulge vs Herniation Cervical Stenosis, ... Spinal Disorders Repeated End-Range Spinal Testing Specialized Nerve Tests: EMG, NCV and SEEP Alternative Medicine Acupuncture ...

38 CFR 4.57 - Static foot deformities.

Science.gov (United States)

2010-07-01

... Achilles tendon, peroneal spasm due to adhesion about the peroneal sheaths, and other evidence of pain and... gaping of bones on the inner border of the foot, and rigid valgus position with loss of the power of...

Analgesia after total hip replacement: epidural versus psoas ...

African Journals Online (AJOL)

tive analgesia following total hip replacement surgery. The research design was a ... matoma, intra-abdominal injury and pain due to spasm of the lumbar paravertebral ..... 2. Brown DL. Spinal, epidural, and caudal anesthesia In: Miller RD, ed.

Alterations of monocarboxylate transporter densities during hypoxia in brain and breast tumour cells

DEFF Research Database (Denmark)

Cheng, Chang; Edin, Nina F Jeppesen; Lauritzen, Knut H

2012-01-01

Tumour cells are characterized by aerobic glycolysis, which provides biomass for tumour proliferation and leads to extracellular acidification through efflux of lactate via monocarboxylate transporters (MCTs). Deficient and spasm-prone tumour vasculature causes variable hypoxia, which favours...

Horse Chestnut

Science.gov (United States)

... gastrointestinal upset, muscle spasm, or headache. Keep in Mind Tell all your health care providers about any ... Privacy and Policies Accessibility en Español FOIA Site Map Contact Us U.S. Department of Health & Human Services , ...

VMA Test

Science.gov (United States)

... spasms and rapid eye movements referred to as "dancing eyes, dancing feet." The VMA test may also be ordered ... a pheochromocytoma or neuroblastoma? People with a family history of multiple endocrine neoplasia (MEN2), a genetic condition ...

A case study of chiropractic management of pregnancy-related heartburn with postulated fetal epigenome implications.

Science.gov (United States)

Peterson, Caroline

2012-01-01

This case study reports on chiropractic care for pregnancy-related heartburn. The purpose of this article is to relate the benefit of chiropractic treatment for one individual, to contrast chiropractic management with the biomedical standard of care for pregnancy-related heartburn, and to point to potential epigenetic implications of the standard of care. A 32-year-old woman who was 24 weeks pregnant presented with persistent heartburn that she was treating with ranitidine (Zantac®) and calcium carbonate (Tums®) daily at the initiation of chiropractic care. Findings of the initial examination were thoracic intersegmental dysfunction and pain upon palpation of the diaphragm, with hypertonicity noted. Therapy localization was positive for reflexes associated with the esophagus and lower esophageal sphincter, suggesting spasms. Emotional components also were identified in association with the symptoms by the use of a mind-body therapy called NeuroEmotional Technique. The patient was treated by adjusting the thoracic spine, manually releasing the diaphragm spasms, and releasing the esophageal spasm with an activator (a small hand-held instrument that creates a percussive force). The patient was symptom-free and did not use medication after the fifth treatment. She was followed throughout the remainder of her pregnancy and was asymptomatic and required no further treatment. A larger study should investigate the effectiveness of chiropractic care for the treatment of pregnancy-related heartburn. Copyright © 2012 Elsevier Inc. All rights reserved.

A pharmaceutical study on chlorzoxazone orodispersible tablets: formulation, in-vitro and in-vivo evaluation.

Science.gov (United States)

Moqbel, Helal Abdo; ElMeshad, Aliaa Nabil; El-Nabarawi, Mohamed Ahmed

2016-10-01

Muscle spasm needs prompt relief of symptoms. Chlorzoxazone is a centrally muscle relaxant. The aim of this study was to prepare chlorzoxazone orodispersible tablets (ODTs) allowing the drug to directly enter the systemic circulation and bypassing the first-pass metabolism for both enhancing its bioavailability and exerting a rapid relief of muscular spasm. ODTs were prepared by direct compression method using Pharmaburst®500, Starlac®, Pearlitol flash®, Prosolv® odt and F-melt® as co-processed excipients. Three ratios of the drug to the other excipients were used (0.5:1, 1:1 and 2:1). All ODTs were within the pharmacopeial limits for weight and content. ODTs containing Pharmaburst®500 showed the shortest wetting time (∼45.33 s), disintegration time (DT) (∼43.33 s) and dissolution (Q 15min 100.63%). By increasing the ratio of CLZ: Pharmaburst®500 from 0.5:1 to 1:1 and 2:1, the DT increased from 26.43 to 28.0 and 43.33 s, respectively. By using Prosolv® odt, ODTs failed to disintegrate in an acceptable time >180 s. DT of ODTs using different co-processed excipients can be arranged as follows: Pharmaburst® 500 tablets are a promising carrier for CLZ designed for management of muscle spasm due to the enhanced dissolution and rapid absorption of the drug through the oral mucosa.

Prevention: Exercise

Medline Plus

Full Text Available ... Chronic Low Back Pain SI Joint Pain Other Scoliosis Back Pain and Emotional Distress Muscle Spasms Pinched ... DC Directional Exercises Electrothermal Modalities Ergonomic Changes Hydrotherapy Manual Therapy Physical Therapy Postural Training Traction Watchful Waiting and ...

Diagnostic value of plain radiographs in patients with low back pain ...

African Journals Online (AJOL)

PID), osteoporosis, scoliosis, muscle spasm and sacroilitis (k=0.13-0.21). Conclusion: Most of the patients presenting to KNH with low back pain have a chronic type and therefore have increased probability of having positive radiological findings.

Browse Title Index

African Journals Online (AJOL)

PET) in South African public hospitals: nuclear medicine, Abstract PDF. MM Sathekge, J Warwick, MDT Vangu, A Ellmann, M Mann. Vol 106, No 12 (2016), A case of convergence spasms – do not be caught off-guard ...

Heat Waves

Science.gov (United States)

Heat Waves Dangers we face during periods of very high temperatures include: Heat cramps: These are muscular pains and spasms due ... that the body is having trouble with the heat. If a heat wave is predicted or happening… - ...

High Peak Power Microwaves: A Health Hazard

Science.gov (United States)

1993-12-01

administration of specific drugs, including beta blockers , can significantly enhance these effects, as evidenced by a lowered damage threshold exposure...impotence, hot flashes, weight gain, palpitations, sle.p disruption, muscle cramps, bladder spasms, hallucinations, salivation increased, tremors , scizurcs

Case Series of Ruptured Jamaican Berry Aneurysms Four Decades ...

African Journals Online (AJOL)

Having bled, the aneurysms exposed themselves by causing sudden severe headaches, and various neurological problems, depending on their locations. We found more females than males. The posterior communicating artery aneurysm was predominant. There were no cerebro-vascular spasms postoperatively.

Heat-Related Illnesses

Science.gov (United States)

... Share this! EmergencyCareForYou » Emergency 101 » Heat-Related Illnesses Heat-Related Illnesses Dr. Glenn Mitchell , Emergency physician at ... about heat cramps and heat stroke and exhaustion. Heat Cramps Symptoms include muscle spasms, usually in the ...

Hjertestop induceret af vasospastisk angina pectoris efter vaginalt administreret misoprostol

DEFF Research Database (Denmark)

Matthesen, Terese; Olsen, Rasmus Huan; Bosselmann, Helle Skovmand

2017-01-01

A 41-year-old woman developed cardiac arrest after administration of misoprostol in order to induce an abortion. She was successfully resuscitated. Coronary angiography revealed coronary artery spasm which responded to nitroglycerine. Misoprostol is first-line treatment for medically induced abor...

Malignant Hyperthermia in Dental and Facial Plastic surgeries

Directory of Open Access Journals (Sweden)

Ramin Maheri

2015-04-01

Full Text Available Preoperative evaluation of the patients scheduled for ambulatory surgery is of great importance in regards of both surgery and anesthesia considerations. Malignant Hyperthermia (MH is a pharmacogenetic clinical syndrome which mostly arises from volatile anesthesia with halothane and the depolarizing muscle relaxant succinylcholine. Clinical manifestations of MH are acidosis and rhabdomyolysis which occur following uncontrolled increases in skeletal muscle metabolism and rapidly increasing body temperature (by as much as 1°C/5 min (1. Primary cases of MH were reported to be of a 70% mortality rate; however, thanks to the emergence of diagnostic tools such as end-expired carbon dioxide and the administration of dantrolene, this rate has decreased to less than 5%.      MH might occur even in those with no previous or familial history. Even a safe history of the previous surgery under the administration of MH triggering agents cannot guarantee a next safe surgery. A noteworthy point in the preoperative evaluation is the probable association of MH with certain musculoskeletal disorders including Duchenne, Becker, and myotonic muscular dystrophies, strabismus, osteogenesis imperfecta, ptosis, myelomeningocele, kyphoscoliosis, King-Denborough syndrome, periodic paralysis, hernias, marfanoid syndrome, and central core disease (1. These diseases are frequently encountered by dental and facial plastic surgeons and having the knowledge of the probable association between MH and these conditions could aware the dental and facial plastic surgeons and the anesthesiologists to be more vigilant.      Although MH workup leads to the definite diagnosis, no convincing intraoperative diagnostic tool has been introduced so far. Masseter spasm is a condition which might follow administration of depolarizing neuromuscular blocking agents due to the slow tonic fibers of masseter and lateral pterygoid muscles (2-4. The severer forms of masseter spasm would

Angina de Prinzmetal Angina de Prinzmetal Prinzmetal's angina

Directory of Open Access Journals (Sweden)

Eduardo Contreras Zuniga

2009-08-01

Full Text Available Essa síndrome é causada por um espasmo focal de uma artéria coronária epicárdica, levando a isquemia miocárdica grave. Embora freqüentemente acredite-se que o espasmo ocorra em artérias sem estenose, muitos pacientes com angina de Prinzmetal apresentam espasmo adjacente a placas ateromatosas. A causa exata do espasmo não está bem definida, mas pode estar relacionada à hipercontratilidade do músculo liso vascular devido a mitógenos vasoconstrictores, leucotrienos ou serotonina. Em alguns pacientes, é uma manifestação de distúrbio vasoespástico e está associado à migrânea, fenômeno de Raynaud ou asma induzida por aspirina. Apresentamos um caso associado com depressão transitória do segmento ST.Este síndrome es causado por un espasmo focal de una arteria coronaria epicárdica, llevando a isquemia miocárdica grave. Aunque frecuentemente se crea que el espasmo ocurra en arterias sin estenosis, muchos pacientes con angina de Prinzmetal presentan espasmo adyacente a placas ateromatosas. La causa exacta del espasmo no está bien definida, pero puede estar relacionada a la hipercontractilidad del músculo liso vascular debido a mitógenos vasoconstrictores, leucotrienos o serotonina. En algunos pacientes, es una manifestación de disturbio vasoespástico y está asociado a la migraña, fenómeno de Raynaud o asma inducida por aspirina. Presentamos un caso asociado con depresión transitoria del segmento ST.This syndrome is due to focal spasm of an epicardial coronary artery, leading to severe myocardial ischemia. Although it is frequently thought that the spasm occurs in arteries without stenosis, many Prinzmetal patients have spasm adjacent to atheromatous plaques. The exact cause of the spasm has not been well defined, but it may be related to the hypercontractility of the vascular smooth muscle due to vasoconstrictor mitogens, leukotrienes, or serotonin. In some patients, it is a manifestation of a vasospastic disorder and it

Long-term intrathecal baclofen: outcomes after more than 10 years of treatment.

Science.gov (United States)

Mathur, Sunjay N; Chu, Samuel K; McCormick, Zack; Chang Chien, George C; Marciniak, Christina M

2014-06-01

To report outcomes of intrathecal baclofen (ITB) therapy for spasticity management in a cohort of patients who had received this treatment for at least 10 years. A cross-sectional survey and retrospective chart review. An academic rehabilitation outpatient clinic. Adult patients with spasticity treated with ITB for at least 10 years. Surveys included the Brief Pain Inventory, Penn Spasm Frequency Scale, Epworth Sleepiness Scale, Fatigue Severity Scale, Diener Satisfaction with Life, Life Satisfaction Questionnaire, and Intrathecal Baclofen Survey. Twenty-four subjects completed the surveys. The subjects had been treated with ITB from 10.0-28.4 years, with a mean (standard deviation) of 14.7 ± 4.2 years. The mean (standard deviation) dose of ITB was 627.9 ± 306.7 μg/d, with only 6 subjects averaging daily dose changes of more than 10% compared with 3 years earlier. The mean (standard deviation) scores on outcomes surveys were the following: 2.6 ± 2.3 for pain severity on the Brief Pain Inventory, 1.4 ± 0.7 for spasm severity on the Penn Spasm Frequency Scale, 7.9 ± 5.4 on the Epworth Sleepiness Scale, 4.1 ± 1.6 on the Fatigue Severity Scale, 19.4 ± 8.1 on the Diener Satisfaction with Life, 3.9 ± 0.9 on the Life Satisfaction Questionnaire, and 8.8 ± 1.9 for overall satisfaction with ITB on the Intrathecal Baclofen Survey. On the Brief Pain Inventory, the mean scores for pain severity and interference of pain with life showed moderate inverse correlations with ITB dose (r = -0.386, P = .115; and r = -0.447, P = .062, respectively). On the Life Satisfaction Questionnaire, the mean scores for life satisfaction showed statistically significant positive correlation with ITB dose (r = 0.549, P = .021). The subjects reported low levels of pain, moderate levels of life satisfaction, normal levels of sleepiness, low-to-moderate levels of fatigue, infrequent spasms at mild-to-moderate severity, and high levels of satisfaction. The efficacy and favorable adverse

Swallowing difficulty

Science.gov (United States)

... Schatzki ring ) Abnormal spasms of the esophagus muscles Cancer of the esophagus Failure of the muscle bundle at the bottom ... or surgery: These treatments may be used if cancer is causing the ... of the esophagus may also respond to surgery. You may need ...

Medical marijuana

Science.gov (United States)

... different amounts of cannabinoids. This sometimes makes the effects of medical marijuana hard to predict or control. The effects also ... wasting syndrome) Severe muscle spasms Multiple sclerosis Side Effects ... physical symptoms from using marijuana include: A fast or irregular heartbeat Dizziness Slow ...

Tetany: A diagnostic dilemma

Directory of Open Access Journals (Sweden)

Aparna Williams

2011-01-01

Full Text Available Tetany is a disorder of increased neuronal excitability usually associated with hypocalcemia. We report a patient with typical tetanic cramps and carpopedal spasm in the postoperative period, despite normal serum concentrations of calcium, which responded to intravenous infusion of calcium.

Heat-Related Illnesses

Medline Plus

Full Text Available ... Share this! EmergencyCareForYou » Emergency 101 » Heat-Related Illnesses Heat-Related Illnesses Dr. Glenn Mitchell , Emergency physician at ... about heat cramps and heat stroke and exhaustion. Heat Cramps Symptoms include muscle spasms, usually in the ...

Application of rare variant transmission disequilibrium tests to epileptic encephalopathy trio sequence data

NARCIS (Netherlands)

Allen, Andrew S.; Berkovic, Samuel F.; Bridgers, Joshua; Cossette, Patrick; Dlugos, Dennis; Epstein, Michael P.; Glauser, Tracy; Goldstein, David B.; Heinzen, Erin L.; Jiang, Yu; Johnson, Michael R.; Kuzniecky, Ruben; Lowenstein, Daniel H.; Marson, Anthony G.; Mefford, Heather C.; O'Brien, Terence J.; Ottman, Ruth; Petrou, Steven; Petrovski, Slavé; Poduri, Annapurna; Ren, Zhong; Scheffer, Ingrid E.; Sherr, Elliott; Wang, Quanli; Balling, Rudi; Barisic, Nina; Baulac, Stéphanie; Caglayan, Hande; Craiu, Dana; De Jonghe, Peter; Depienne, Christel; Guerrini, Renzo; Helbig, Ingo; Hjalgrim, Helle; Hoffman-Zacharska, Dorota; Jähn, Johanna A.; Klein, Karl Martin; Koeleman, Bobby; Komarek, Vladimir; Krause, Roland; Leguern, Eric; Lehesjoki, Anna-Elina; Lemke, Johannes R.; Lerche, Holger; Linnankivi, Tarja; Marini, Carla; May, Patrick; Møller, Rikke S.; Muhle, Hiltrud; Pal, Deb; Palotie, Aarno; Rosenow, Felix; Selmer, Kaja; Serratosa, Jose M.; Sisodiya, Sanjay M.; Stephani, Ulrich; Sterbova, Katalin; Striano, Pasquale; Suls, Arvid; Talvik, Tiina; von Spiczak, Sarah; Weber, Yvonne G.; Weckhuysen, Sarah; Zara, Federico; Abou-Khalil, Bassel; Alldredge, Brian K.; Amrom, Dina; Andermann, Eva; Andermann, Frederick; Bautista, Jocelyn F.; Bluvstein, Judith; Cascino, Gregory D.; Consalvo, Damian; Crumrine, Patricia; Devinsky, Orrin; Fiol, Miguel E.; Fountain, Nathan B.; French, Jacqueline; Friedman, Daniel; Haas, Kevin; Haut, Sheryl R.; Hayward, Jean; Joshi, Sucheta; Kanner, Andres; Kirsch, Heidi E.; Kossoff, Eric H.; Kuperman, Rachel; McGuire, Shannon M.; Motika, Paul V.; Novotny, Edward J.; Paolicchi, Juliann M.; Parent, Jack; Park, Kristen; Shellhaas, Renée A; Sirven, Joseph; Smith, Michael C.; Sullivan, Joseph; Thio, Liu Lin; Venkat, Anu; Vining, Eileen P. G.; Von Allmen, Gretchen K.; Weisenberg, Judith L.; Widdess-Walsh, Peter; Winawer, Melodie R.

2017-01-01

The classic epileptic encephalopathies, including infantile spasms (IS) and Lennox-Gastaut syndrome (LGS), are severe seizure disorders that usually arise sporadically. De novo variants in genes mainly encoding ion channel and synaptic proteins have been found to account for over 15% of patients

Cerebral gigantism with West syndrome.

Science.gov (United States)

Ray, Munni; Malhi, P; Bhalla, A K; Singhi, P D

2003-07-01

A case of cerebral gigantism (Sotos syndrome) with West syndrome in a one-year-old male child is reported. The case had a large stature, typical facies and neurodevelopmental delay along with infantile spasms, which were refractory to treatment with valproate and clonazepam.

Rodding Surgery

Science.gov (United States)

... Physical activity prior to surgery,  Length of the operation; anesthesia issues,  Reason for the choice of rod,  Time in the hospital,  Length of recovery time at home,  Pain management including control of muscle spasms,  The rehabilitation plan. ...

Intravesical knotting of feeding tube used as urinary catheter in an infant

Directory of Open Access Journals (Sweden)

Mamatha Basavaraju

2016-09-01

Full Text Available Infant feeding tube is commonly used to temporarily drain the bladder in pediatric population. A case is described where the tube got knotted inside the bladder probably due to over insertion or bladder spasm caused by irritation of catheter.

Changes in muscle and tendons due to neural motor disorders : implications for therapeutical intervention

NARCIS (Netherlands)

Hof, A.L.

2001-01-01

Patients with an upper motor neurone syndrome (CP) suffer from many disabling primary symptoms: spasms, weakness, and loss of dexterity. These primary ‘neurogenic’ symptoms often lead to secondary disabilities, muscle contractures, and tertiary effects, bone deformations. A common symptom of CP is

How Many Dystonias? Clinical Evidence.

Science.gov (United States)

Albanese, Alberto

2017-01-01

Literary reports on dystonia date back to post-Medieval times. Medical reports are instead more recent. We review here the early descriptions and the historical establishment of a consensus on the clinical phenomenology and the diagnostic features of dystonia syndromes. Lumping and splitting exercises have characterized this area of knowledge, and it remains largely unclear how many dystonia types we are to count. This review describes the history leading to recognize that focal dystonia syndromes are a coherent clinical set encompassing cranial dystonia (including blepharospasm), oromandibular dystonia, spasmodic torticollis, truncal dystonia, writer's cramp, and other occupational dystonias. Papers describing features of dystonia and diagnostic criteria are critically analyzed and put into historical perspective. Issues and inconsistencies in this lumping effort are discussed, and the currently unmet needs are critically reviewed.

Involuntary movements in the elderly. Parkinson's disease and other causes.

Science.gov (United States)

Miller, J Q

1986-03-01

Dyskinesia is usually lifelong and progressive; therefore, physicians generally see the disorder in elderly patients. Medical treatment must be carefully selected on the basis of the cause of the dyskinesia. Parkinsonian dyskinesia is well controlled by drug therapy. However, patients can become less responsive to a drug after years of use and may experience unwelcome side effects. Cerebellar tremor is extremely disabling because it worsens with activity, but no satisfactory therapy is available. Senile, essential, and familial tremors are also intensified by action, but they can often be suppressed with a mild tranquilizer or a beta blocker. Drug treatment of blepharospasm and spastic dysphonia has been disappointing: Facial or laryngeal surgery is sometimes required. Tardive dyskinesia is caused by neuroleptic drugs, so the only therapy for the disorder is withdrawal of the offending drug.

Neuroinflammation in epileptogenesis: Insights and translational perspectives from new models of epilepsy.

Science.gov (United States)

Barker-Haliski, Melissa L; Löscher, Wolfgang; White, H Steve; Galanopoulou, Aristea S

2017-07-01

Animal models have provided a wealth of information on mechanisms of epileptogenesis and comorbidogenesis, and have significantly advanced our ability to investigate the potential of new therapies. Processes implicating brain inflammation have been increasingly observed in epilepsy research. Herein we discuss the progress on animal models of epilepsy and comorbidities that inform us on the potential role of inflammation in epileptogenesis and comorbidity pathogenesis in rodent models of West syndrome and the Theiler's murine encephalomyelitis virus (TMEV) mouse model of viral encephalitis-induced epilepsy. Rat models of infantile spasms were generated in rat pups after right intracerebral injections of proinflammatory compounds (lipopolysaccharides with or without doxorubicin, or cytokines) and were longitudinally monitored for epileptic spasms and neurodevelopmental and cognitive deficits. Anti-inflammatory treatments were tested after the onset of spasms. The TMEV mouse model was induced with intracerebral administration of TMEV and prospective monitoring for handling-induced seizures or seizure susceptibility, as well as long-term evaluations of behavioral comorbidities of epilepsy. Inflammatory processes are evident in both models and are implicated in the pathogenesis of the observed seizures and comorbidities. A common feature of these models, based on the data so far available, is their pharmacoresistant profile. The presented data support the role of inflammatory pathways in epileptogenesis and comorbidities in two distinct epilepsy models. Pharmacoresistance is a common feature of both inflammation-based models. Utilization of these models may facilitate the identification of age-specific, syndrome- or etiology-specific therapies for the epilepsies and attendant comorbidities, including the drug-resistant forms. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

Diagnostic yield of 24-hour esophageal manometry in non-cardiac chest pain.

Science.gov (United States)

Barret, M; Herregods, T V K; Oors, J M; Smout, A J P M; Bredenoord, A J

2016-08-01

In the past, ambulatory 24-h manometry has been shown useful for the evaluation of patients with non-cardiac chest pain (NCCP). With the diagnostic improvements brought by pH-impedance monitoring and high-resolution manometry (HRM), the contribution of ambulatory 24-h manometry to the diagnosis of esophageal hypertensive disorders has become uncertain. Our aim was to assess the additional diagnostic yield of ambulatory manometry to HRM and ambulatory pH-impedance monitoring in this patient population. All patients underwent 24-h ambulatory pressure-pH-impedance monitoring and HRM. Patients had retrosternal pain as a predominant symptom and no explanation after cardiologic and digestive endoscopic evaluations. Diagnostic measurements were analyzed by two independent physicians. Fifty-nine patients met the inclusion criteria; 37.3% of the patients had their symptoms explained by abnormalities on pH-impedance monitoring and 6.8% by ambulatory manometry. Functional chest pain was diagnosed in 52.5% of the patients. High-resolution manometry, using the Chicago Classification v3.0 criteria alone, did not identify any of the four patients with esophageal spasm on ambulatory manometry. However, taking into account other abnormalities, such as simultaneous (rapid) or repetitive contractions, HRM had a sensitivity of 75% and a specificity of 98.2% for the diagnosis of esophageal spasm. In the work-up of NCCP, ambulatory 24-h manometry has a low additional diagnostic yield. However, it remains the best technique to identify esophageal spasm as the cause of symptoms. This is particularly useful when an unequivocal diagnosis is needed before treatment. © 2016 John Wiley & Sons Ltd.

Developmental neurotoxicity of pyrethroid insecticides in zebrafish embryos.

Science.gov (United States)

DeMicco, Amy; Cooper, Keith R; Richardson, Jason R; White, Lori A

2010-01-01

Pyrethroid insecticides are one of the most commonly used residential and agricultural insecticides. Based on the increased use of pyrethroids and recent studies showing that pregnant women and children are exposed to pyrethroids, there are concerns over the potential for developmental neurotoxicity. However, there have been relatively few studies on the developmental neurotoxicity of pyrethroids. In this study, we sought to investigate the developmental toxicity of six common pyrethroids, three type I compounds (permethrin, resmethrin, and bifenthrin) and three type II compounds (deltamethrin, cypermethrin, and lambda-cyhalothrin), and to determine whether zebrafish embryos may be an appropriate model for studying the developmental neurotoxicity of pyrethroids. Exposure of zebrafish embryos to pyrethroids caused a dose-dependent increase in mortality and pericardial edema, with type II compounds being the most potent. At doses approaching the LC(50), permethrin and deltamethrin caused craniofacial abnormalities. These findings are consistent with mammalian studies demonstrating that pyrethroids are mildly teratogenic at very high doses. However, at lower doses, body axis curvature and spasms were observed, which were reminiscent of the classic syndromes observed with pyrethroid toxicity. Treatment with diazepam ameliorated the spasms, while treatment with the sodium channel antagonist MS-222 ameliorated both spasms and body curvature, suggesting that pyrethroid-induced neurotoxicity is similar in zebrafish and mammals. Taken in concert, these data suggest that zebrafish may be an appropriate alternative model to study the mechanism(s) responsible for the developmental neurotoxicity of pyrethroid insecticides and aid in identification of compounds that should be further tested in mammalian systems.

Tidlig epileptisk encefalopati forårsaget af CDKL-5-mutation

DEFF Research Database (Denmark)

Hjalmgrim, Helle; Hansen, Lars Kjærsgaard; Ousager, Lilian Bomme

2014-01-01

Two girls suffering from early-onset epileptic encephalopathy are described. Both girls had changes involving the gene CDKL5. They both had seizures in the first weeks of life and normal EEG interictally. Both developed infantile spasms and severe developmental defect. The epilepsy was difficult...

Experience of Soviet Medicine in a Great Patriotic War, 1941-1945. Volume 16, Section 3.

Science.gov (United States)

1980-10-22

with spasm or compression of vessels by scars. For explaining the presence of the silent infection valuable information gave accurate anamnesis : the...detected accidentally. V. entered apropos of feaoral arteriovenous aneurisms. In anamnesis there was a bullet injury of shin. In the process of

Some Aspects of Epilepsy and Convulsions in Childhood | Brett ...

African Journals Online (AJOL)

The diagnostic and therapeutic problems of epilepsy and convulsions in childhood are reviewed. Their non-specific aetiology is stressed, particularly with neonatal convulsions and infantile spasms. The role of febrile convulsions in causing later temporal lobe epilepsy and gross neurological deficit, underlines the ...

SpaSM: A MATLAB Toolbox for Sparse Statistical Modeling

DEFF Research Database (Denmark)

Sjöstrand, Karl; Clemmensen, Line Harder; Larsen, Rasmus

2018-01-01

Applications in biotechnology such as gene expression analysis and image processing have led to a tremendous development of statistical methods with emphasis on reliable solutions to severely underdetermined systems. Furthermore, interpretations of such solutions are of importance, meaning...

Treatment of Gastrointestinal Sphincters Spasms with Botulinum Toxin A

Directory of Open Access Journals (Sweden)

Giuseppe Brisinda

2015-05-01

Full Text Available Botulinum toxin A inhibits neuromuscular transmission. It has become a drug with many indications. The range of clinical applications has grown to encompass several neurological and non-neurological conditions. One of the most recent achievements in the field is the observation that botulinum toxin A provides benefit in diseases of the gastrointestinal tract. Although toxin blocks cholinergic nerve endings in the autonomic nervous system, it has also been shown that it does not block non-adrenergic non-cholinergic responses mediated by nitric oxide. This has promoted further interest in using botulinum toxin A as a treatment for overactive smooth muscles and sphincters. The introduction of this therapy has made the treatment of several clinical conditions easier, in the outpatient setting, at a lower cost and without permanent complications. This review presents current data on the use of botulinum toxin A in the treatment of pathological conditions of the gastrointestinal tract.

Therapeutic consequences of oesophageal function studies in patients with benign oesophageal disease

DEFF Research Database (Denmark)

Jakobsen, Erik; Kruse-Andersen, S; Wallin, Lene

1988-01-01

making the final decision for therapy. Eleven percent of the patients referred with a diagnosis of hiatal hernia or reflux had achalasia or oesophageal spasm. Nine percent of the patients referred for motility disorders had reflux-related disease. The referral diagnosis was changed to a diagnosis...

(1S, 3S)-3-amino-4-difluoromethylenyl-1-cyclopentanoic acid (CPP-115), a potent gamma-aminobutyric acid aminotransferase inactivator for the treatment of cocaine addiction

DEFF Research Database (Denmark)

Pan, Yue; Gerasimov, Madina R; Kvist, Trine

2012-01-01

Vigabatrin, a GABA aminotransferase (GABA-AT) inactivator, is used to treat infantile spasms and refractory complex partial seizures and is in clinical trials to treat addiction. We evaluated a novel GABA-AT inactivator (CPP-115) and observed that it does not exhibit other GABAergic or off-target...

Tamsulosin therapy improved the outcome of ureterorenoscopy for ...

African Journals Online (AJOL)

Introduction: Tamsulosin is an α-1A-specific blocker inducing selective relaxation of ureteral smooth muscle and inhibition of ureteral spasms leading to ureteral dilatation that can facilitates retrograde ureterorenoscopy (URS). Objective: To assess the efficacy of tamsulosin in improving the outcome of URS management of ...

Complications and Causes of Death in Spinal Cord Injury Patients in ...

African Journals Online (AJOL)

Conclusion: Most common complication and cause of death following SCI are muscle spasm and respiratory failure respectively. The risk factors associated with mortality are age, GCS<9, cervical spinal injury, and complete neurologic injury and those for complications were cervical spinal injury and Frankel Type A injury.

Anhidrosis (Hypohidrosis)

Science.gov (United States)

... release heat less efficiently. Heat-related problems include: Heat cramps. Symptoms include muscle pain or spasms. Rest in a cool place and drink water or a sports drink. Get medical help if cramps last longer than an hour. Heat exhaustion. Signs and symptoms include weakness, nausea and ...

Homocystinuria due to cystathionine beta synthase deficiency

Directory of Open Access Journals (Sweden)

Rao T

2008-01-01

Full Text Available A two year-old male child presented with cutis marmorata congenita universalis, brittle hair, mild mental retardation, and finger spasms. Biochemical findings include increased levels of homocysteine in the blood-106.62 µmol/L (normal levels: 5.90-16µmol/L. Biochemical tests such as the silver nitroprusside and nitroprusside tests were positive suggesting homocystinuria. The patient was treated with oral pyridoxine therapy for three months. The child responded well to this therapy and the muscle spasms as well as skin manifestations such as cutis marmorata subsided. The treatment is being continued; the case is reported here because of its rarity. Homocysteinuria arising due to cystathionine beta-synthase (CBS deficiency is an autosomal recessive disorder of methionine metabolism that produces increased levels of urinary homocysteine and methionine It manifests itself in vascular, central nervous system, cutaneous, and connective tissue disturbances and phenotypically resembles Marfan′s syndrome. Skin manifestations include malar flush, thin hair, and cutis reticulata / marmorata.

Treatment of Atlantoaxial Rotatory Fixation With Botulinum Toxin Muscle Block and Manipulation

Directory of Open Access Journals (Sweden)

Chia-Hung Lin

2010-04-01

Full Text Available Slippage after reduction of atlantoaxial rotatory fixation (AARF is usually treated with repeated cervical traction and brace immobilization. To date, no data have been published on the management of muscle spasm during treatment. Here, we describe the case of a 7-year-old girl with AARF for 1 month who visited our hospital for treatment. During physical examination, spasm of the sternocleidomastoid muscle was noted. The patient was treated with manipulative reduction, and slippage after reduction was managed with botulinum spasticity block of the sternocleidomastoid and splenius capitis muscles, and repeated manipulation. Cervical orthosis immobilization with a rehabilitation program of isometric contract–relax exercise for the neck was conducted for 3 months. The subject had full recovery from AARF at 1-year follow-up. This report demonstrates that, in selected cases of slippage after reduction from AARF, conservative management with manipulation under anesthesia is a good method, and the muscle components may play a crucial role in AARF.

Constraint-induced movement therapy promotes brain functional reorganization in stroke patients with hemiplegia

Science.gov (United States)

Wang, Wenqing; Wang, Aihui; Yu, Limin; Han, Xuesong; Jiang, Guiyun; Weng, Changshui; Zhang, Hongwei; Zhou, Zhiqiang

2012-01-01

Stroke patients with hemiplegia exhibit flexor spasms in the upper limb and extensor spasms in the lower limb, and their movement patterns vary greatly. Constraint-induced movement therapy is an upper limb rehabilitation technique used in stroke patients with hemiplegia; however, studies of lower extremity rehabilitation are scarce. In this study, stroke patients with lower limb hemiplegia underwent conventional Bobath therapy for 4 weeks as baseline treatment, followed by constraint-induced movement therapy for an additional 4 weeks. The 10-m maximum walking speed and Berg balance scale scores significantly improved following treatment, and lower extremity motor function also improved. The results of functional MRI showed that constraint-induced movement therapy alleviates the reduction in cerebral functional activation in patients, which indicates activation of functional brain regions and a significant increase in cerebral blood perfusion. These results demonstrate that constraint-induced movement therapy promotes brain functional reorganization in stroke patients with lower limb hemiplegia. PMID:25337108

Prevalence, prenatal diagnosis and clinical features of oculo-auriculo-vertebral spectrum: a registry-based study in Europe.

LENUS (Irish Health Repository)

Barisic, Ingeborg

2014-01-08

Oculo-auriculo-vertebral spectrum is a complex developmental disorder characterised mainly by anomalies of the ear, hemifacial microsomia, epibulbar dermoids and vertebral anomalies. The aetiology is largely unknown, and the epidemiological data are limited and inconsistent. We present the largest population-based epidemiological study to date, using data provided by the large network of congenital anomalies registries in Europe. The study population included infants diagnosed with oculo-auriculo-vertebral spectrum during the 1990-2009 period from 34 registries active in 16 European countries. Of the 355 infants diagnosed with oculo-auriculo-vertebral spectrum, there were 95.8% (340\\/355) live born, 0.8% (3\\/355) fetal deaths, 3.4% (12\\/355) terminations of pregnancy for fetal anomaly and 1.5% (5\\/340) neonatal deaths. In 18.9%, there was prenatal detection of anomaly\\/anomalies associated with oculo-auriculo-vertebral spectrum, 69.7% were diagnosed at birth, 3.9% in the first week of life and 6.1% within 1 year of life. Microtia (88.8%), hemifacial microsomia (49.0%) and ear tags (44.4%) were the most frequent anomalies, followed by atresia\\/stenosis of external auditory canal (25.1%), diverse vertebral (24.3%) and eye (24.3%) anomalies. There was a high rate (69.5%) of associated anomalies of other organs\\/systems. The most common were congenital heart defects present in 27.8% of patients. The prevalence of oculo-auriculo-vertebral spectrum, defined as microtia\\/ear anomalies and at least one major characteristic anomaly, was 3.8 per 100 000 births. Twinning, assisted reproductive techniques and maternal pre-pregnancy diabetes were confirmed as risk factors. The high rate of different associated anomalies points to the need of performing an early ultrasound screening in all infants born with this disorder.European Journal of Human Genetics advance online publication, 8 January 2014; doi:10.1038\\/ejhg.2013.287.

A case of trigeminal hypersensitivity after administration of intrathecal sufentanil and bupivacaine for labor analgesia

Directory of Open Access Journals (Sweden)

Adriano Bechara de Souza Hobaika

2014-01-01

Full Text Available Rostral spread of intrathecal drugs and sensitization of supraspinal sites may provoke several adverse effects. This case describes a patient with right hemifacial paresthesia, trismus and dysphasia on the trigeminal nerve distribution after intrathecal sufentanil administration. Primigravida, 34 years, 39 weeks of pregnancy, with hypothyroidism and pregnancy induced hypertension. Allergic to latex. In the use of puran T4, 50 μg /day. When the patient presented cervical dilatation of 4 cm, she requested analgesia. She was placed in the sitting position and a spinal puncture was performed with a 27G needle pencil point in L4/L5 (1.5 mg of bupivacaine plus 7.5 μg of sufentanil. Next, was performed an epidural puncture in the same space. It was injected bupivacaine 0.065%, 10 ml, to facilitate the passage of the catheter. After 5 min lying down in the lateral upright position, she complained of perioral and right hemifacial paresthesia, mainly maxillary and periorbital, as well as trismus and difficulty to speak. The symptoms lasted for 30 min and resolved spontaneously. After 1 h, patient requested supplementary analgesia (12 ml of bupivacaine 0.125% and a healthy baby girl was born. Temporary mental alterations have been described with the use of fentanyl and sufentanil in combined epidural-spinal analgesia, such as aphasia, difficulty of swallowing, mental confusion and even unconsciousness. In this patient, facial areas with paresthesia indicated by patient appear in clear association with the ophthalmic and maxillary branches of the trigeminal nerve and the occurrence of trismus and dysphagia are in association with the mandibular motor branch. The exact mechanism of rostral spread is not known, but it is speculated that after spinal drug administration, a subsequent epidural dose may reduce the intratecal space and propel the drug into the supraspinal sites.

Anti-motility and reductions in the concentrations of gut electrolytes ...

African Journals Online (AJOL)

The seeds of avocado (Persea americana Mill) are used in traditional medicine to treat, allay or prevent some spasm-related disorders, for instance, diarrhoea. The chloroform and methanol fractions of the chloroform-methanol extract of the seeds of P. americana were investigated for their qualitative and quantitative ...

Muscle activation patterns in posttraumatic neck pain

NARCIS (Netherlands)

Nederhand, Marcus Johannes

2003-01-01

As an important consequence of our research, we question the relevance of the criteria of the WAD injury severity classification system. We showed that the musculoskeletal signs in WAD grade II are not characterized by muscle spasm, (i.e. increase of muscle activity), but rather by a decrease in

Spasmodic dysphonia may respond to bilateral thalamic deep brain ...

African Journals Online (AJOL)

Background Spasmodic dysphonia is a primary focal dystonia manifested by loss of control of the vocal muscles during speech secondary to laryngeal muscle spasms. The pathophysiology is not well understood. Deep brain stimulation surgery (DBS) for other focal dystonias has been well reported. Methods We report the ...

Accuracy of a Computer Assisted Program for ’Classic’ Presentations of Dental Pain

Science.gov (United States)

1989-04-11

diagnosis of trauma and non-trauma related dental pain. Abscess /infection/cellulitis Acute apical abscess Acute apical periodontitis Acute...spasms Necrotizing ulcerative gingivitis Neurologic injury Osseous sequestrum Occlusal trauma Periodontal abscess Periocoronitis/erupting tooth...Acute apical abscess Acute apical periodontitis Acute gingivitis Carious lesion (decay) Dentin hypersensitivity Defective restoration

21 CFR 341.3 - Definitions.

Science.gov (United States)

2010-04-01

... overcome spasms that cause narrowing of the bronchial air tubes, such as in the symptomatic treatment of... congestion caused by acute or chronic rhinitis. (g) Topical nasal decongestant drug. A drug that when applied... nasal congestion caused by acute or chronic rhinitis. (h) Calibrated dropper. A dropper calibrated such...

Vestibular schwannoma with contralateral facial pain – case report

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Ghodsi Mohammad

2003-03-01

Full Text Available Abstract Background Vestibular schwannoma (acoustic neuroma most commonly presents with ipsilateral disturbances of acoustic, vestibular, trigeminal and facial nerves. Presentation of vestibular schwannoma with contralateral facial pain is quite uncommon. Case presentation Among 156 cases of operated vestibular schwannoma, we found one case with unusual presentation of contralateral hemifacial pain. Conclusion The presentation of contralateral facial pain in the vestibular schwannoma is rare. It seems that displacement and distortion of the brainstem and compression of the contralateral trigeminal nerve in Meckel's cave by the large mass lesion may lead to this atypical presentation. The best practice in these patients is removal of the tumour, although persistent contralateral pain after operation has been reported.

CDKL5 gene-related epileptic encephalopathy: electroclinical findings in the first year of life.

Science.gov (United States)

Melani, Federico; Mei, Davide; Pisano, Tiziana; Savasta, Salvatore; Franzoni, Emilio; Ferrari, Anna Rita; Marini, Carla; Guerrini, Renzo

2011-04-01

Cyclin-dependent kinase-like 5 (CDKL5) gene abnormalities cause an early-onset epileptic encephalopathy. We performed video-electroencephalography (video-EEG) monitoring early in the course of CDKL5-related epileptic encephalopathy in order to examine the early electroclinical characteristics of the condition. We used video-EEG to monitor six infants (five females, one male) with CDKL5-related epileptic encephalopathy (five mutations; one deletion), at ages 45 days to 12 months and followed them up to the ages of 14 months to 5 years (mean age 23 mo). We focused our analysis on the first year of life. The results were evaluated against those of a comparison group of nine infants (aged below 1y) with epileptic encephalography who had tested negative for CDKL5 mutations and deletions. One infant exhibited normal background activity, three exhibited moderate slowing, and two exhibited a suppression burst pattern. Two participants had epileptic spasms and four had a stereotyped complex seizure pattern, which we defined as a 'prolonged' generalized tonic-clonic event consisting of a tonic-tonic/vibratory contraction, followed by a clonic phase with series of spasms, gradually translating into repetitive distal myoclonic jerks. Seizure duration ranged from 2 to 4 minutes. The EEG correlate of each clinical phase included an initial electrodecremental event (tonic vibratory phase), irregular series of sharp waves and spike slow waves (clonic phase with series of spasms), and bilateral rhythmic sharp waves (time locked with myoclonus). Infants with CDKL5-related early epileptic encephalopathy can present in the first year of life with an unusual electroclinical pattern of 'prolonged' generalized tonic-clonic seizures. © The Authors. Journal compilation © Mac Keith Press 2011.

Distonia laríngea de adução: proposta e avaliação de protocolo de nasofibrolaringoscopia Adduction laryngeal dystonia: proposal and evaluation of nasofibroscopy

Directory of Open Access Journals (Sweden)

Noemi Grigoletto De Biase

2006-08-01

Full Text Available Distonias são desordens orgânicas do processamento motor central caracterizadas por contrações musculares involuntárias e espasmos à fonação nas formas laríngeas adutoras, com quebras de sonoridade. O diagnóstico é clínico e baseado na avaliação perceptivo-auditiva da voz e nasofibroscopia. OBJETIVO: O nosso objetivo é propor e avaliar um protocolo de exame de nasofibrolaringoscopia que contemple tarefas que evidenciem os espasmos e tarefas que diminuam ou façam desaparecer os espasmos, visando facilitar a análise e o diagnóstico. MATERIAL E MÉTODO: Estudo transversal. Análise de imagens de 15 videonasolaringoscopias de pacientes com distonia laríngea de adução por meio do protocolo proposto. RESULTADOS: A maior parte das tarefas de fala e não-fonatórias permitiram a identificação de espasmos e a diminuição ou desaparecimento destes. Propomos a exclusão de duas delas que não acrescentaram dados à avaliação. CONCLUSÃO: O protocolo foi útil na avaliação dos pacientes, mostrando mudança de comportamento da musculatura nas estruturas estudadas conforme as tarefas executadas.Dystonias are organic central motor processing disorders characterized by involuntary muscular contractions or incontrollable spasms induced by task-specific movements. Adduction laryngeal dystonias present with important speech impairments, with inappropriate spasms and abrupt voice breaks. The diagnosis is based on clinical features, evaluation by a speech therapist and transnasal fiber optic laryngoscopy. AIM: Our objective is to propose and evaluate a task-oriented transnasal fiber optic laryngoscopy protocol, which shows the spasms, and propose maneuvers that reduce or make them disappear, in order to facilitate the diagnosis. METHODS: transversal study. Analysis of the transnasal fiber optic laryngoscopy records of 15 patients with adductor laryngeal dystonia using the proposed protocol. RESULTS: most of the speech and non-vocal tasks

Facial tics

Science.gov (United States)

... are common in childhood. A chronic motor tic disorder also exists. It may last for years. This form is very rare compared to the common short-lived childhood tic. Tourette syndrome is a separate condition in which tics are a major symptom. Symptoms Tics may involve repeated, uncontrolled spasm-like ...

21 CFR 890.5860 - Ultrasound and muscle stimulator.

Science.gov (United States)

2010-04-01

..., muscle spasms, and joint contractures, but not for the treatment of malignancies. The device also passes... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Ultrasound and muscle stimulator. 890.5860 Section... Ultrasound and muscle stimulator. (a) Ultrasound and muscle stimulator for use in applying therapeutic deep...

Levetiracetam in spinal cord injury pain: a randomized controlled trial

DEFF Research Database (Denmark)

Finnerup, N B; Grydehøj, J; Bing, J

2009-01-01

. OBJECTIVES: The objective of the study was primarily to evaluate the efficacy of the anticonvulsant levetiracetam in patients with spinal cord injury (SCI) at- and below-level pain and secondarily to evaluate the effect on spasm severity. SETTING: Outpatients at two spinal cord units and a pain center...... severity following spinal cord injury....

Manual Vacuum Aspiration In The Management Of Post Abortal ...

African Journals Online (AJOL)

Eleven (11) patients with tetanus were managed by uterine evacuation using the manual vacuum aspiration at the University of Port Harcourt Teaching Hospital, Nigeria over a period of ten years. Eight (8) of the patients had uterine evacuation while in coma, while three (3) were conscious but had spasm and features of ...

Compare Of the West Syndrome with Other Syndromes in the Epileptic Encephalopathy - Kosovo Experience

Science.gov (United States)

Zeka, Naim; Gërguri, Abdurrahim; Bejiqi, Ramush; Retkoceri, Ragip; Vuciterna, Armend

2017-01-01

BACKGROUND: West Syndrome (WS) represents as a specific epileptic encephalopathy characterised with a unique type of attacks, called infantile spasms, severe forms of abnormalities in electroencephalographic (EEG) records as a hypsarythmias and delays in the psychomotoric development. The characteristics of the disease, mostly affecting male gender, are infantile spasms and typical findings in EEG as a hypsarythmia. Infantile spasms are a consequence of many factors in the undeveloped brain. AIM: We aimed: (1) to see the incidence of the illness and the spreading out because of gender in rapport with other syndromes in the epileptic encephalopathies group; (2) to show principles of the treatment for the illness; and (3) to present the effects of the disease in the psycho-motoric development of affected children. METHODS: The study was designed as a cross-sectional study of the patients with epileptic encephalopathies, treated in Paediatric Clinic in Prishtina, from 1st of January 2013 until the 31st of December 2015. RESULTS: From the cohort group of 97 children diagnosed with epileptic encephalopathies, in 14 of them clinical and EEG signs of WS were noted. The earliest age of disease manifestation was 74 days (± 63.8 days). On the group of children with WS, 13 of them with Natrium Valpropat were treated, with the doses of 301.9 mg (± 64.1). From the cohort group, in 89 children (91.8%) psychomotoric retardation was documented, within the higher reoccurrence in the undifferentiated epileptic encephalopathies (96%) and the WS (78.6%). CONCLUSION: WS is a frequent disease of the encephalopathies with the epileptogenic framework. The resistance in anticonvulsive therapy is huge, and psychomotoric retardation follows a big percentage of children with this syndrome. PMID:29362620

Extravesical (modified Gregoir Lich versus intravesical (Cohen′s ureteric reimplantation for vesicoureteral reflux in children: A single center experience

Directory of Open Access Journals (Sweden)

Krishnamoorthy Sriram

2016-01-01

Full Text Available Introduction: There are multiple techniques for surgical correction of vesicoureteral reflux (VUR. We compared the outcomes of extravesical versus Cohen′s reimplantation for VUR in children. Methods: Records of all children (n = 118 who underwent reimplantation for VUR between 2003 and 2014 were analyzed (male: female = 43:75. Children with secondary VUR, duplication anomalies, and ectopic ureter were excluded from our study. Extravesical reimplantation (EVR was performed bilateral in 32 children (Group 1a and unilateral in 19 (Group 1b, while bilateral Cohen′s reimplantation was performed in 67 (Group 2. Parameters compared were length of the surgical procedure, average duration of stay in the hospital, postoperative bladder spasms, significant hematuria >72 h, and long-term complications. Results: The mean age at operation was 15 months in Group 1, and 36 months in Group 2. The mean duration of surgery was significantly less (P = 0.0001 in Group 1a (n = 32; mean 104 min; standard deviation [SD] 18 min compared to Group 2 (n = 67; mean 128 min; SD 15 min. The mean (SD postoperative stay was significantly lower (P = 0.0001 at 4.5 (1.5 days in Group 1a compared to 6.5 (0.5 days in Group 2. Postoperative bladder spasms were significantly lower (P = 0.03 at 10/32 in Group 1a compared to 37/67 in Group 2. All patients responded well with anticholinergics. Postoperative hematuria and bladder spasms were significantly lower (P = 0.03 in Group 1a compared to Group 2. There was no significant difference in persistent VUR between Group 1 and Group 2. At 1 year follow, none of them had any evidence of ureteral obstruction. Conclusions: EVR has lower operative time, less postoperative discomfort and shorter hospital stay compared to Cohen′s reimplantation. Both techniques are equally effective in treating reflux.

Congenital hypoparathyroidism presenting as recurrent seizures in an adult

OpenAIRE

Acharya, Sourya; Shukla, Samarth; Singh, Dinesh; Deshpande, Rohit; Mahajan, S. N.

2012-01-01

Hypocalcemia due to hypoparathyroidism may manifest as serious neurologic symptoms such as seizures, movement disorders, or raised intracranial pressure. Several patients were observed to have these dangerous neurologic complications even without subtle signs of hypocalcemia like tetany, chvostek's sign or carpopedal spasms. We present a case of recurrent hypocalcemic seizures due to congenital hypoparathyroidism.

Association between nucleotide mutation of eNOS gene and serum ...

African Journals Online (AJOL)

Galaxy

2013-05-15

May 15, 2013 ... spasm among Japanese (Nakayama et al., 1999; Casas et al., 2006). It is believed that these mutations might result in altered NO metabolism and impaired .... ship between T-786C mutation of eNOS gene and CAD specifically in the Iranian population. To our knowledge, this polymorphism has never been ...

Milrinone infusion: A therapeutic option in coronary vasospasm after primary percutaneous transluminal coronary angioplasty

Directory of Open Access Journals (Sweden)

Singh Amarpal

2009-01-01

Full Text Available A 42-year-old male presented to the emergency department with acute chest pain. The electrocardiogram revealed inferior wall myocardial infarction. Emergency coronary angiography revealed total occlusion of the distal right coronary artery with thrombus. Patient was taken up for primary percutaneous coronary angioplasty with stenting of distal right coronary artery. Six hours following the procedure, the patient developed re-elevation of ST-segment in inferior leads of electrocardiogram and subsequent haemodynamic instability. Repeat coronary angiography revealed patent stent and coronary artery spasm in proximal part, which was relieved by intracoronary injection of nitroglycerine. After an hour, the patient re-developed symptoms of chest pain along with bradycardia, hypotension and ST segment elevation. Intravenous infusion of nitroglycerine did not improve the condition but produced persistent hypotension. Infusion of milrinone was then started. Over time, normalisation of electrocardiogram occurred. The patient was discharged in stable condition. This case suggests that milrinone may be effective in alleviating coronary artery spasm when the use of other agents fails

Successful Treatment with Microvascular Decompression Surgery of a Patient with Hemiparesis Caused by Vertebral Artery Compression of the Medulla Oblongata: Case Report and Review of the Literature.

Science.gov (United States)

Ren, Jibin; Sun, Hongtao; Diao, Yunfeng; Niu, Xuegang; Wang, Hang; Wei, Zhengjun; Yuan, Fei

2017-12-01

There are few reports on hemiparesis caused by vascular medullary compression, which can occur because of dolichoectasia of the vertebrobasilar arterial system. In this article, we report a case of vertebral artery compression of the medulla oblongata in a 67-year-old woman. The patient was hypertensive, and she developed hemiparesis and intermittent spasms over 5 years. These spasms had worsened during the last year. Cranial nerve magnetic resonance imaging showed compression of the medulla oblongata by the left vertebral artery. A motor evoked potential (MEP) examination showed abnormal conduction of MEPs of bilateral toe abductors. The patient underwent microvascular decompression surgery under general anesthesia through a retrosigmoid keyhole approach. This operation led to relief of vascular compression and symptomatic improvement. Our case suggests that detailed history, imaging studies, and electrophysiologic studies help lead to a correct and early diagnosis of hemiparesis caused by vascular compression of the rostral ventrolateral medulla. Microvascular decompression surgery improves patient symptoms, and intraoperative electrophysiologic monitoring helps to avoid injury to important adjacent nerves. Copyright © 2017 Elsevier Inc. All rights reserved.

Perinatal occipital lobe injury in children: analysis of twenty-one cases.

Science.gov (United States)

Wang, San-Mei; Yang, Chang-Shuan; Hou, Yu; Ma, Xiu-Wei; Feng, Zhi-Chun; Liao, Yu-Zhen

2012-12-01

This study used magnetic resonance imaging to analyze causes and clinical courses of pediatric occipital lobe injury. Patients undergoing magnetic resonance imaging for suspected bilateral occipital lobe injury at our Neurodevelopmental Department between July 2007 and June 2011 were included. We evaluated magnetic resonance imaging characteristics, clinical courses, electroencephalogram monitoring, and Denver Development Screen Test scores. Twenty-one infants were examined. Of these, 10 had been born preterm. Thirteen patients demonstrated hypoglycemia. Perinatal period hypoglycemia comprised the most common cause (71.4%) of occipital brain injury. Visual abnormalities were evident in 18 patients. Seventeen (80.9%) patients manifested epilepsy. Infantile spasms were observed in 13 cases (76.5%). According to Denver Development Screen Test assessment, 17 patients demonstrated delayed motor development. Motor function and language improved in 10 patients after effective control of their seizures. Hypoglycemia constitutes the most common cause of occipital injury in infants. Visual impairment, startle episodes, infantile spasms, and motor developmental delay comprise the most common complications, whereas language function is usually spared. Copyright © 2012 Elsevier Inc. All rights reserved.

[Treatment of vaginismus with EMDR: a report of two cases].

Science.gov (United States)

Torun, Fuat

2010-01-01

Vaginismus is a type of sexual dysfunction in which spasm of the vaginal musculature prevents penetrative intercourse. The main diagnostic criterion is the presence of recurrent or persistent involuntary spasm of the musculature of the outer third of the vagina that interferes with sexual intercourse. In many cases associated pain or the fear of pain may contribute to its persistence. Herein we report 2 patients that presented with vaginismus that developed secondary to childhood sexual trauma, which was treated with the Eye Movement Desensitization and Reprocessing (EMDR) technique. EMDR is a non-pharmacologic treatment for psychological trauma. Randomized controlled trials with posttraumatic stress disorder patients and with victims of sexual abuse have shown that EMDR is effective. The standard 8-phase EMDR protocol was used in both of the presented cases. Following 3 sessions of EMDR, the patients exhibited a substantial reduction in self-reported and clinician-rated anxiety, and a reduction in the credibility of dysfunctional beliefs concerning sexual intercourse. These findings support the notion that EMDR could be an effective treatment alternative for patients with vaginismus of traumatic etiology.

Treatment of atlantoaxial rotatory fixation with botulinum toxin muscle block and manipulation.

Science.gov (United States)

Lin, Chia-Hung; Chen, Chun-Jung; Chen, Chuan-Mu; Liao, Su-Lan; Raung, Shue-Ling; Tsai, Sen-Wei

2010-04-01

Slippage after reduction of atlantoaxial rotatory fixation (AARF) is usually treated with repeated cervical traction and brace immobilization. To date, no data have been published on the management of muscle spasm during treatment. Here, we describe the case of a 7-year-old girl with AARF for 1 month who visited our hospital for treatment. During physical examination, spasm of the sternocleidomastoid muscle was noted. The patient was treated with manipulative reduction, and slippage after reduction was managed with botulinum spasticity block of the sternocleidomastoid and splenius capitis muscles, and repeated manipulation. Cervical orthosis immobilization with a rehabilitation program of isometric contract-relax exercise for the neck was conducted for 3 months. The subject had full recovery from AARF at 1-year follow-up. This report demonstrates that, in selected cases of slippage after reduction from AARF, conservative management with manipulation under anesthesia is a good method, and the muscle components may play a crucial role in AARF. Copyright 2010 Elsevier. Published by Elsevier B.V. All rights reserved.

Side effects after myelography with Dimer Xsup(R) (Iocarmat) with subsequent positioning of the patients in the sitting or lying position

International Nuclear Information System (INIS)

Norstedt, M.

1982-01-01

92 patients were examined after lumbar myelography with the water-soluble contrast medium Iocarmat (Dimer Xsup(R)) in order to find out if side effects have any relation with the patient's position after the myelographic examination. In one group, the patients were laid with their upper part of the body positioned higher while the others were allowed to lie flat. The comparative investigation covering both groups of patients revealed the following results: 1) In 11% of the flat-lying patients there were generalised spasms which was not the case as far as the sitting patients were concerned. This is why the author advises against a flat positioning of the patient. 2) The frequency of headache decreased when the patients were lying flat which, however, does not mean a statistical significance. 3) Other side effects registered (myoclonus, tonic spasm in the legs, paraesthesia, increase in existing root pain and neck pain, nausea and vomiting) occurred in both groups nearly to the same extent, independently of the position of the patient. (orig./MG) [de

Usefulness of hyperventilation myocardial SPECT for diagnosing vasospastic angina pectoris

International Nuclear Information System (INIS)

Tahara, Yorio; Shimoyama, Katsuya; Nishimura, Tooru; Sasaki, Akira; Taya, Makoto; Mizuno, Haruyoshi; Ono, Akifumi; Tsuya, Tsuneo; Ishikawa, Kyozo

1990-01-01

We evaluated the usefulness of hyperventilation myocardial scintigraphy (HV-SPECT) for diagnosing vasospastic angina pectoris. The subjects consisted of 11 patients with rest angina and 13 with rest and exertional angina in whom coronary spasm was demonstrated by coronary angiography, and 6 with exertional angina in whom a significant stenotic lesion was observed by coronary angiography. Hyperventilation was performed at a rate of 40/min for 5 minutes in all the patients. Ischemia was considered to be present when defect and redistribution were observed by SPECT. HV-SPECT revealed positive findings in 8 patients with rest angina (73%) and 9 with exertional and rest angina (69%) but only in 1 with exertional angina (17%). Ischemic ST changes on monitor ECG were observed in 13 of the 30 patients (43%); ST was enhanced in 4 patients and depressed in 9 patients. In the patients with rest angina, HV-SPECT induced ischemic findings more frequently than exercise SPECT (73% vs 55%). HV-SPECT seems to be useful as a non-invasive method for diagnosing angina pectoris resulting from coronary spasm. (author)

Current Therapeutic Options for Esophageal Motor Disorders as Defined by the Chicago Classification.

Science.gov (United States)

Zerbib, Frank; Roman, Sabine

2015-07-01

With the development of high-resolution manometry and specific metrics to characterize esophageal motility, the Chicago Classification has become the gold standard for the diagnosis of esophageal motor disorders. Major and significant disorders, that is, never observed in healthy subjects, are achalasia, esophagogastric junction outflow obstruction, distal esophageal spasm, absent peristalsis, and hypercontractile (Jackhammer) esophagus. Achalasia subtyping is relevant to predict the response to endoscopic and surgical therapies as several studies suggest that, pneumatic dilation is less effective than Heller myotomy, in type III achalasia. Peroral endoscopic myotomy, initially developed in expert centers, is a promising technique for the treatment of achalasia. The medical therapeutic options for distal esophageal spasm and hypercontractile esophagus are smooth muscle relaxants and pain modulators. Intraesophageal injection of botulinum toxin might be an interesting option for treatment of these disorders but further studies are required to determine the optimal injection protocol and the best candidates based on manometric patterns. The treatment of hypotensive motility disorders is disappointing and relies mainly on dietary and lifestyle changes as no effective esophageal prokinetic is currently available.

Communicating hydrocephalus subsequent to purulent meningitis

International Nuclear Information System (INIS)

Suzuki, Hiroshi; Ogawa, Kimio; Hibio, Shuichi; Goto, Kazuhiko; Shiihara, Hiroaki

1984-01-01

Based on CT findings one year after shunting, ventricular dialtion was classified into five degrees for examining prognosis of communicating hydrocephalus subsequent to purulent meningitis. Factors causing and aggravating hydrocephalus were also examined. Patients with hydrocephalus tended to have spasms frequently as the first symptom within one month after birth when there were few characteristic findings. Spasm and disturbance of consciousness occurred frequently during the first week of the occurrence of disease. Large numbers of cells in the spinal fluid and high volume of spinal cord protein were persistent in patients aged one month or less. Chloride transport decreased in patients aged two months or more. The occurrence of syndrome of the pyramidal tract, eye symptoms, movement of head to the left and right, and involuntary movement suggested serious conditions of the disease. Disturbance of movement could be relieved by giving adequate antibiotics as soon as meningitis was discovered within one month after birth and by giving chloramphenicol when symptoms suggesting the development of serious conditions occurred. However, mental retardation and epilepsy could not be prevented. (Namekawa, K.)

DSA in digital replantations

International Nuclear Information System (INIS)

Wang Liuhong; Chao Ming; Jiang Dingyao; Zhang Guangqiang; Wu Jianjun; Chen Xianyi; Li Bin; Sun Jihong

2008-01-01

Objective: To assess revascularization and vessel anastomosis in digital replantations with DSA. Methods: Twelve cases of digital replantations underwent digital subtract angiography during 2 to 4 days after fingers reattachment. The vessel anastomosis, hemodynamics, stenosis and discontinuation were investigated. The unobstructed and smooth anastomosis was suggested as early stage survival of the reattached fingers, the spasm and stenosis of the reattached vessels were considered as mild vascular crisis, and the discontinuation of hemodynamics were indicated as severe vascular crisis. Results: The total 27 vessels were clearly displayed on DSA. Of these vessels, 23 vessels were unobstructed and smooth, all digits were survived. Diagnosis coincidence of early stage survival was 100% (23/23). Two vessels were obstructed, which were testified having thrombus by operation research. The other 2 vessels were spasm, the digits were also survived ultimately by expectant treatment. All 4 abnormal vessel anatomosis were found by DSA. Conclusion: DSA is important modality in assessing revascularization and blood circulation for digital replantations, guiding in dealing with the vascular crisis, and in predicting early stage survival of the reattached digits. (authors)

Clinical aspects of coronary heart diseases without myocardial infarction and in the chronic infarction phase

International Nuclear Information System (INIS)

Kober, G.

1984-01-01

Reductions in coronary vessel diameters of more than 50% are usually caused by atherosclerosis, seldom by predominant spasme. They can lead via an inadequate myocardial perfusion to angina pectoris. Whereas in patients with organic coronary stenoses exertional angina is typical, patients with coronary spasms usually tend to angina at rest. Depending on the symptoms, a stable or unstable angina can be distinguished from the status anginosus and a variant form of angina. During anginal attacks signs of myocardial ischemia can often be seen in the electrocardiogram. This applies to patients with and without earlier myocardial infarctions. When pathologic ECG-changes can already be seen in the ECG at rest, difficulties often arise during evaluation of the exercise-ECG. In those cases, diagnosis can be improved by additional investigations, especially with radionuclear techniques. For a conclusive diagnosis, left heart catheterization and a functional coronary angiography is necessary. Coronary heart disease can be treated either by drug therapy, coronary angioplasty or bypass surgery. The same diagnostic procedures are suitable for the evaluation of a therapeutical success and for primary diagnosis. (orig.) [de

Unilateral facial pain and lung cancer

International Nuclear Information System (INIS)

Shakespeare, T.P.; Stevens, M.J.

1996-01-01

Facial pain in lung cancer patients may be secondary to metastatic disease to the brain or skull base. Since 1983 there have been 19 published reports of hemi-facial pain as a non-metastatic complication of lung carcinoma. This report describes an additional case in whom unilateral face pain preceded the diagnosis of lung cancer by 9 months. A clinical diagnosis of trigeminal neuralgia was made after a normal brain CT scan. Later on the patient complained of global lethargy, weight loss and haemoptysis. A chest X-ray disclosed a 6 cm right hilar mass that was further defined with a whole body CT scan. The neural mechanism of the unilateral facial pain is discussed and the literature reviewed. 14 refs., 1 tab

Unilateral facial pain and lung cancer

Energy Technology Data Exchange (ETDEWEB)

Shakespeare, T.P.; Stevens, M.J. [Royal North Shore Hospital, Crows Nest, NSW (Australia)

1996-02-01

Facial pain in lung cancer patients may be secondary to metastatic disease to the brain or skull base. Since 1983 there have been 19 published reports of hemi-facial pain as a non-metastatic complication of lung carcinoma. This report describes an additional case in whom unilateral face pain preceded the diagnosis of lung cancer by 9 months. A clinical diagnosis of trigeminal neuralgia was made after a normal brain CT scan. Later on the patient complained of global lethargy, weight loss and haemoptysis. A chest X-ray disclosed a 6 cm right hilar mass that was further defined with a whole body CT scan. The neural mechanism of the unilateral facial pain is discussed and the literature reviewed. 14 refs., 1 tab.

Facial nerve hemangioma: a rare case involving the vertical segment.

Science.gov (United States)

Ahmadi, Neda; Newkirk, Kenneth; Kim, H Jeffrey

2013-02-01

This case report and literature review reports on a rare case of facial nerve hemangioma (FNH) involving the vertical facial nerve (FN) segment, and discusses the clinical presentation, imaging, pathogenesis, and management of these rare lesions. A 53-year-old male presented with a 10-year history of right hemifacial twitching and progressive facial paresis (House-Brackmann grading score V/VI). The computed tomography and magnetic resonance imaging studies confirmed an expansile lesion along the vertical FN segment. Excision and histopathologic examination demonstrated FNH. FNHs involving the vertical FN segment are extremely rare. Despite being rare lesions, we believe that familiarity with the presentation and management of FNHs are imperative. Laryngoscope, 2012. Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.

Development of future indications for BOTOX.

Science.gov (United States)

Brin, Mitchell F

2009-10-01

Since the late 1970s, local injections of BoNT have provided clinical benefit for patients with inappropriately contracting muscles with or without pain or sensory disturbance. Marketing authorization for some BoNTs, depending on country, include core indications of dystonia (blepharospasm and cervical dystonia), large muscle spastic disorders (not yet approved in the United States, e.g., adult post-stroke spasticity and equinus foot deformity), hyperhidrosis and aesthetic. Subsequent development has extended to selected conditions characterized by recurrent or chronic pain (migraine headache), and urologic indications (neurogenic/idiopathic overactive bladder; prostate hyperplasia), with multiple additional opportunities available. Portfolio management requires a careful individual opportunity assessment of scientific and technical aspects (basic science foundation, potential to treat unmet medical need, product-specific risk in specific populations, therapeutic margin/safety profile, and probability of successful registration pathway). This article describes ongoing development targets for BOTOX.

Topical botulinum toxin.

Science.gov (United States)

Collins, Ashley; Nasir, Adnan

2010-03-01

Nanotechnology is a rapidly growing discipline that capitalizes on the unique properties of matter engineered on the nanoscale. Vehicles incorporating nanotechnology have led to great strides in drug delivery, allowing for increased active ingredient stability, bioavailability, and site-specific targeting. Botulinum toxin has historically been used for the correction of neurological and neuromuscular disorders, such as torticollis, blepharospasm, and strabismus. Recent dermatological indications have been for the management of axillary hyperhydrosis and facial rhytides. Traditional methods of botulinum toxin delivery have been needle-based. These have been associated with increased pain and cost. Newer methods of botulinum toxin formulation have yielded topical preparations that are bioactive in small pilot clinical studies. While there are some risks associated with topical delivery, the refinement and standardization of delivery systems and techniques for the topical administration of botulinum toxin using nanotechnology is anticipated in the near future.

Key Clinical Features to Identify Girls with "CDKL5" Mutations

Science.gov (United States)

Bahi-Buisson, Nadia; Nectoux, Juliette; Rosas-Vargas, Haydee; Milh, Mathieu; Boddaert, Nathalie; Girard, Benoit; Cances, Claude; Ville, Dorothee; Afenjar, Alexandra; Rio, Marlene; Heron, Delphine; Morel, Marie Ange N'Guyen; Arzimanoglou, Alexis; Philippe, Christophe; Jonveaux, Philippe; Chelly, Jamel; Bienvenu, Thierry

2008-01-01

Mutations in the human X-linked cyclin-dependent kinase-like 5 ("CDKL5") gene have been shown to cause infantile spasms as well as Rett syndrome (RTT)-like phenotype. To date, less than 25 different mutations have been reported. So far, there are still little data on the key clinical diagnosis criteria and on the natural history of…

Anesthetic management of a patient with stiff-person syndrome and thymoma: a case report

Institute of Scientific and Technical Information of China (English)

QIN Xiang; WANG Dong-xin; WU Xin-min

2006-01-01

@@ Stiff-person syndrome (SPS, also called stiff-man syndrome) is a rare neurological disease with autoimmune features. It is characterized by fluctuating and progressive muscle rigidity, and episodic spasm that prominently involve axial and limb musculature.1,2 Herein we report a case of anesthetic management of a patient with SPS for thymectomy and review several other cases.

D-bifunctional protein deficiency associated with drug resistant infantile spasms

NARCIS (Netherlands)

Buoni, Sabrina; Zannolli, Raffaella; Waterham, Hans; Wanders, Ronald; Fois, Alberto

2007-01-01

Peroxisomal disorders appear with a frequency of about 1:5000 in newborns. Peroxisomal D-bifunctional protein (D-BP), encoded by the HSD17B4 gene (gene ID: 3294; locus tag: HGNC:5213, chromosome 5q2; official symbol: HSD17B4; name: hydroxysteroid (17-beta) dehydrogenase; gene type: protein coding)

Children’s Hospice

Science.gov (United States)

2007-05-01

which treatment is exclusively palliative from diagnosis. Spinal muscular atrophy, trisomy 13 or 18, severe infantile asphyxia...exploded b/c of it, has pulmonary stenosis, slight cerebral palsy husband Active Duty 7 diagnosed with infantile spasms at 4 mo, has cerebral palsy...officer white ? multiple disabilities, ventilator- dependent Active Duty Marine Corps African American ? 1 with diabetes ; 1 with asthma

Diagnosis of the bile reflux into the introhepatic biliary ducts using using radionuclide hepatocholecystography

International Nuclear Information System (INIS)

Mtvaradze, A.S.

1984-01-01

To reveal functional disorders of bile secretion 165 patients with diseases of gastrointestinal tract were examined. It was established that radionuclide hepatocholecystography enables to reveal dyskinesia of bile secretion, as well as bile reflux into the intrahepatic biliary ducts. Bile reflux into the intrahepatic biliary ducts is observed more often in patients with spasm of oddii sphincter and hyperkinetic dyskinesia of bile cyst

Primary Esophageal Motility Disorders: Beyond Achalasia

OpenAIRE

Schlottmann, Francisco; Patti, Marco G.

2017-01-01

The best-defined primary esophageal motor disorder is achalasia. However, symptoms such as dysphagia, regurgitation and chest pain can be caused by other esophageal motility disorders. The Chicago classification introduced new manometric parameters and better defined esophageal motility disorders. Motility disorders beyond achalasia with the current classification are: esophagogastric junction outflow obstruction, major disorders of peristalsis (distal esophageal spasm, hypercontractile esoph...

Osteogénesis por distracción esquelética maxilomandibular: Análisis de esta novedosa terapéutica OSTEOGENESIS BY MAXILLOMANDIBULAR SKELETAL DISTRACTION: ANALYSIS OF THIS NOVEL THERAPEUTICS

Directory of Open Access Journals (Sweden)

Dadonim Vila Morales

2005-04-01

Full Text Available El proceso de generación de nuevo hueso por estiramiento del callo óseo se denomina osteogénesis por distracción. Este proceso se aplica también a los huesos del macizo craneofacial, y en especial a la mandíbula, para tratar defectos mandibulares que aparecen en diferentes síndromes, como el Treacher Collins, microsomía hemifacial, Pierre Robins, apnea obstructiva del sueño y otras deformidades secundarias a anquilosis de la articulación temporomandibular y traumas. Se realizó una revisión bibliográfica del tema. Nuestro estudio de 45 series publicadas arrojó que el tipo de distractor más usado es el extraoral, que el hueso más tratado es la mandíbula, que solo el 4,4 % de los autores realizan estudios cefalométricos para la evaluación de los resultados y que las complicaciones más frecuentes son la avulsión parcial de los pines, dolor en la ATM y limitación de la apertura bucal.The process of generation of a new bone by lengthening the osteal callus is called osteogenesis by distraction. This process is also applied to bones of the craniofacial skeleton and, specially, to the mandible to treat mandibular defects appearing in different syndromes, such as Treacher Collins, hemifacial microsomy, Pierre Robins, obstructive sleeping apnea, and other deformities secondary to ankylosis of the temporomandibular joint and traumas. A bibliographic review of the topic was made. Our study of 45 series published showed that the extraoral was the most used type of distractor, that the mandible was the most treated bone, that only 4.4 % of the authors conduct cephalometric studies to evaluate the results, and that partial avulsion of the pins, pain in the TMA and limitation to open the mouth were the most frequent complications.

Quality of life differences in patients with right- versus left-sided facial paralysis: Universal preference of right-sided human face recognition.

Science.gov (United States)

Ryu, Nam Gyu; Lim, Byung Woo; Cho, Jae Keun; Kim, Jin

2016-09-01

We investigated whether experiencing right- or left-sided facial paralysis would affect an individual's ability to recognize one side of the human face using hybrid hemi-facial photos by preliminary study. Further investigation looked at the relationship between facial recognition ability, stress, and quality of life. To investigate predominance of one side of the human face for face recognition, 100 normal participants (right-handed: n = 97, left-handed: n = 3, right brain dominance: n = 56, left brain dominance: n = 44) answered a questionnaire that included hybrid hemi-facial photos developed to determine decide superiority of one side for human face recognition. To determine differences of stress level and quality of life between individuals experiencing right- and left-sided facial paralysis, 100 patients (right side:50, left side:50, not including traumatic facial nerve paralysis) answered a questionnaire about facial disability index test and quality of life (SF-36 Korean version). Regardless of handedness or hemispheric dominance, the proportion of predominance of the right side in human face recognition was larger than the left side (71% versus 12%, neutral: 17%). Facial distress index of the patients with right-sided facial paralysis was lower than that of left-sided patients (68.8 ± 9.42 versus 76.4 ± 8.28), and the SF-36 scores of right-sided patients were lower than left-sided patients (119.07 ± 15.24 versus 123.25 ± 16.48, total score: 166). Universal preference for the right side in human face recognition showed worse psychological mood and social interaction in patients with right-side facial paralysis than left-sided paralysis. This information is helpful to clinicians in that psychological and social factors should be considered when treating patients with facial-paralysis. Copyright © 2016 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Study on placental blood flow in late pregnancy by intravenous sup(99m)Tc method

International Nuclear Information System (INIS)

Kitagawa, Hiroshi

1985-01-01

A method for the continuous recording of uteroplacental blood flow (PBF) in late pregnancies by using sup(99m)Tc-albumin has been described. 1) The PBF curve of toxemia of pregnancy has been plotted to indicate small artery spasm in proving ischemic necrosis of placenta. 2) In the PBF of placental insufficiency evidenced by the values for urinary E 3 , an unfavorable build-up and a delayed build-up time were observed. The pathologic diagnosis showed condensation, fusion and necrosis of villi. 3) In the PBF in which a intrauterine fetal death (IUFD) was caused by placental factors, a sudden change in the PBF was observed showing the presence of an ischemia. 4) In the PBF of pregnancy with diabetes, a large wave pattern change was observed indicating a decrease in the PBF. The pathologic diagnosis showed the fusion, hyalinization and necrosis of villi. 5) The PBF wave patterns were classified into four kinds: (1) normal pattern, (2) angio-spasm pattern, (3) delayed build-up pattern, (4) circulation pattern. It has become clear that these abnormal wave patterns are frequently observed in toxemia of pregnancy, placental insufficiency and pregnancy with diabetes. (author)

THE MANAGEMENT OF LIMITED MANDIBULAR MOVEMENT CAUSED BY CONDYLAR FRACTURE WITH REPOSITIONING SPLINT

Directory of Open Access Journals (Sweden)

Ira Tanti

2015-06-01

Full Text Available Fractures of the neck of condyle usually are the result of a blow to the mandible. A lateral blow to the body of the mandible commonly causes a contralateral condyle fracture. There are many signs and symptoms of a condylar fracture, for example crepitation, deviation of the mandible to the side of injury, and spasm of the associated group of muscles. These will result in a functional disability, which is usually seen as a limited mandibular movement. This paper reported a patient with a fracture of the condylar neck. Patient had been treated with closed reduction and immobilization for 2 months. After that, she felt that her bite was changed, she could not occlude her teeth well, and she had clicking sound in the right joint when she opened her mouth. Besides that, patient had difficulties to move the mandible to the left side, and she could not open her mouth widely. The patient was treated with a repositioning splint and she had to do some jaw exercises. The purposes were to regain the position of condyle, to reduce the muscle spasm and finally got the normal jaw movement.

Radiosurgery of epidermoid tumors with gamma knife. Possiblity of radiosurgical nerve decompression

International Nuclear Information System (INIS)

Kida, Yoshihisa; Yoshimoto, Masayuki; Hasegawa, Toshinori; Fujitani, Shigeru

2006-01-01

Long-term results of radiosurgery for epidermoid tumors are reported. There are 7 cases including 2 males and 5 females, ages ranging from 6 to 46 (mean: 33.3 years). At radiosurgery whole tumor was covered in 4 cases and partially covered in 3 cases in attempting to relieve cranial nerve signs like trigeminal neuralgia and facial spasm. The mean maximum and marginal doses were 25.6 Gy and 14.6 Gy respectively. In the mean follow-up of 52.7 months, all the tumors showed good tumor control without any progression and tumor shrinkage has been confirmed in 2 out of the 7 cases. Symptomatic trigeminal neuralgia improved or disappeared in all 4 cases and facial spasm disappeared in one. No neurological deterioration was found in any of the cases after the treatment. In conclusion, it is apparent that epidermoid tumors do respond well to radiosurgery and the accompanying hyperactive dysfunction of cranial nerves is significantly improved by gamma knife treatment with either entire or partial tumor coverage. Therefore the radiosurgical nerve decompression for epidermoid tumor seems to be achieved by gamma-radiosurgery. (author)

Extracranial Facial Nerve Schwannoma Treated by Hypo-fractionated CyberKnife Radiosurgery.

Science.gov (United States)

Sasaki, Ayaka; Miyazaki, Shinichiro; Hori, Tomokatsu

2016-09-21

Facial nerve schwannoma is a rare intracranial tumor. Treatment for this benign tumor has been controversial. Here, we report a case of extracranial facial nerve schwannoma treated successfully by hypo-fractionated CyberKnife (Accuray, Sunnyvale, CA) radiosurgery and discuss the efficacy of this treatment. A 34-year-old female noticed a swelling in her right mastoid process. The lesion enlarged over a seven-month period, and she experienced facial spasm on the right side. She was diagnosed with a facial schwannoma via a magnetic resonance imaging (MRI) scan of the head and neck and was told to wait until the facial nerve palsy subsides. She was referred to our hospital for radiation therapy. We planned a fractionated CyberKnife radiosurgery for three consecutive days. After CyberKnife radiosurgery, the mass in the right parotid gradually decreased in size, and the facial nerve palsy disappeared. At her eight-month follow-up, her facial spasm had completely disappeared. There has been no recurrence and the facial nerve function has been normal. We successfully demonstrated the efficacy of CyberKnife radiosurgery as an alternative treatment that also preserves neurofunction for facial nerve schwannomas.

Examination of Acute Sensitivity to Morphine and Morphine Self-Administration Following Physical and Environmental Stressors in Fischer-344 and Lewis Female Rats

Science.gov (United States)

1997-01-16

within an individual’s life span can often be described by an inverted-U shaped function, with drug use beginning in the early teen years, peaking during...the late teens and early twenties, and then declining shortly afterward (Kandel & Raveis, 1989). Unfortunately, however, some individuals continue to...heart rate and blood pressure, anorexia , gooseflesh, muscle spasms, irritability, insomnia, vomiting, diarrhea, and tremor. Without treatment or

Ergotism of the lower limb complicating DHE-heparin thrombosis prophylaxis. Observation by serial angiography

Energy Technology Data Exchange (ETDEWEB)

Warmuth-Metz, M.

1988-10-01

Today ergotism is becoming more and more important as a complication in the treatment of migraine headache or thrombosis prophylaxis with DHE heparin. Although complete recovery is seldom reported in the current literature, in our case it was possible to resolve a spasm of the left lower limb completely by early diagnosis and adaequate pharmacological treatment. The case was well documented by serial angiography.

A System Approach to Navy Medical Education and Training. Appendix 11. Advanced General Duty Corpsman.

Science.gov (United States)

1974-08-31

curricula based upon job analysis was implemented to a level of methodology determination. These methods and curriculum materials constituted a third...Technician 8495 Dermatology Technician 8496 Embalming Technician 8497 Medical Illustration Technician 8498 Medical Equipment Repair Technician 8703 DT...IGIVE MEDICATED BATH 11 IGIVE MASSAGE FOP RELAXATION (SEDATIVE MASSAGED 12 IGIVE MASSAGE TO REDUCE MUSCLE SPASM 13 1APPLY WET COMPRESSES/SOAKS/PACKS 14

Occlusal Therapy in the Management of Chronic Orofacial Pain

OpenAIRE

Bush, Francis M.

1984-01-01

Review of the literature indicates that most routine orofacial dysfunctions are characterized by deep pain. Various disorders of the masticatory systems, particularly musculoskeletal conditions, are thought to be triggered by occlusal disharmonies. The pain component develops following a pattern of bruxism, muscle hyperactivity, fatigue and spasm. Treatment for most disorders has been to modify the occlusion, although the rational for doing so appears questionable.

Finding a better drug for epilepsy: The mTOR pathway as an antiepileptogenic target

Science.gov (United States)

Galanopoulou, Aristea S.; Gorter, Jan A.; Cepeda, Carlos

2012-01-01

Summary The mTOR signaling pathway regulates cell growth, differentiation, proliferation and metabolism. Loss of function mutations in upstream regulators of mTOR have been highly associated with dysplasias, epilepsy and neurodevelopmental disorders. These include tuberous sclerosis, which is due to mutations in TSC1 or TSC2 genes, mutations in phosphatase and tensin homolog (PTEN) as in Cowden syndrome, polyhydramnios, megalencephaly, symptomatic epilepsy syndrome (PMSE) due to mutations in the STE20-related kinase adaptor alpha (STRADalpha), and neurofibromatosis type 1 attributed to neurofibromin 1 mutations. Inhibition of the mTOR pathway with rapamycin may prevent epilepsy and improve the underlying pathology in mouse models with disrupted mTOR signaling, due to PTEN or TSC mutations. However the timing and duration of its administration appear critical in defining the seizure and pathology-related outcomes. Rapamycin application in human cortical slices from patients with cortical dysplasias reduces the 4-aminopyridine induced oscillations. In the multiple-hit model of infantile spasms, pulse high dose rapamycin administration can reduce the cortical overactivation of the mTOR pathway, suppresses spasms and has disease-modifying effects by partially improving cognitive deficits. In post-status epilepticus models of temporal lobe epilepsy, rapamycin may ameliorate the development of epilepsy-related pathology and reduce the expression of spontaneous seizures, but its effects depend on the timing and duration of administration, and possibly the model used. The observed recurrence of seizures and epilepsy-related pathology after rapamycin discontinuation suggests the need for continuous administration to maintain the benefit. However, the use of pulse administration protocols may be useful in certain age-specific epilepsy syndromes, like infantile spasms, whereas repetitive pulse rapamycin protocols may suffice to sustain a long-term benefit in genetic disorders

Exploring relationships of catheter-associated urinary tract infection and blockage in people with long-term indwelling urinary catheters.

Science.gov (United States)

Wilde, Mary H; McMahon, James M; Crean, Hugh F; Brasch, Judith

2017-09-01

To describe and explore relationships among catheter problems in long-term indwelling urinary catheter users, including excess healthcare use for treating catheter problems. Long-term urinary catheter users experience repeated problems with catheter-related urinary tract infection and blockage of the device, yet little has been reported of the patterns and relationships among relevant catheter variables. Secondary data analysis was conducted from a sample in a randomised clinical trial, using data from the entire sample of 202 persons over 12 months' participation. Descriptive statistics were used to characterise the sample over time. Zero-inflated negative binomial models were employed for logistic regressions to evaluate predictor variables of the presence/absence and frequencies of catheter-related urinary tract infection and blockage. Catheter-related urinary tract infection was marginally associated with catheter blockage. Problems reported at least once per person in the 12 months were as follows: catheter-related urinary tract infection 57%, blockage 34%, accidental dislodgment 28%, sediment 87%, leakage (bypassing) 67%, bladder spasms 59%, kinks/twists 42% and catheter pain 49%. Regression analysis demonstrated that bladder spasms were significantly related to catheter-related urinary tract infection and sediment amount, and catheter leakages were marginally significantly and positively related to catheter-related urinary tract infection. Frequencies of higher levels of sediment and catheter leakage were significantly associated with higher levels of blockage, and being female was associated with fewer blockages. Persons who need help with eating (more disabled) were also more likely to have blockages. Catheter-related urinary tract infection and blockage appear to be related and both are associated with additional healthcare expenditures. More research is needed to better understand how to prevent adverse catheter outcomes and patterns of problems in

Seizure characteristics of epilepsy in childhood after acute encephalopathy with biphasic seizures and late reduced diffusion.

Science.gov (United States)

Ito, Yuji; Natsume, Jun; Kidokoro, Hiroyuki; Ishihara, Naoko; Azuma, Yoshiteru; Tsuji, Takeshi; Okumura, Akihisa; Kubota, Tetsuo; Ando, Naoki; Saitoh, Shinji; Miura, Kiyokuni; Negoro, Tamiko; Watanabe, Kazuyoshi; Kojima, Seiji

2015-08-01

The aim of this study was to clarify characteristics of post-encephalopathic epilepsy (PEE) in children after acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), paying particular attention to precise diagnosis of seizure types. Among 262 children with acute encephalopathy/encephalitis registered in a database of the Tokai Pediatric Neurology Society between 2005 and 2012, 44 were diagnosed with AESD according to the clinical course and magnetic resonance imaging (MRI) findings and were included in this study. Medical records were reviewed to investigate clinical data, MRI findings, neurologic outcomes, and presence or absence of PEE. Seizure types of PEE were determined by both clinical observation by pediatric neurologists and ictal video-electroencephalography (EEG) recordings. Of the 44 patients after AESD, 10 (23%) had PEE. The period between the onset of encephalopathy and PEE ranged from 2 to 39 months (median 8.5 months). Cognitive impairment was more severe in patients with PEE than in those without. Biphasic seizures and status epilepticus during the acute phase of encephalopathy did not influence the risk of PEE. The most common seizure type of PEE on clinical observation was focal seizures (n = 5), followed by epileptic spasms (n = 4), myoclonic seizures (n = 3), and tonic seizures (n = 2). In six patients with PEE, seizures were induced by sudden unexpected sounds. Seizure types confirmed by ictal video-EEG recordings were epileptic spasms and focal seizures with frontal onset, and all focal seizures were startle seizures induced by sudden acoustic stimulation. Intractable daily seizures remain in six patients with PEE. We demonstrate seizure characteristics of PEE in children after AESD. Epileptic spasms and startle focal seizures are common seizure types. The specific seizure types may be determined by the pattern of diffuse subcortical white matter injury in AESD and age-dependent reorganization of the brain

Clinical features of tuberous sclerosis complex in children with epilepsy

Directory of Open Access Journals (Sweden)

Dong LI

2014-12-01

Full Text Available Objective To explore the clinical features of tuberous sclerosis complex (TSC in children with epilepsy.  Methods The clinical data of 36 TSC children complicated with epilepsy were analyzed retrospectively.  Results All children had epilepsy as first symptom: 23 cases of seizures happened on age less than one year old (63.89%, 8 cases on age 1-3 years old (22.22%, and 5 cases on age more than 3 years old (13.89% . Main attack types were as follows: 12 cases with focal seizures (33.33% , 5 cases with generalized seizures (13.89% , 7 cases with spasms (19.44% and 12 cases (33.33% with mixed seizures. Mental retardation was found in 19 cases (67.86%. Among 30 cases who received brain MRI examination, all had subependymal nodules, 26 cases (86.67% were complicated with cortical and subcortical nodules, 2 cases (6.67% with subependymal giant cell astrocytoma (SEGA, one case (3.33% with pachygyria. EEG examination showed interictal epileptiform discharges in 34 cases (94.44% , including multifocal discharges in 12 patients (35.29% , generalized discharges in 8 patients (23.53% , focal discharges and hypsarrhythmia in 7 patients (20.59% respectively. Seizures were monitored in 8 patients, and the types included spasms in 4 cases, focal seizures in 3 cases, and myoclonic seizures in one case. Seventeen cases (47.22% took one single antiepileptic drug, 12 cases (33.33% took two drugs, and 7 cases (19.44% took three drugs, while one of them underwent surgical treatment. After 0.50-10 year follow-up, seizures free happened in 7 cases (19.44%, improved in 16 cases (44.44%, and invalid in 13 cases (36.11%.  Conclusions Epilepsy is the most common nervous system performance in children TSC, and most seizures begin to happen in infants. The main types of onset are partial seizures and spasms. Most of the patients get improved with antiepileptic drugs. doi: 10.3969/j.issn.1672-6731.2014.12.011

A new treatment for focal dystonias: incobotulinumtoxinA (Xeomin®, a botulinum neurotoxin type A free from complexing proteins

Directory of Open Access Journals (Sweden)

Jimenez-Shahed J

2011-12-01

Full Text Available Joohi Jimenez-ShahedDepartment of Neurology, Baylor College of Medicine, Houston, TX, USAAbstract: Dystonia is a movement disorder of uncertain pathogenesis that is characterized by involuntary and inappropriate muscle contractions which cause sustained abnormal postures and movements of multiple or single (focal body regions. The most common focal dystonias are cervical dystonia (CD and blepharospasm (BSP. The first-line recommended treatment for CD and BSP is injection with botulinum toxin (BoNT, of which two serotypes are available: BoNT type A (BoNT/A and BoNT type B (BoNT/B. Conventional BoNT formulations include inactive complexing proteins, which may increase the risk for antigenicity, possibly leading to treatment failure. IncobotulinumtoxinA (Xeomin®; Merz Pharmaceuticals GmbH, Frankfurt, Germany is a BoNT/A agent that has been recently Food and Drug Administration-approved for the treatment of adults with CD and adults with BSP previously treated with onabotulinumtoxinA (Botox®; Allergen, Inc, Irvine, CA – a conventional BoNT/A. IncobotulinumtoxinA is the only BoNT product that is free of complexing proteins. The necessity of complexing proteins for the effectiveness of botulinum toxin treatment has been challenged by preclinical and clinical studies with incobotulinumtoxinA. These studies have also suggested that incobotulinumtoxinA is associated with a lower risk for stimulating antibody formation than onabotulinumtoxinA. In phase 3 noninferiority trials, incobotulinumtoxinA demonstrated significant improvements in CD and BSP symptoms in both primary and secondary measures, compared with baseline, and met criteria for noninferiority versus onabotulinumtoxinA. In placebo-controlled trials, incobotulinumtoxinA also significantly improved the symptoms of CD and BSP, with robust outcomes in both primary and secondary measures. The use of incobotulinumtoxinA has been well tolerated in all trials, with an adverse event profile similar

Medical marijuana: Medical necessity versus political agenda

OpenAIRE

Clark, Peter A.; Capuzzi, Kevin; Fick, Cameron

2011-01-01

Summary Marijuana is classified by the Drug Enforcement Agency (DEA) as an illegal Schedule I drug which has no accepted medical use. However, recent studies have shown that medical marijuana is effective in controlling chronic non-cancer pain, alleviating nausea and vomiting associated with chemotherapy, treating wasting syndrome associated with AIDS, and controlling muscle spasms due to multiple sclerosis. These studies state that the alleviating benefits of marijuana outweigh the negative ...

CDKL5, a novel MYCN-repressed gene, blocks cell cycle and promotes differentiation of neuronal cells

OpenAIRE

Valli, Emanuele; Trazzi, Stefania; Fuchs, Claudia; Erriquez, Daniela; Bartesaghi, Renata; Perini, Giovanni; Ciani, Elisabetta

2012-01-01

Mutations in the CDKL5 (cyclin-dependent kinase-like 5) gene are associated with a severe epileptic encephalopathy (early infantile epileptic encephalopathy type 2, EIEE2) characterized by early-onset intractable seizures, infantile spasms, severe developmental delay, intellectual disability, and Rett syndrome (RTT)-like features. Despite the clear involvement of CDKL5 mutations in intellectual disability, the function of this protein during brain development and the molecular mechanisms invo...

Chamomile: A herbal medicine of the past with bright future

OpenAIRE

Srivastava, Janmejai K; Shankar, Eswar; Gupta, Sanjay

2010-01-01

Chamomile is one of the most ancient medicinal herbs known to mankind. It is a member of Asteraceae/Compositae family and represented by two common varieties viz. German Chamomile (Chamomilla recutita) and Roman Chamomile (Chamaemelum nobile). The dried flowers of chamomile contain many terpenoids and flavonoids contributing to its medicinal properties. Chamomile preparations are commonly used for many human ailments such as hay fever, inflammation, muscle spasms, menstrual disorders, insomni...

Myopathy in acute hypothyroidism

OpenAIRE

Ma, JTC; Yu, YL; Kung, AWC

1987-01-01

Hypothyroid myopathy has so far been reported in long standing cases of hypothyroidism. We describe two adult patients with myopathy associated with acute transient hypothyroidism. Both presented with severe muscle aches and cramps, stiffness and spasms. Muscle enzymes were markedly elevated and electromyography in one patient showed myopathic features. Histological changes were absent in muscle biopsy, probably because of the short duration of metabolic disturbance. The myopathy subsided pro...

Myopathy in acute hypothyroidism.

OpenAIRE

Kung, A. W.; Ma, J. T.; Yu, Y. L.; Wang, C. C.; Woo, E. K.; Lam, K. S.; Huang, C. Y.; Yeung, R. T.

1987-01-01

Hypothyroid myopathy has so far been reported in long standing cases of hypothyroidism. We describe two adult patients with myopathy associated with acute transient hypothyroidism. Both presented with severe muscle aches and cramps, stiffness and spasms. Muscle enzymes were markedly elevated and electromyography in one patient showed myopathic features. Histological changes were absent in muscle biopsy, probably because of the short duration of metabolic disturbance. The myopathy subsided pro...

[Proctalgia fugax. Differential diagnosis and therapy of fleeting anal cramp].

Science.gov (United States)

Staude, G

1992-05-30

Proctalgia fugax--short-lived anal spasm--is a common, extremely unpleasant, painful condition that occurs completely unexpectedly, often waking the victim at night. Scientific assessment is difficult on account of the functional nature of the condition and its multifactorial genesis. Before the patient is labeled "anal neurotic", however, he/she should be investigated by a specialist. The results of treating the rarely absent pathological organic findings give rise to optimism.

Hypomagnesaemia Posing as Hypoparathyroidism

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Sonia Nasreen Ahmad

2015-09-01

Full Text Available Persistent hypocalcaemia usually is a presentation of hypoparathyroidism. When it is coupled with low parathormone levels the diagnosis seems almost certain. However, the fact that hypomagnesaemia can give rise to both hypocalcaemia and low parathormone levels gives us points to ponder. This case depicts a young woman soon after childbirth presenting with carpopedal spasm and biochemical abnormalities. Subsequent correction of hypomagnesaemia readjusts calcium, potassium and parathormone levels.

Risk Factors, Co-Morbid Conditions and Epidemiology of Autism in Children

Science.gov (United States)

2015-10-01

more likely to receive a diagnosis of status epilepticus , and 12 times more likely to have an inpatient hospital stay compared to children without ASD...more likely children with autism are to be diagnosed with a specific seizure problem including status epilepticus , infantile spasms and petit mal...APPENDICITIS: There was no statistically significant difference in the rate of appendicitis based on ASD status . However, those with ASD had 1.06

Estrabismo após toxina botulínica para fins estéticos

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Marta Halfeld Ferrari Alves Lacordia

2011-06-01

Full Text Available RESUMO Nas últimas décadas, a toxina botulínica tem sido aplicada para fins terapêuticos em inúmeras afecções. A toxina do tipo A foi aprovada primeiramente em 1989 para o uso em estrabismo, blefaroespasmo e espasmo hemifacial de pacientes maiores que 12 anos de idade. A observação de que pacientes tratados de blefaroespasmo com toxina botulínica apresentavam um efeito adicional de diminuição das rugas de expressão inspirou estudos sobre a aplicação cosmética da toxina. Recentemente, a toxina botulínica do tipo A foi aprovada para fins estéticos. Este trabalho relata um caso de ocorrência de estrabismo após aplicação de toxina botulínica na face para fins estéticos.

[Diagnosis of facial and craniofacial asymmetry].

Science.gov (United States)

Arnaud, E; Marchac, D; Renier, D

2001-10-01

Craniofacial asymmetry is caused by various aetiologies but clinical examination remains the most important criteria since minor asymmetry is always present. The diagnosis can be confirmed by anthropometric measurements and radiological examinations but only severe asymmetries or asymmetries with an associated functional impairment should be treated. The treatment depends on the cause, and on the time of appearance. Congenital asymmetries might be treated early, during the first year of life if a craniosynostosis is present. Hemifacial microsomia are treated later if there is no breathing impairment. Since the pediatricians have recommended the dorsal position for infant sleeping, an increasing number of posterior flattening of the skull has been appearing, and could be prevented by adequate nursing. Other causes of craniofacial asymmetries are rare and should be adapted to the cause (tumors, atrophies, neurological paralysis, hypertrophies) by a specialized multidisciplinar team.

Impact of lesion location on procedural and acute angiographic outcomes in patients with critical limb ischemia treated for peripheral artery disease with orbital atherectomy: A CONFIRM registries subanalysis.

Science.gov (United States)

Lee, Michael S; Mustapha, Jihad; Beasley, Robert; Chopra, Paramjit; Das, Tony; Adams, George L

2016-02-15

This analysis compares the procedural and acute angiographic outcomes in patients with critical limb ischemia (CLI) treated with orbital atherectomy in above-the-knee (ATK)/popliteal (POP) lesions versus below-the-knee (BTK) lesions. Lesion location affects the procedural outcomes and the opportunity for limb salvage in patients with CLI suffering from peripheral artery disease (PAD). The CONFIRM registry series was analyzed and includes 1109 real-world patients (1544 lesions) suffering from CLI treated with orbital atherectomy. The rates of dissection, perforation, slow flow, vessel closure, spasm, embolism, and thrombus formation were compared between CLI patients with ATK/POP lesions and BTK lesions. Patients with ATK/POP lesions had a higher final residual stenosis (10 vs. 9%; P = 0.004) and use of more adjunctive therapies (e.g. balloons and stents; 1.3 vs. 1.1%; P atherectomy was successful in CLI patients regardless of lesion location. BTK lesions were associated with increased rates of perforation, slow flow and spasm which may be explained by more challenging procedural characteristics in these patients such as smaller vessel size and tortuosity. The higher incidence of emboli in ATK/POP lesions is most likely attributed to the higher prevalence of severe calcium observed in this cohort. © 2015 Wiley Periodicals, Inc.

Vascular changes in sporadic ergotism. Epidemiology, pathogenesis, clinical practice, and diagnosis with special regard to angiographic documentation

Energy Technology Data Exchange (ETDEWEB)

Hagen, B.

1986-08-01

Whereas the epidemic form of ergotism has been rare in the 20th century, the sporadic form has a wide distribution as a consequence of therapy with ergot-derivative drugs. Favourable prognosis of the condition, with complete recovery from the ischemic peripheral circulatory disorders can be obtained by early diagnosis. Precise evaluation of the history, with recognition of treatment of migraine headache or postpartum hemorraghe with ergot alkaloids, can be determinative in diagnosis. The specific pattern of the angiographic findings, as we saw it in 6 cases within the last 4 years, decisively confirms the diagnosis. We could demonstrate thread-, thorn- and hour glass-like narrowing of the vessels, due to spasm. Total occlusion with the development of collaterals may occur, but we saw no thrombus formation. The stenotic arterial segments had smooth margins. The distribution of the involved arteries was more focal than generalized and more peripheral than central. In two cases spasm could be abolished immediately by a pharmaceutical (i.a. injection of tolazoline) or by anesthetic procedures (halothane). The phenomenologic data, together with the complete reversal of the circulatory ischemic disorders after therapy (particularly the absolute ban of ergots), is so specific that other diseases of ischemic nature (emboli, arteriosclerosis, Buerger's disease, fibromuscular hyperplasia) can be excluded.

The Pathophysiology and Etiology of Vaginismus

Directory of Open Access Journals (Sweden)

Cherng-Jye Jeng

2004-03-01

Full Text Available Vaginismus is defined as an involuntary spasm of the pelvic muscles surrounding the outer third of the vagina, especially the perineal muscles and the levator ani muscles. Its severe form usually makes penetration virtually impossible and causes a severe, burning pain, and leads to unconsummated marriage. There appears to be basic agreement that vaginismus is a psychosociologic disorder with phobic elements resulting from actual or imagined negative experiences with penetration attempts. Fear and anxiety concerning penetration is expressed physiologically via the involuntary vaginal muscle spasm that characterizes vaginismus. Since 1547, when vaginismus was first described, thousands of research papers have been published on this female sexual disorder. However, the etiology of vaginismus remains controversial. Women with vaginismus generally experience shame, disgust and dislike toward their genitals. They frequently have or have had other phobias. They are usually overprotected by their fathers and have been “good girls” since childhood. Their sexual partners are usually kind, gentle, considerate and passive “nice guys”. The male partner's lack of aggressiveness actually leads to unconsummation of the marriage. The sexually secure husband can usually overcome mild degrees of vaginismus by persistent but firm penile insertion. For moderate to severe degrees of vaginismus, medical intervention is usually necessary to lead to consummation of the marriage.

Evaluation of coronary hemodynamics and exercise 201Tl-myocardial scintigraphy in patients with vasospastic angina

International Nuclear Information System (INIS)

Matsumura, Kentaro; Nakase, Emiko; Haiyama, Tohru; Hasegawa, Akira; Saito, Takayuki

1992-01-01

To clarify the coronary hemodynamics and myocardial perfusion in patients with vasospastic angina, we performed exercise 201 Tl-myocardial scintigraphy (planar and SPECT) in 72 patients and left coronary digital subtraction angiography (DSA) in 37 patients without significant organic coronary artery stenosis. Coronary artery spasm was documented by coronary angiography in all patients. Fifty-four patients (75%) developed exercise-induced 201 Tl-myocardial perfusion defect on SPECT. 201 Tl pulmonary uptake (L/H) was significantly increased in patients with vasospastic angina. Especially, L/H was higher in patients with multiple small perfusion defect on 201 Tl-SPECT, so that exercise-induced left ventricular dysfunction existed in patients with vasospastic angina and especially in cases of multiple small perfusion defect on 201 Tl-SPECT. The left coronary circulation time (CCT) was prolonged in patients with vasospastic angina. The mechanism of prolonged CCT is still unknown, but we suspected that prolonged CCT was induced by increased peripheral coronary vascular resistance in patients with vasospastic angina. It was concluded that the peripheral coronary circulation was disturbed in patients with vasospastic angina, but its abnormal coronary circulation had no relation to location of spasm-induced vessels. We concluded that impaired coronary microcirculation was taken a part of pathophysiology in vasospastic angina. (author)

Fournier's gangrene during ACTH therapy.

Science.gov (United States)

Numoto, Shingo; Kurahashi, Hirokazu; Azuma, Yoshiteru; Numaguchi, Atsushi; Nakahara, Kozaburo; Tainaka, Takahisa; Takasu, Michihiko; Yamakawa, Kiyoshi; Nago, Nozomi; Muto, Taichiro; Kitagawa, Yoshiro; Okumura, Akihisa

2017-05-01

Fournier's gangrene is an infectious necrotizing fasciitis of the perineal, genital, or perianal regions and is uncommon in children. Adrenocorticotropic hormone (ACTH) is effective for the treatment of infantile spasms; however, suppression of immune function is one of the major adverse effects of this approach. We encountered a 2-month-old boy with infantile spasms that had been treated with ACTH and had developed complicating Fournier's gangrene. Strangulation of a right inguinal hernia was observed after ACTH treatment. Although surgical repair was successful and no intestinal injuries were detected, swelling and discoloration of the right scrotum developed in association with pyrexia and a severe inflammatory response. A scrotal incision revealed pus with a putrid smell. The patient was subsequently diagnosed with Fournier's gangrene complicated by septic shock and disseminated intravascular coagulation. Extensive debridement and intensive care was performed. Enterobactor aerogenes, methicillin-resistant Staphylococcus aureus, and Enterococcus faecalis were isolated from the pus. Meropenem, teicoplanin, and clindamycin were administered to control the bacterial infection. The patient was discharged from the intensive care unit without any obvious neurological sequelae. Suppression of immune function associated with ACTH therapy may have been related to the development of Fournier's gangrene in this case. Copyright © 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Prognostic value of SPECT in newly diagnosed symptomatic west syndrome

Energy Technology Data Exchange (ETDEWEB)

Goto, Megumi; Suzuki, Yasuhiro; Kato, Tomomi; Futagi, Yasuyuki [Osaka Medical Center and Research Inst. for Maternal and Child Health, Izumi (Japan)

1999-07-01

In 19 cases with newly diagnosed symptomatic West syndrome, we assessed interictal regional cerebral blood flow (rCBF) before ACTH therapy with single photon emission computed tomography (SPECT). Based on the SPECT findings, we divided these cases into 3 groups: normal rCBF (Group A, 7 cases), abnormal rCBF corresponding to cerebral lesions on MRI and CT (Group B, 6 cases), and abnormal rCBF in areas different from lesions on MRI and CT (Group C, 6 cases). We compared clinical features, response to initial treatment, and short-term outcome among these 3 groups. No significant differences were found in clinical characteristics (sex, age of onset, prior seizures before onset of spasms, EEG findings). Four cases in Group B (67%) and 5 in Group C (83%) showed complete cessation of spasms after initial treatment (high dose vitamin B{sub 6}{yields}zonisamide{yields}ACTH therapy), while in Group A only 2 patients (29%, p>0.05; compared to Group B or Group C) responded. Although not statistically significant, short-term prognosis (both seizures and development) after a mean follow-up of 2 years and 8 months was also worst in Group A. Our results suggest that normal SPECT findings may be predictive of unfavorable prognosis in infants with symptomatic West syndrome. (author)

The Pathophysiology and Etiology of Vaginismus

OpenAIRE

Cherng-Jye Jeng

2004-01-01

Vaginismus is defined as an involuntary spasm of the pelvic muscles surrounding the outer third of the vagina, especially the perineal muscles and the levator ani muscles. Its severe form usually makes penetration virtually impossible and causes a severe, burning pain, and leads to unconsummated marriage. There appears to be basic agreement that vaginismus is a psychosociologic disorder with phobic elements resulting from actual or imagined negative experiences with penetration attempts. Fear...

Rehabilitation of Bell's palsy patient with complete dentures

OpenAIRE

Muthuvignesh, J.; Kumar, N. Suman; Reddy, D. Narayana; Rathinavelu, Pradeep; Egammai, S.; Adarsh, A.

2015-01-01

Facial nerve disorders may be of sudden onset and more often of unknown etiology. Edema of the facial nerve within the fallopian canal results in Bell's palsy. This causes compression of the nerve and affects the microcirculation. Many authors have suggested treatment for facial nerve paralysis ranging from simple physiotherapy to complicated microvascular decompression. It more often results in symptoms like synkinesis and muscle spasm after the decompression surgery of the nerve because of ...