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Sample records for central cord syndrome

  1. Central Cord Syndrome

    Science.gov (United States)

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  2. MAGNETIC RESONANCE IMAGING OF ACUTE CENTRAL CORD SYNDROME: CORRELATION WITH PROGNOSIS

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective. To report the magnetic resonance imaging (MRI) findings of acute cervical central cord syndrome and to determine their correlation with the prognosis. Methods. MRI findings of 35 patients with acute central cord syndrome were studied and compared with the recovery rate of ASIA score at presentation and in follow-up. Results. MRI data demonstrated spinal cord compression for 32 patients, spinal cord swelling for 16 patients, and abnormal signal intensity within the spinal cord for 19 patients, including 14 with edema and 3 with hematoma. No significant difference of the recovery rate was noted between the patients treated nonoperatively and operatively (P >0.05). There was a significant inverse correlation between the recovery rate and the degree of spinal cord compression as shown in MRI scans (P<0.01). The presence of hematoma in MRI scans was associated with poor prognosis, as demonstrated by a significant difference of the recovery rate (P< 0.01) among the patients with normal intensity, edema and hematoma within the spinal cord. Conclusions. MRI scans provide an efficient assistance for decision-making and accurate prognostic information regarding neurological function, and therefore should routinely be performed within the early phase of acute central cord syndrome.

  3. Anterior cervical surgery methods for central cord syndrome without radiographic spinal fracture-dislocation

    Institute of Scientific and Technical Information of China (English)

    Chengwei JING; Qin FU; Xiaojun XU

    2009-01-01

    This study was aimed to explore the anterior cervical surgery methods to treat central cord syndrome without radiographic spinal fracture-dislocation (CCSWORFD), retrospectively analyze the cases of CCSWORFD, and evaluate the curative effect of anterior cervical surgery methods for CCSWORFD. Twenty four cases of CCSWORFD (19 males and 5 females), all suffering from cervical hyperextension injury, between 45-68 (average 59) years old, were operated on by anterior cervical surgery methods. Among these, 18 cases had been followed up for 6-24 (average 15) months; 18 cases, who had anterior decompression and plate fixation with titanium mesh bone grafting or iliac bone grafting achieved reliable effects based on the Japanese Orthopedics Association (JOA) evaluation (improved scores of cases with titanium mesh bone grafting, t = 2.800, P0.05). Most of these cases had degeneration of cervical vertebra. The decompression which relieves the oppression to the spinal cord can help lessen edema of the spinal cord, and early fixation for stability of cervical vertebra is better for the recovery of spinal cord injury. Anterior operation with titanium mesh bone grafting or iliac bone grafting are both reliable curative methods for CCSWORFD, and titanium mesh bone grafting can avoid the trauma of the supplying graft. Mesh bone grafting can also shorten hospital stay.

  4. Gait kinematic analysis in patients with a mild form of central cord syndrome

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    Crespo-Ruiz Beatriz

    2011-02-01

    Full Text Available Abstract Background Central cord syndrome (CCS is considered the most common incomplete spinal cord injury (SCI. Independent ambulation was achieved in 87-97% in young patients with CCS but no gait analysis studies have been reported before in such pathology. The aim of this study was to analyze the gait characteristics of subjects with CCS and to compare the findings with a healthy age, sex and anthropomorphically matched control group (CG, walking both at a self-selected speed and at the same speed. Methods Twelve CCS patients and a CG of twenty subjects were analyzed. Kinematic data were obtained using a three-dimensional motion analysis system with two scanner units. The CG were asked to walk at two different speeds, at a self-selected speed and at a slower one, similar to the mean gait speed previously registered in the CCS patient group. Temporal, spatial variables and kinematic variables (maximum and minimum lower limb joint angles throughout the gait cycle in each plane, along with the gait cycle instants of occurrence and the joint range of motion - ROM were compared between the two groups walking at similar speeds. Results The kinematic parameters were compared when both groups walked at a similar speed, given that there was a significant difference in the self-selected speeds (p Conclusions The gait pattern of CCS patients showed a decrease of knee and ankle sagittal ROM during level walking and an increase in hip abduction to increase base of support. The findings of this study help to improve the understanding how CCS affects gait changes in the lower limbs.

  5. The effect of increased T2 signal intensity in the spinal cord on the injury severity and early neurological recovery in patients with central cord syndrome.

    Science.gov (United States)

    Schroeder, Gregory D; Hjelm, Nik; Vaccaro, Alexander R; Weinstein, Michael S; Kepler, Christopher K

    2016-05-01

    OBJECTIVE The aim of this paper was to compare the severity of the initial neurological injury as well as the early changes in the American Spinal Injury Association (ASIA) motor score (AMS) between central cord syndrome (CCS) patients with and without an increased T2 signal intensity in their spinal cord. METHODS Patients with CCS were identified and stratified based on the presence of increased T2 signal intensity in their spinal cord. The severity of the initial neurological injury and the progression of the neurological injury over the 1st week were measured according to the patient's AMS. The effect of age, sex, congenital stenosis, surgery within 24 hours, and surgery in the initial hospitalization on the change in AMS was determined using an analysis of variance. RESULTS Patients with increased signal intensity had a more severe initial neurological injury (AMS 57.6 vs 75.3, respectively, p = 0.01). However, the change in AMS over the 1st week was less severe in patients with an increase in T2 signal intensity (-0.85 vs -4.3, p = 0.07). Analysis of variance did not find that age, sex, Injury Severity Score, congenital stenosis, surgery within 24 hours, or surgery during the initial hospitalization affected the change in AMS. CONCLUSIONS The neurological injury is different between patients with and without an increased T2 signal intensity. Patients with an increased T2 signal intensity are likely to have a more severe initial neurological deficit but will have relatively minimal early neurological deterioration. Comparatively, patients without an increase in the T2 signal intensity will likely have a less severe initial injury but can expect to have a slight decline in neurological function in the 1st week.

  6. Central Neuropathic Pain Syndromes.

    Science.gov (United States)

    Watson, James C; Sandroni, Paola

    2016-03-01

    Chronic pain is common in patients with neurologic complications of a central nervous system insult such as stroke. The pain is most commonly musculoskeletal or related to obligatory overuse of neurologically unaffected limbs. However, neuropathic pain can result directly from the central nervous system injury. Impaired sensory discrimination can make it challenging to differentiate central neuropathic pain from other pain types or spasticity. Central neuropathic pain may also begin months to years after the injury, further obscuring recognition of its association with a past neurologic injury. This review focuses on unique clinical features that help distinguish central neuropathic pain. The most common clinical central pain syndromes-central poststroke pain, multiple sclerosis-related pain, and spinal cord injury-related pain-are reviewed in detail. Recent progress in understanding of the pathogenesis of central neuropathic pain is reviewed, and pharmacological, surgical, and neuromodulatory treatments of this notoriously difficult to treat pain syndrome are discussed.

  7. Bilateral upper-extremity deep vein thrombosis following central cord syndrome

    OpenAIRE

    Onmez, Hilal; Cingoz, Havva Turac; Kucuksen, Sami; Anliacık, Emel; Yaşar, Ozan; Yilmaz, Halim; Salli, Ali

    2013-01-01

    Deep vein thrombosis (DVT) is a common complication following spinal cord injury (SCI). Although DVT of the upper extremity is much less common than DVT of the lower extremities, the risk of pulmonary embolism following upper-extremity DVT should not be disregarded.

  8. Tethered Spinal Cord Syndrome

    Science.gov (United States)

    ... roots may be cut to relieve pain. In adults, surgery to free (detether) the spinal cord can reduce the size ... is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord ...

  9. Vocal cord collapse during phrenic nerve-paced respiration in congenital central hypoventilation syndrome [v1; ref status: indexed, http://f1000r.es/Sh1pwj

    Directory of Open Access Journals (Sweden)

    Mark C Domanski

    2012-11-01

    Full Text Available Objective: Phrenic nerve pacing can be used to treat congenital central hypoventilation syndrome (CCHS. We report how the lack of normal vocal cord tone during phrenic paced respiration can result in passive vocal cord collapse and produce obstructive symptoms. Methods: We describe a case of passive vocal cord collapse during phrenic nerve paced respiration in a patient with CCHS. As far as we know, this is the first report of this etiology of airway obstruction. The patient, a 7-year-old with CCHS and normal waking vocal cord movement, continued to require nightly continuous positive airway pressure (CPAP despite successful utilization of phrenic nerve pacers. On direct laryngoscopy, the patient’s larynx was observed while the diaphragmatic pacers were sequentially engaged. Results: No abnormal vocal cord stimulation was witnessed during engaging of either phrenic nerve stimulator. However, the lack of normal inspiratory vocal cord abduction during phrenic nerve-paced respiration resulted in vocal cord collapse and partial obstruction due to passive adduction of the vocal cords through the Bernoulli effect. Bilateral phrenic nerve stimulation resulted in more vocal cord collapse than unilateral stimulation. Conclusions: The lack of vocal cord abduction on inspiration presents a limit to phrenic nerve pacers.

  10. Central Pain Syndrome

    Science.gov (United States)

    ... or hands. Central pain syndrome often begins shortly after the causative injury or damage, but may be delayed by months or even years, especially if it is related to post-stroke pain. × Definition Central pain syndrome is a neurological ...

  11. MAGNETIC RESONANCE IMAGING OF ACUTE CENTRAL CORD SYNDROME: CORRELATION WITH PROGNOSIS

    Institute of Scientific and Technical Information of China (English)

    戴力扬

    2001-01-01

    Objective. To report the magnetic resonance imaging (MRI) findings of acute cervical central cord syndrome and to determine their correlation with the prognosis. Methods. MRI findings of 35 patients with acute central cord syndrome were studied and compared with the recovery rate of ASIA score at presentation and in follow-up. Results. MRI data demonstrated spinal cord compression for 32 patients, spinal cord swelling for 16 patients,and abnormal signal intensity within the spinal cord for 19 patients, including 14 with edema and 3 with hematoma. No significant difference of the recovery rate was noted between the patients treated nonoperatively and operatively ( P > 0. 05). There was a significant inverse correlation between the recovery rate and the degree of spinal cord compression as shown in MRI scans ( P < 0.01 ). The presence of hematoma in MRI scans was associated with poor prognosis, as demonstrated by a significant difference of the recovery rate ( P < 0. 01) among the patients with normal intensity, edema and hematoma within the spinal cord. Conclusions. MRI scans provide an efficient assistance for decision-making and accurate prognostic information regarding neurological function, and therefore should routinely be performed within the early phase of acute central cord syndrome.

  12. Comparison of outcomes of treating central cord injury syndrome with surgery and conservative regime%脊髓中央管损伤综合征手术和保守治疗的疗效比较

    Institute of Scientific and Technical Information of China (English)

    韩玮; 胡志毅; 张宁; 殷国勇

    2011-01-01

    目的 比较前路手术、后路手术及保守治疗脊髓中央管损伤综合征的疗效.方法 脊髓中央管损伤综合征患者37例,前路手术治疗24例,后路手术治疗7例,保守治疗6例.比较治疗前、后的JOA评分.结果 37例患者均获得随访4~24个月.术后改善率:前路手术组(68.21±25.57)%,后路手术组(60.61±9.63)%,均明显高于保守治疗组的(46.41±7.18)%(P<0.05).结论 前路手术及后路手术治疗脊髓中央管损伤综合征效果明显好于保守治疗.%Objective To compare the outcomes of treating central cord injury syndrome with surgery and conservative regime. Methods The treatments of 37 cases with central cord injury syndrome were performed with surgery via anterior aproach in 24 cases, with surgery via posterior aproach in 7 cases, and with conservative therapy in 6 cases evaluated by Japanese Orthopaedic Association(JOA) score. Results All cases were followed up for 4 to 24 months. The recovery rate was significantly higher in the patients underwent surgery than that in those recieved conservative therapy [(68. 21± 25. 57 )% and (60. 61 ± 9. 63)% vs. (46. 41 ± 7. 18) % ] (P< 0.05). Conclusion The efficiency of surgical treatment for cervical central cord injury via anterior or posterior aproach is better than that of conservative therapy.

  13. Magnetic resonance imaging of spinal cord syndromes

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    Einsiedel, H. von; Stepan, R.

    1985-05-01

    Thirty-four patients with intramedullary space-occupying lesions or cord compression syndromes were examined with a resistive and two different superconductive magnetic resonance (MR) imaging units. Studies were done primarily by the spin-echo (SE) technique and in the majority of patients different pulse sequences were used. Images with short echo-time (TE) and short recovery-time (TR) were best for demonstration of spinal cord anatomy, for depicting cystic portions in intramedullary tumours and for showing syringomyelia. Solid intramedullary tumours showed normal cord signal intensity. Images with prolonged TE and TR predominantly enhanced CSF signal intensity and, to a more considerable extent, solid intramedullary tumours. Thus, the diameter of the subarachnoid space and the presence of a solid intramedullary tumour, not concomittant with a significant enlargement of the spinal cord, could only be recognized on these prolonged SE images. Major advantages of MR in comparison to CT are that the spinal cord can be imaged in the sagittal plane and that beam hardening artifacts do not occur; in comparison to myelography the cord can be imaged directly by MR. Partial volume is a major limitation of MR, not only in the preferably applied sagittal plane. The choice of slice thickness adequate to the diameter of the lesion and straight positioning of the patient for sagittal single slice midline images are fundamental for reliable MR investigations. Another limitation to MR is that cortical bone gives no signal. The actual diameter of the spinal canal therefore cannot be correctly appreciated and consequently it was difficult or impossible to assess spinal stenosis.

  14. [Central anticholinergic syndrome].

    Science.gov (United States)

    Fernández Urretavizcaya, P; Cenoz Osinaga, J C; Jáuregui Garía, M L; Gállego Culleré, J

    1991-10-01

    Two new cases of anticolinergic central syndrome are described. The first case, a 8 year old girl, suffered a severe encefalopathy after topical application of mydriatic cholirio as an aid in a rutine study of ocular refraction. The second case, 67 year old man presented a severe neurological picture after accidental intake of a silvester plantground (Atropa belladonna). His neurological condition returned quickly to normal whith administration of physostigmine. Differents aspects of the etiology, clinical picture and diagnosis are discussed with special emphasis in patients with delirium or acute confusional states. Finally, attention is drawn in the necessity of a properly use of anticholinergic drugs overcoat in aged or children.

  15. Diagnosis and surgical treatment of terminal syringomyelia within spinal cord combined with tethered cord syndrome

    OpenAIRE

    Jing-cheng XIE; Wang, Zhen-Yu; Chen, Xiao-Dong

    2016-01-01

    Objective To summarize the clinical manifestations, imaging characteristics and experience of surgical treatment of spinal cord terminal syringomyelia with tethered cord syndrome (TCS).  Methods and Results Clinical data of 10 patients with spinal cord syringomyelia combined with TCS surgically treated under microscope from January 1999 to March 2014 in our hospital were retrospectively analyzed. There were 3 males and 7 females with average age of 15.06 years old (ranged from 2 to 35 y...

  16. Transitory spinal cord swelling in a 6-year-old boy with Guillain-Barre syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Delhaas, T. [Section of Child Neurology, Wilhelmina Children`s Hospital, University Hospital for Children and Youth, Utrecht (Netherlands)]|[Department of Pediatrics, University Hospital Maastricht, P0 Box 5800, 6202 AZ Maastricht (Netherlands); Kamphuis, D.J. [Section of Child Neurology, Wilhelmina Children`s Hospital, University Hospital for Children and Youth, Utrecht (Netherlands); Witkamp, T.D. [Department of Radiology, University Hospital Utrecht, Utrecht (Netherlands)

    1998-07-01

    A 6-year-old boy developed progressive motor weakness and areflexia. The clinical picture, combined with electrophysiological findings, indicated a diagnosis of Guillain-Barre syndrome (GBS). MRI on admission revealed spinal cord swelling and increased signal intensity within the cord. It is concluded that, since a degree of central nervous system involvement can occasionally be part of the spectrum of GBS, swelling of the spinal cord without contrast enhancement does not exclude a diagnosis of GBS. (orig.) With 2 figs., 8 refs.

  17. Imaging central pain syndromes.

    Science.gov (United States)

    Veldhuijzen, Dieuwke S; Greenspan, Joel D; Kim, Jong H; Coghill, Robert C; Treede, Rolf-Detlef; Ohara, Shinji; Lenz, Frederick A

    2007-06-01

    Anatomic, functional, and neurochemical imaging studies have provided new investigative tools in the study of central pain. High-resolution imaging studies allow for precise determination of lesion location, whereas functional neuroimaging studies measure pathophysiologic consequences of injury to the central nervous system. Additionally, magnetic resonance spectroscopy evaluates lesion-induced neurochemical changes in specific brain regions that may be related to central pain. The small number of studies to date precludes definitive conclusions, but the recent findings provide information that either supports or refutes current hypotheses and can serve to generate new ideas.

  18. Spinal cord stimulation in chronic pain syndromes

    NARCIS (Netherlands)

    ten Vaarwerk, IAM; Staal, MJ

    1998-01-01

    Spinal cord stimulation (SCS) has been used for more than 30 years now, and although it has shown to be effective under certain well-described conditions of chronic pain, conclusive evidence on its effectiveness is still sparse. There is a need for more prospective and methodological good studies, i

  19. Tethered spinal cord syndrome with symptomatic onset in adulthood

    Institute of Scientific and Technical Information of China (English)

    HE Shi-sheng; ZHAO Ying-chuan; SHI Zhi-cai; LI Ming; HOU Tie-sheng; ZHANG Ye; WU Yun-gang

    2009-01-01

    @@ Tethered spinal cord syndrome(TCS)is a condition of overstretching or compression of the caudal part of the spinal cord caused by various spinal lesions,such as a tight filum terminale or an intraspinal lipoma.~(1-9) Though it is a well-recognized cause of neurological deterioration in childhood,its symptomatic onset in adulthood is uncommon.~(10-23) Eleven cases of TCS are presented here.In addition,their related clinical features,surgical procedures and outcomes are investigated.

  20. Spinal cord ischemia: aetiology, clinical syndromes and imaging features

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    Weidauer, Stefan [Frankfurt Univ., Sankt Katharinen Hospital Teaching Hospital, Frankfurt am Main (Germany). Dept. of Neurology; Hattingen, Elke; Berkefeld, Joachim [Frankfurt Univ., Frankfurt am Main (Germany). Inst. of Neuroradiology; Nichtweiss, Michael

    2015-03-01

    The purpose of this study was to analyse MR imaging features and lesion patterns as defined by compromised vascular territories, correlating them to different clinical syndromes and aetiological aspects. In a 19.8-year period, clinical records and magnetic resonance imaging (MRI) features of 55 consecutive patients suffering from spinal cord ischemia were evaluated. Aetiologies of infarcts were arteriosclerosis of the aorta and vertebral arteries (23.6 %), aortic surgery or interventional aneurysm repair (11 %) and aortic and vertebral artery dissection (11 %), and in 23.6 %, aetiology remained unclear. Infarcts occurred in 38.2 % at the cervical and thoracic level, respectively, and 49 % of patients suffered from centromedullar syndrome caused by anterior spinal artery ischemia. MRI disclosed hyperintense pencil-like lesion pattern on T2WI in 98.2 %, cord swelling in 40 %, enhancement on post-contrast T1WI in 42.9 % and always hyperintense signal on diffusion-weighted imaging (DWI) when acquired. The most common clinical feature in spinal cord ischemia is a centromedullar syndrome, and in contrast to anterior spinal artery ischemia, infarcts in the posterior spinal artery territory are rare. The exclusively cervical location of the spinal sulcal artery syndrome seems to be a likely consequence of anterior spinal artery duplication which is observed preferentially here. (orig.)

  1. Diagnosis and surgical treatment of terminal syringomyelia within spinal cord combined with tethered cord syndrome

    Directory of Open Access Journals (Sweden)

    Jing-cheng XIE

    2016-04-01

    Full Text Available Objective To summarize the clinical manifestations, imaging characteristics and experience of surgical treatment of spinal cord terminal syringomyelia with tethered cord syndrome (TCS.  Methods and Results Clinical data of 10 patients with spinal cord syringomyelia combined with TCS surgically treated under microscope from January 1999 to March 2014 in our hospital were retrospectively analyzed. There were 3 males and 7 females with average age of 15.06 years old (ranged from 2 to 35 years old. The course of disease ranged from 3 months to 20 years (average 42.17 months. Among those patients, one patient presented hydromyelia, 8 patients suffered from meningeal cyst within the sacral canal, and one patient were concurrent with sacral dermal sinus. The weakness of lower extremities, especially distal limbs, was the main clinical manifestation. Five patients were accompanied with bowel and bladder dysfunction and 5 patients with sensory disturbance below the level of syringomyelia, especially hypesthesia. Preoperative MRI showed conus medullaris disappeared at the end of spinal cord, and there was fluid signal in the lower spinal cord with hypo-intensity signal in T1WI and hyper-intensity signal in T2WI without enhancement. All patients underwent surgical procedures. Under microscope, filum terminale was cut off, drainage was performed, meningeal cyst within the sacral canal was removed, and tethered cord was released. The success rate of operations was 100%. The duration of surgery ranged from 1.52 to 3.07 h (average 2.15 h, with average intraoperative blood loss 220 ml (ranged from 100 to 410 ml. The tethering filum had been totally resected and histological examination showed typical filum tissue in all cases. No operative complication was found. Visual Analogue Scale (VAS score was decreased, and the lower limbs weakness as well as bowel and bladder dysfunction was gradually relieved after operation. The period of follow-up was ranged from 6

  2. Tethered Cord Syndrome Secondary to the Unusual Constellation of a Split Cord Malformation, Lumbar Myelomeningocele, and Coexisting Neurenteric Cyst

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    Humphrey Okechi

    2012-01-01

    Full Text Available We describe a seminal case report of a child with a tethered cord syndrome secondary to the unusual constellation of a split cord malformation, lumbar myelomeningocele, and coexisting neurenteric cyst. A 17-year-old adolescent girl with a several-month history of myelopathy and urinary incontinence was examined whose spinal MRI scan demonstrated a type II split cord malformation with a large bone spur and an intradural neurenteric cyst in addition to lumbar myelomeningocele. Untethering of the spinal cord was achieved via a lumbar laminectomy. Pathological examination confirmed the intradural cyst to be a neurenteric cyst. Postoperatively there was stabilization of the neurological symptoms. Prophylactic surgery with total resection of the neurenteric cyst when feasible and spinal cord un-tethering appears to be associated with excellent outcomes.

  3. Clinical and MR findings of tethered cord syndrome

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    Chung, Ho In; Kim, Hyae Young; Chung, Eun Chul; Suh, Jeong Soo [Ewha Womans University College of Medicine, Seoul (Korea, Republic of); Lim, Hyo Keun [Hallym University College of Medicine, Seoul (Korea, Republic of); Lee, Seoung Ro [Hanyang University College of Medicine, Seoul (Korea, Republic of); Lee, Young Seok [Chung Ang Gil Hospital, Incheon (Korea, Republic of)

    1994-09-15

    Tethered cord syndrome(TCS)is defined as low position of the conus medullaris by the abnormally fixed spinal cord with progressive neurologic deficit. To evaluate the findings of TCS at MRI and its diagnostic value, we performed a retrospective analysis of MRI of 30 patients with emphasis on clinical manifestation, level of conus medullaris, cause of tethering, and associated findings. Clinical presentation included back mass(26 cases), neurogenic bladder(5 cases), urinary incontinences(5 cases), progressive constipation(2 cases), skin dimpling(1 case), gait disturbance(1 case) and club foot (1 case). Neurologic deficit was developed 11 cases(40%) and mean age of these patients at the time of diagnosis was 8.6 years. The most common cause of tethering was lipoma(63%). The tips of conus medullaris were below the level of the second lumbar spine ia all patients. The causes of tethering were lipomatous components(spinal lipoma and lipomyelomenigocele) in 67% myelomeningocele in 20%, presacral mass in 7%, thickened filum terminale in 3% and postoperative change in 3%. Associated anomalies included syringomyelia(20%) and hydrocephalus was associated in 3 out 5 patients who underwent brain MRI. MRI clearly delineated the location of conus, tethering of the filum terminate with their causes and associated abnormalities. MRI examination is a very useful diagnostic tool for the early evaluation of TCS and the postoperative follow up.

  4. Spinal cord atrophy in triple A syndrome associated with a novel compound heterozygous mutation.

    Science.gov (United States)

    Kunte, Hagen; Trendelenburg, George; Matzen, Julia; Ventz, Manfred; Kornak, Uwe; Harms, Lutz

    2010-01-01

    A 38-year-old male patient was admitted with slowly progressive spastic gait disturbance. Imaging revealed general spinal cord atrophy. Because of adrenal insufficiency, alacrima and achalasia, triple A syndrome was suspected. This is a case report of a triple A syndrome patient with a predominance of neurological features and a new heterozygous compound mutation in triple A syndrome gene.

  5. Toxoplasmosis of Spinal Cord in Acquired Immunodeficiency Syndrome Patient Presenting as Paraparesis: A Rare Entity

    OpenAIRE

    2014-01-01

    Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count

  6. Intramedullary spinal cord metastasis from colonic carcinoma presenting as Brown-Sequard syndrome: a case report

    LENUS (Irish Health Repository)

    Kaballo, Mohammed A

    2011-08-02

    Abstract Introduction Intramedullary spinal cord metastasis is very rare. The majority are discovered incidentally during autopsy. Most symptomatic patients present with rapidly progressive neurological deficits and require immediate examination. Few patients demonstrate features of Brown-Séquard syndrome. Radiotherapy is the gold-standard of therapy for Intramedullary spinal cord metastasis. The overall prognosis is poor and the mortality rate is very high. We present what is, to the best of our knowledge, the first case of Intramedullary spinal cord metastasis of colorectal carcinoma presenting as Brown-Séquard syndrome. Case presentation We present the case of a 71-year-old Caucasian man with colonic adenocarcinoma who developed Intramedullary spinal cord metastasis and showed features of Brown-Séquard syndrome, which is an uncommon presentation of Intramedullary spinal cord metastasis. Conclusion This patient had an Intramedullary spinal cord metastasis, a rare form of metastatic disease, secondary to colonic carcinoma. The metastasis manifested clinically as Brown-Séquard syndrome, itself a very uncommon condition. This syndrome is rarely caused by intramedullary tumors. This unique case has particular interest in medicine, especially for the specialties of medical, surgical and radiation oncology. We hope that it will add more information to the literature about these entities.

  7. Loss of Central Inhibition: Implications for Behavioral Hypersensitivity after Contusive Spinal Cord Injury in Rats

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    Yerko A. Berrocal

    2014-01-01

    Full Text Available Behavioral hypersensitivity is common following spinal cord injury (SCI, producing significant discomfort and often developing into chronic pain syndromes. While the mechanisms underlying the development of behavioral hypersensitivity after SCI are poorly understood, previous studies of SCI contusion have shown an increase in amino acids, namely, aspartate and glutamate, along with a decrease in GABA and glycine, particularly below the injury. The current study sought to identify alterations in key enzymes and receptors involved in mediating central inhibition via GABA and glycine after a clinically-relevant contusion SCI model. Following thoracic (T8 25.0 mm NYU contusion SCI in rodents, significant and persistent behavioral hypersensitivity developed as evidenced by cutaneous allodynia and thermal hyperalgesia. Biochemical analyses confirmed upregulation of glutamate receptor GluR3 with downregulation of the GABA synthesizing enzyme (GAD65/67 and the glycine receptor α3 (GLRA3, notably below the injury. Combined, these changes result in the disinhibition of excitatory impulses and contribute to behavioral hyperexcitability. This study demonstrates a loss of central inhibition and the development of behavioral hypersensitivity in a contusive SCI paradigm. Future use of this model will permit the evaluation of different antinociceptive strategies and help in the elucidation of new targets for the treatment of neuropathic pain.

  8. Loss of central inhibition: implications for behavioral hypersensitivity after contusive spinal cord injury in rats.

    Science.gov (United States)

    Berrocal, Yerko A; Almeida, Vania W; Puentes, Rocio; Knott, Eric P; Hechtman, Jaclyn F; Garland, Mary; Pearse, Damien D

    2014-01-01

    Behavioral hypersensitivity is common following spinal cord injury (SCI), producing significant discomfort and often developing into chronic pain syndromes. While the mechanisms underlying the development of behavioral hypersensitivity after SCI are poorly understood, previous studies of SCI contusion have shown an increase in amino acids, namely, aspartate and glutamate, along with a decrease in GABA and glycine, particularly below the injury. The current study sought to identify alterations in key enzymes and receptors involved in mediating central inhibition via GABA and glycine after a clinically-relevant contusion SCI model. Following thoracic (T8) 25.0 mm NYU contusion SCI in rodents, significant and persistent behavioral hypersensitivity developed as evidenced by cutaneous allodynia and thermal hyperalgesia. Biochemical analyses confirmed upregulation of glutamate receptor GluR3 with downregulation of the GABA synthesizing enzyme (GAD65/67) and the glycine receptor α3 (GLRA3), notably below the injury. Combined, these changes result in the disinhibition of excitatory impulses and contribute to behavioral hyperexcitability. This study demonstrates a loss of central inhibition and the development of behavioral hypersensitivity in a contusive SCI paradigm. Future use of this model will permit the evaluation of different antinociceptive strategies and help in the elucidation of new targets for the treatment of neuropathic pain.

  9. Reaction to topical capsaicin in spinal cord injury patients with and without central pain

    DEFF Research Database (Denmark)

    Finnerup, Nanna Brix; Pedersen, Louise H.; Terkelsen, Astrid J.

    2007-01-01

    Central neuropathic pain is a debilitating and frequent complication to spinal cord injury (SCI). Excitatory input from hyperexcitable cells around the injured grey matter zone is suggested to play a role for central neuropathic pain felt below the level of a spinal cord injury. Direct evidence...... of a spinal cord injury which already is hyperexcitable, would cause enhanced responses in patients with central pain at the level of injury compared to patients without neuropathic pain and healthy controls. Touch, punctuate stimuli, cold stimuli and topical capsaicin was applied above, at, and below injury...... level in 10 SCI patients with central pain below a thoracic injury, in 10 SCI patients with a thoracic injury but without neuropathic pain, and in corresponding areas in 10 healthy control subjects. The study found increased responses to touch at injury level compared to controls (p = 0...

  10. Apoptosis of lumbar spinal cord neurons in cauda equina syndrome rats

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    Objective To explore the law of apoptosis of lumbar spinal cord neurons in cauda equina syndrome (CES). Methods Cauda equina of rats was compressed by a piece of silica gel stick. From day 1 to day 28,the lumbar spinal cord specimens were harvested and assessed by Nissl's staining and TUNEL staining. Results Compression of cauda equina caused lesion and apoptosis of neurons in lumbar spinal cord,and the extent of apoptosis reached the peak on 7th day after compression. Conclusion Apoptosis of neurons in lum...

  11. Metabolic syndrome and central retinal artery occlusion

    Directory of Open Access Journals (Sweden)

    Kosanović-Jaković Natalija

    2005-01-01

    Full Text Available Background. The accumulation of risk factors for central retinal artery occlusion can be seen in a single person and might be explained by the metabolic syndrome. Case report. We presented the case of a 52-year-old man with no light perception in his right eye. The visual loss was monocular and painless, fundoscopy showed central retinal artery occlusion and the laboratory investigation showed the raised erythrocyte sedimentation rate of 105 mm/h and the raised C-reactive protein of 22 mg/l. Specific laboratory investigations and fluorescein angiography excluded the presence of vasculitis, collagen vascular diseases, hypercoagulable state and antiphospholipid syndrome. Conclusion. The patient met all the five of the National Cholesterol Education Program (NCEP criteria for the metabolic syndrome: hypertension, abnormal lipid profile, abnormal glucose metabolism, obesity and hyperuricemia. Measurement of C-reactive protein is useful for the assessment of therapeutic systemic effect on any abnormality in the metabolic syndrome. Individual therapy for all risk factors in the metabolic syndrome is necessary to prevent complications such as cardiovascular, retinal vascular diseases and stroke.

  12. Sheehan's syndrome with central diabetes insipidus.

    Science.gov (United States)

    Laway, Bashir Ahmad; Mir, Shahnaz Ahmad; Dar, Mohd Iqbal; Zargar, Abdul Hamid

    2011-03-01

    Sheehan's syndrome refers to the occurrence of hypopituitarism after delivery, usually preceded by postpartum hemorrhage. The condition still continues to be a common cause of hypopituitarism in developing countries like India. The disorder usually presents with anterior pituitary failure with preservation of posterior pituitary functions. Posterior pituitary dysfunction in the form of central diabetes insipidus is rare in patients with Sheehan's syndrome. We describe the clinical course of a young lady who after her sixth childbirth developed severe postpartum hemorrhage followed by development of panhypopituitarism which was confirmed by hormonal investigation and demonstration of empty sella on imaging. In addition, she developed Polyuria. The water deprivation test and response to vasopressin test results indicated central diabetes insipidus. She needed oral desmopressin on a continuous basis to control polyuria.

  13. Postpartum spinal cord injury in a woman with HELLP syndrome.

    NARCIS (Netherlands)

    Groothuis, J.T.; Kuppevelt, DH van

    2008-01-01

    OBJECTIVE: To report a rare cause of spinal cord injury. STUDY DESIGN: Case report. CASE REPORT: A 36-year-old woman presented with acute onset of paresis of the upper and lower extremity (level C5, ASIA B) the day after delivering a healthy daughter (39 weeks' gestation). Prior to giving birth, she

  14. Serotonin syndrome caused by minimum doses of SSRIS in a patient with spinal cord injury

    OpenAIRE

    Satoh, Koichiro; Takano, Shizuko; Onogi, Takashi; Ohtsuki, Koji; Kobayashi, Toshio

    2006-01-01

    There have been only a few reports of serotonin syndrome developing after mono-therapy with a selective serotonin reuptake inhibitor (SSRI). We report a case of serotonin syndrome caused by long-term therapy with fluvoxamine prior to treatment with paroxetine. An 18-year-old man with spinal cord injury (SCI) at thoracic level 2-3 presented with onset of serotonin syndrome after taking fluvoxamine (50 mg per day) for 8 weeks prior to treatment with paroxetine (10 mg per day) for 6 days. He had...

  15. A Review of Select Centralized Pain Syndromes

    Directory of Open Access Journals (Sweden)

    David R. Spiegel

    2015-01-01

    Full Text Available Pain can be broadly divided into 3 classes, including nociceptive or inflammatory pain (protective, neuropathic (pathological, occurring after damage to the nervous system, or centralized (pathological, due to abnormal function but with no damage or inflammation to the nervous system. The latter has been posited to occur when descending analgesic pathways are attenuated and/or glutamatergic transmission is facilitated. Additionally, this “pain prone phenotype” can be associated with early life trauma and a suboptimal response to opiates. This article will review the relationships between centralized pain syndromes (ie, fibromyalgia, chronic low back pain, childhood sexual abuse, and opiate misuse. Finally, treatment implications, potentially effecting primary care physicians, will be discussed.

  16. Central Pain Following Cord Severance for Cephalosomatic Anastomosis.

    Science.gov (United States)

    Canavero, Sergio; Bonicalzi, Vincenzo

    2016-04-01

    One of the key obstacles to a successful head transplant is the possible onset of central pain, a chronic pain condition that would impair the quality of life of the transplantee. In this review, we provide the reader with a knowledge of this neglected aspect of the head transplant initiative and outline the management should this eventuality occur.

  17. The adult macaque spinal cord central canal zone contains proliferative cells and closely resembles the human.

    Science.gov (United States)

    Alfaro-Cervello, Clara; Cebrian-Silla, Arantxa; Soriano-Navarro, Mario; Garcia-Tarraga, Patricia; Matías-Guiu, Jorge; Gomez-Pinedo, Ulises; Molina Aguilar, Pilar; Alvarez-Buylla, Arturo; Luquin, Maria-Rosario; Garcia-Verdugo, Jose Manuel

    2014-06-01

    The persistence of proliferative cells, which could correspond to progenitor populations or potential cells of origin for tumors, has been extensively studied in the adult mammalian forebrain, including human and nonhuman primates. Proliferating cells have been found along the entire ventricular system, including around the central canal, of rodents, but little is known about the primate spinal cord. Here we describe the central canal cellular composition of the Old World primate Macaca fascicularis via scanning and transmission electron microscopy and immunohistochemistry and identify central canal proliferating cells with Ki67 and newly generated cells with bromodeoxyuridine incorporation 3 months after the injection. The central canal is composed of uniciliated, biciliated, and multiciliated ependymal cells, astrocytes, and neurons. Multiciliated ependymal cells show morphological characteristics similar to multiciliated ependymal cells from the lateral ventricles, and uniciliated and biciliated ependymal cells display cilia with large, star-shaped basal bodies, similar to the Ecc cells described for the rodent central canal. Here we show that ependymal cells with one or two cilia, but not multiciliated ependymal cells, proliferate and give rise to new ependymal cells that presumably remain in the macaque central canal. We found that the infant and adult human spinal cord contains ependymal cell types that resemble those present in the macaque. Interestingly, a wide hypocellular layer formed by bundles of intermediate filaments surrounded the central canal both in the monkey and in the human, being more prominent in the stenosed adult human central canal.

  18. The spinal cord ependymal region: a stem cell niche in the caudal central nervous system.

    Science.gov (United States)

    Hugnot, Jean Philippe; Franzen, Rachelle

    2011-01-01

    In the brain, specific signalling pathways localized in highly organized regions called niches, allow the persistence of a pool of stem and progenitor cells that generate new neurons and glial cells in adulthood. Much less is known on the spinal cord central canal niche where a sustained adult neurogenesis is not observed. Here we review our current knowledge of this caudal niche in normal and pathological situations. Far from being a simple layer of homogenous cells, this region is composed of several cell types localized at specific locations, expressing characteristic markers and with different morphologies and functions. We further report on a screen of online gene-expression databases to better define this spinal cord niche. Several genes were found to be preferentially expressed within or around the central canal region (Bmp6, CXCR4, Gdf10, Fzd3, Mdk, Nrtn, Rbp1, Shh, Sox4, Wnt7a) some of which by specific cellular subtypes. In depth characterization of the spinal cord niche constitutes a framework to make the most out of this endogenous cell pool in spinal cord disorders.

  19. Skin vasomotor hemiparesis followed by overactivity: characteristic thermography findings in a patient with Horner syndrome due to spinal cord infarction.

    Science.gov (United States)

    Kobayashi, Makoto

    2016-04-01

    We present a 21-year-old female with Horner syndrome due to spinal cord infarction. In this patient, infrared thermography revealed a hemibody skin temperature increase followed by excessive focal decreases, indicating skin vasomotor hemiparesis and overactivity.

  20. Toxoplasmosis of spinal cord in acquired immunodeficiency syndrome patient presenting as paraparesis: A rare entity

    Directory of Open Access Journals (Sweden)

    Sachin R Agrawal

    2014-01-01

    Full Text Available Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count <100/mm 3 based on a positive serum Toxoplasma gondii IgG antibodies, no recent prophylaxis against toxoplasmosis, intramedullary ring enhancing lesion in spinal cord supported by similar lesions in brain parenchyma. Institutions of antitoxoplasma treatment in such patients result in prompt clinical response and therefore avoiding the need of unnecessary invasive diagnostic tests. Here, we report a case of toxoplasmic myelitis in immunocompromised patient presenting as myelopathy who showed significant clinical improvement after starting antitoxoplasma treatment. Hence toxoplasmic myelitis should be considered in toxoplasma seropositive immunocompromised patients presenting as myelopathy and imaging studies showing ring enhancing intramedullary lesion.

  1. Obstructive sleep apnea syndrome and snoring in patients with bilateral vocal cord paralysis.

    Science.gov (United States)

    Misiolek, Maciej; Namyslowski, Grzegorz; Karpe, Jacek; Ziora, Dariusz; Misiolek, Hanna; Czecior, Eugeniusz; Scierski, Wojciech

    2003-04-01

    The analysis of the correlation between bilateral vocal cord paralysis and the occurrence of obstructive sleep apnea syndrome and snoring is presented. The aim of the study was to establish whether the decrease of the air flow in the upper airway in patients with bilateral vocal cord paralysis involves OSAS and/or snoring occurrence and whether arytenoidectomy affects an improvement of breathing parameters measured during sleep. Fourteen patients with bilateral vocal cord paralysis underwent Poly-MESAM examination before and 3 months after arytenoidectomy. They had never complained of snoring before. The Epworth sleepiness scale was used to quantify excessive daytime somnolence. The RDI, DI, mean saturation and percentage of snoring, loud snoring and sleep without snoring were estimated and compared pre- and postoperatively. The results were compared by the Student's t-test for dependent values. No significant differences were shown between the Epworth scores before and after the treatment. The RDI, DI and mean saturation were normal before and after the operation. The percentage of loud snoring decreased and the percentage of sleep without snoring increased significantly after arytenoidectomy in both cases. The mechanism of snoring in patients with vocal cord paralysis seems to be similar to OSAS. The difference consists in the level of flow limitation. On the basis of the results there is no reason to diagnose OSAS and UARS in patients with bilateral vocal cord paralysis. On the other hand, the intensive snoring that occurs after paralysis was significantly reduced as a result of arytenoidectomy.

  2. Spinal cord central canal of the German shepherd dog: morphological, histological, and ultrastructural considerations.

    Science.gov (United States)

    Marín-García, P; González-Soriano, J; Martinez-Sainz, P; Contreras-Rodríguez, J; Del Corral-Gros, C; Rodríguez-Veiga, E

    1995-05-01

    This study deals with some macroscopical, microscopical, and ultrastructural aspects of the spinal cord central canal of the German shepherd dog. The caudal end of the spinal cord is constituted by the conus medullaris, which may extend to the first sacral vertebra, the terminal ventricle, and the filum terminale. The latter structure is considered as internum (second to third sacral vertebrae) or externum (fifth caudal vertebra), according to its relation to the dura mater. Occasionally, there is a second anchorage which is close to the level of the sixth caudal vertebra. The central canal is surrounded by a ciliated ependymal epithelium, which differs depending upon the levels. The most caudal part of the filum terminale bears a columnar ciliated ependymal epithelium surrounded by two layers of glia and pia mater, which separate the central canal from the subarachnoid space. Microfil injections show a communication between the cavity and the subarachnoid space, as the plastic is able to pass through the ependymal epithelium. At the level of the terminal ventricle there are real separations of the ependymal epithelium, which seem to connect the lumen of the spinal canal with the subarachnoid space. These structures probably constitute one of the drainage pathways of the cerebrospinal fluid. The diameter of the central canal is related to the age of the animal. However, even in very old animals the spinal cord central canal reaches the tip of the filum terminale and remains patent until death. At the ultrastructural level the ependymal cells present villi, located on cytoplasmic projections, cilia, dense mitochondria, and oval nuclei.

  3. Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome: a case report

    Science.gov (United States)

    Lo, Tony Chung Tung; Yeung, Stephen Tung; Lee, Sujin; Skavinski, Kira; Liao, Solomon

    2016-01-01

    Objective Ehlers–Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers–Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative. Case report A 27-year-old Caucasian female with a history of Ehlers–Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7–8 out of 10 to 0–3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs. Conclusion Ketamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of physiological balance between pain inhibition and facilitation. The present report shows that this treatment option can be used in patients with refractory central pain syndrome in the setting of spinal cord myelopathy secondary to Ehlers–Danlos syndrome. In addition, as seen in this case, this protocol can potentially decrease the chronic use of pain medication, such as opioids.

  4. Management of adult tethered cord syndrome: Our experience and review of literature

    Directory of Open Access Journals (Sweden)

    Kanwaljeet Garg

    2014-01-01

    Full Text Available Background: Tethered cord syndrome (TCS is a complex clinicopathologic entity, mostly described in children with limited number of studies describing in adults. This unique and rare subgroup of patients presents with characteristic features of TCS, but unlike children, pain is a predominant clinical symptom. Materials and Methods: Case records of 24 patients aged ≥16 years who had undergone surgery with a diagnosis of TCS between 2001 and 2011 were reviewed. Patients who have underwent surgery earlier for tethered cord or for diastematomyelia/spinal dysraphism and patients who had radiological evidence of tethering elements like lipoma of the cord on magnetic resonance imaging (MRI were excluded from the study. Results: Low backache was the most common presenting symptom. At the time of final follow-up, 15 (83.3% patients had shown improvement in backache. Weakness improved by at least one grade in seven (77.8% patients. Bladder symptoms improved in six (50% patients. Conclusion: In case of symptomatic patient with low-lying cord, detethering is an advisable option.

  5. Solitary median maxillary central incisor (SMMCI syndrome

    Directory of Open Access Journals (Sweden)

    Hall Roger K

    2006-04-01

    Full Text Available Abstract Solitary median maxillary central incisor syndrome (SMMCI is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factor(s operating in utero about the 35th–38th day(s from conception. It is estimated to occur in 1:50,000 live births. Aetiology is uncertain. Missense mutation in the SHH gene (I111F at 7q36 may be associated with SMMCI. The SMMCI tooth differs from the normal central incisor, in that the crown form is symmetric; it develops and erupts precisely in the midline of the maxillary dental arch in both primary and permanent dentitions. Congenital nasal malformation (choanal atresia, midnasal stenosis or congenital pyriform aperture stenosis is positively associated with SMMCI. The presence of an SMMCI tooth can predict associated anomalies and in particular the serious anomaly holoprosencephaly. Common congenital anomalies associated with SMMCI are: severe to mild intellectual disability, congenital heart disease, cleft lip and/or palate and less frequently, microcephaly, hypopituitarism, hypotelorism, convergent strabismus, oesophageal and duodenal atresia, cervical hemivertebrae, cervical dermoid, hypothyroidism, scoliosis, absent kidney, micropenis and ambiguous genitalia. Short stature is present in half the children. Diagnosis should be made by eight months of age, but can be made at birth and even prenatally at 18–22 weeks from the routine mid-trimester ultrasound scan. Management depends upon the individual anomalies present. Choanal stenosis requires emergency surgical treatment. Short stature may require growth hormone therapy. SMMCI tooth itself is mainly an aesthetic problem, which is ideally managed by combined orthodontic, prosthodontic and oral surgical treatment; alternatively, it can be left untreated.

  6. Umbilical cord blood and neonatal endothelin-1 levels in preterm newborns with and without respiratory distress syndrome

    OpenAIRE

    2005-01-01

    Increased pulmonary vascular resistance in preterm newborn infants with respiratory distress syndrome is suggested, and endothelin-1 plays an important role in pulmonary vascular reactivity in newborns. We determined umbilical cord blood and neonatal (second sample) levels of endothelin-1 in 18 preterm newborns with respiratory distress syndrome who had no clinical or echocardiographic diagnosis of pulmonary hypertension and 22 without respiratory distress syndrome (gestational ages: 31.4 ± 1...

  7. Spinal cord stimulation to abort painful spasms of atypical stiff limb syndrome.

    Science.gov (United States)

    Ughratdar, I; Sivakumar, G; Basu, S

    2010-01-01

    Stiff limb syndrome (SLS) is a rare chronic condition which can result in significant debility. We report the case of a 44-year-old man suffering from severe painful spasms in his right leg with a diagnosis of SLS. He had been initially treated for his pain with a spinal cord stimulator but presented with exacerbation of pain secondary to a lead fracture for which he underwent revision of the stimulator. Postoperative programming unexpectedly resulted in not only control of his pain but also an ability to abort his spasmodic episodes related to SLS. To our knowledge, spinal cord stimulation has not been previously used for SLS and our report opens up another avenue for this rare condition. We provide a brief overview of SLS and propose an underlying mechanism for the observed phenomenon.

  8. Monoaminergic and catecholaminergic activation of the central pattern generator for locomotion following spinal cord injury Innovative therapeutic approaches

    Institute of Scientific and Technical Information of China (English)

    Pierre A.Guertin

    2009-01-01

    The development of secondary health complications following spinal cord injury has been increasingly recognized by healthcare professionals as a major concern.These problems most specifically affect complete or near-complete spinal cord injury patients (e.g.,those with minimal mobility),who are not typically rehabilitated with treadmill training approaches,because motor control and leg movements are largely impaired.However,recent pharmaceutical advances in central pattern generator activation may provide new therapeutic hopes for these spinal cord injury patients.This article provides a comprehensive overview,for the non-specialist,of the most recent advances in this field.

  9. Diagnosis and management of traumatic cervical central spinal cord injury: A review

    Directory of Open Access Journals (Sweden)

    Nancy E Epstein

    2015-01-01

    Full Text Available Background: The classical clinical presentation, neuroradiographic features, and conservative vs. surgical management of traumatic cervical central spinal cord (CSS injury remain controversial. Methods: CSS injuries, occurring in approximately 9.2% of all cord injuries, are usually attributed to significant hyperextension trauma combined with congenital/acquired cervical stenosis/spondylosis. Patients typically present with greater motor deficits in the upper vs. lower extremities accompanied by patchy sensory loss. T2-weighted magnetic resonance (MR scans usually show hyperintense T2 intramedullary signals reflecting acute edema along with ligamentous injury, while noncontrast computed tomography (CT studies typically show no attendant bony pathology (e.g. no fracture, dislocation. Results: CSS constitute only a small percentage of all traumatic spinal cord injuries. Aarabi et al. found CSS patients averaged 58.3 years of age, 83% were male and 52.4% involved accidents/falls in patients with narrowed spinal canals (average 5.6 mm; their average American Spinal Injury Association (ASIA motor score was 63.8, and most pathology was at the C3-C4 and C4-C5 levels (71%. Surgery was performed within 24 h (9 patients, 24-48 h (10 patients, or after 48 h (23 patients. In the Brodell et al. study of 16,134 patients with CSS, 39.7% had surgery. In the Gu et al. series, those with CSS and stenosis/ossification of the posterior longitudinal ligament (OPLL exhibited better outcomes following laminoplasty. Conclusions: Recognizing the unique features of CSS is critical, as the clinical, neuroradiological, and management strategies (e.g. conservative vs. surgical management: early vs. late differ from those utilized for other spinal cord trauma. Increased T2-weighted MR images best document CSS, while CT studies confirm the absence of fracture/dislocation.

  10. Klippel-Feil syndrome – the risk of cervical spinal cord injury: A case report

    Directory of Open Access Journals (Sweden)

    Singh Gurpreet

    2002-04-01

    Full Text Available Abstract Background Klippel-Feil syndrome is defined as congenital fusion of two or more cervical vertebrae and is believed to result from faulty segmentation along the embryo's developing axis during weeks 3–8 of gestation. Persons with Klippel-Feil syndrome and cervical stenosis may be at increased risk for spinal cord injury after minor trauma as a result of hypermobility of the various cervical segments. Persons with Klippel-Feil Syndrome often have congenital anomalies of the urinary tract as well. Case presentation A 51-year male developed incomplete tetraplegia in 1997 when he slipped and fell backwards hitting his head on the floor. X-rays of cervical spine showed fusion at two levels: C2 and C3 vertebrae, and C4 and C5 vertebrae. Intravenous urography (IVU revealed no kidneys in the renal fossa on both sides, but the presence of crossed, fused renal ectopia in the left ilio-lumbar region. This patient had a similar cervical spinal cord injury about 15 years ago, when he developed transient numbness and paresis of the lower limbs following a fall. Discussion and Conclusion 1 Persons with Klippel-Feil syndrome should be made aware of the increased risk of sustaining transient neurologic deterioration after minor trauma if there is associated radiographic evidence of spinal stenosis. 2 Patients with Klippel-Feil syndrome often have congenital anomalies of the urinary tract. Our patient had crossed, fused, ectopia of kidney. 3 When patients with Klippel-Feil syndrome sustain tetraplegia they have increased chances of developing urinary tract calculi. Treatment of kidney stones may pose a challenge because of associated renal anomalies. 4 Health professionals caring for cervical spinal cord injury patients with Klippel-Feil syndrome and renal anomalies should place emphasis on prevention of kidney stones. A large fluid intake is recommended for these patients, as a high intake of fluids is still the most powerful and certainly the most

  11. [The trigemino-cervical complex. Integration of peripheral and central pain mechanisms in primary headache syndromes].

    Science.gov (United States)

    Busch, V; Frese, A; Bartsch, T

    2004-10-01

    The activation of the trigeminal nociceptive system is the neural substrate of pain in primary headache syndromes such as migraine and cluster headache. The nociceptive inflow from the meninges to the spinal cord is relayed in brainstem neurones of the trigemino-cervical complex (TCC). Two important mechanisms of pain transmission are reviewed: convergence of nociceptive trigeminal and cervical afferents and sensitization of trigemino-cervical neurones. These mechanisms have clinical correlates such as hyperalgesia, allodynia, spread and referral of pain to trigeminal or cervical dermatomes. Neurones in the TCC are subject to a modulation of pain-modulatory circuits in the brainstem such as the periaqueductal grey (PAG). Recent experimental and clinical findings of a modulation of these pain processes are discussed. The review focuses on TCC neurones as integrative relay neurones between peripheral and central pain mechanisms. The understanding of these mechanisms has implications for the understanding of the clinical phenomenology in primary headache syndromes and the development of therapeutical options.

  12. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    Science.gov (United States)

    ... Sheets Vasculitis Syndromes of the Central and Peripheral Nervous Systems Fact Sheet Table of Contents (click to jump ... flow of blood. How does vasculitis affect the nervous system? Vasculitis can cause problems in any organ system, ...

  13. Central and peripheral actions of the NSAID ketoprofen on spinal cord nociceptive reflexes.

    Science.gov (United States)

    Herrero, J F; Parrado, A; Cervero, F

    1997-10-01

    Ketoprofen is a non-steroidal antiinflammatory drug (NSAID) which provides effective analgesia in situations of pain provoked by tissue inflammation. However, the location of its analgesic effects, (peripheral tissues versus central nervous system), have not been clearly identified and separated. In the present study the effectiveness of ketoprofen was examined in two different types of experiments: (i) Open field behavioural tests in conscious rats, and (ii) spinal cord nociceptive reflexes (single motor units) activated by electrical and thermal stimulation in chloralose anaesthetised rats. The experiments were performed in rats with carrageenan-induced inflammation of one hindpaw, or of one knee joint. The administration of ketoprofen significantly inhibited the reduction of exploratory movements caused by inflammation in open field experiments. Ketoprofen was also effective in depressing reflex activity evoked by electrical and noxious thermal stimulation of the skin, either in inflamed tissue or in normal tissue of monoarthritic animals. It was also effective in the reduction of reflex wind-up; a phenomenon in which the activity of spinal cord neurones increases progressively with high frequency electrical stimulation. We therefore conclude that ketoprofen has central as well as peripheral analgesic activity.

  14. Cerebral Venous Thrombosis in a Patient with Clinically Isolated Spinal Cord Syndrome

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    Jasem Yousef Al-Hashel

    2013-01-01

    Full Text Available Background. The association between cerebral venous thrombosis (CVT and multiple sclerosis (MS has already been reported in patients with clinically definite MS in relation to intravenous methylprednisolone (IVMP or previously performed lumbar puncture (LP. Case Summery. We report a 29-year-old Indian female who presented with a clinically isolated spinal cord syndrome according to the revised 2010 McDonald Criteria. She developed CVT after a lumbar puncture and two days of finishing the course of IVMP. Conclusion. We conclude that the sequence of doing lumbar puncture followed by high-dose IVMP may increase the risk of CVT. A prophylactic anticoagulation may be indicated in this setting.

  15. Umbilical cord blood and neonatal endothelin-1 levels in preterm newborns with and without respiratory distress syndrome.

    Science.gov (United States)

    Benjamin, A C W; Silveira, R C; Procianoy, R S

    2005-09-01

    Increased pulmonary vascular resistance in preterm newborn infants with respiratory distress syndrome is suggested, and endothelin-1 plays an important role in pulmonary vascular reactivity in newborns. We determined umbilical cord blood and neonatal (second sample) levels of endothelin-1 in 18 preterm newborns with respiratory distress syndrome who had no clinical or echocardiographic diagnosis of pulmonary hypertension and 22 without respiratory distress syndrome (gestational ages: 31.4 +/- 1.6 and 29.3 +/- 2.3 weeks, respectively). Umbilical cord blood and a second blood sample taken 18 to 40 h after birth were used for endothelin-1 determination by enzyme immunoassay. Median umbilical cord blood endothelin-1 levels were similar in both groups (control: 10.9 and respiratory distress syndrome: 11.4 pg/mL) and were significantly higher than in the second sample (control: 1.7 pg/mL and respiratory distress syndrome: 3.5 pg/mL, P respiratory distress syndrome than in control infants (P newborns with respiratory distress syndrome when compared to controls. A significant correlation between neonatal endothelin-1 levels and some illness-severity signs suggests that endothelin-1 plays a role in the natural course of respiratory distress syndrome in preterm newborns.

  16. Protein phosphatase 2A regulates central sensitization in the spinal cord of rats following intradermal injection of capsaicin

    Directory of Open Access Journals (Sweden)

    Fang Li

    2006-03-01

    Full Text Available Abstract Background Intradermal injection of capsaicin into the hind paw of rats induces spinal cord central sensititzation, a process in which the responsiveness of central nociceptive neurons is amplified. In central sensitization, many signal transduction pathways composed of several cascades of intracellular enzymes are involved. As the phosphorylation state of neuronal proteins is strictly controlled and balanced by the opposing activities of protein kinases and phosphatases, the involvement of phosphatases in these events needs to be investigated. This study is designed to determine the influence of serine/threonine protein phosphatase type 2A (PP2A on the central nociceptive amplification process, which is induced by intradermal injection of capsaicin in rats. Results In experiment 1, the expression of PP2A protein in rat spinal cord at different time points following capsaicin or vehicle injection was examined using the Western blot method. In experiment 2, an inhibitor of PP2A (okadaic acid, 20 nM or fostriecin, 30 nM was injected into the subarachnoid space of the spinal cord, and the spontaneous exploratory activity of the rats before and after capsaicin injection was recorded with an automated photobeam activity system. The results showed that PP2A protein expression in the spinal cord was significantly upregulated following intradermal injection of capsaicin in rats. Capsaicin injection caused a significant decrease in exploratory activity of the rats. Thirty minutes after the injection, this decrease in activity had partly recovered. Infusion of a phosphatase inhibitor into the spinal cord intrathecal space enhanced the central sensitization induced by capsaicin by making the decrease in movement last longer. Conclusion These findings indicate that PP2A plays an important role in the cellular mechanisms of spinal cord central sensitization induced by intradermal injection of capsaicin in rats, which may have implications in

  17. [A case of Avellis' syndrome with ipsilateral central facial palsy due to a small medullary infarction].

    Science.gov (United States)

    Takahashi, K; Kitani, M; Fukuda, H

    2000-04-01

    We report a 51-year-old man with mild left central facial palsy and left Avellis' syndrome due to a small medullary infarction. On admission, neurological examination revealed hoarseness, dysphasia, absent left gag reflex, palsies of the left vocal cord and left soft palate, and hypalgesia and thermohypesthesia on the right side of the trunk and extremities. In addition, he had a mild left central facial palsy. He had no nausea, vomiting, vertigo, hiccups, nystagmus, Horner's sign, facial numbness, or paresis or ataxia of the limbs. A T2 weighted MRI showed a small, high signal intensity area in the left dorsal region of the medulla and this lesion was presumed to involve the nucleus ambiguus and a part of the spinothalamic tract. These findings suggest that an aberrant supranuclear pathway, looping around the nucleus ambiguus to the facial nucleus exists in our patient.

  18. Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Lo TC

    2016-09-01

    Full Text Available Tony Chung Tung Lo,1,* Stephen Tung Yeung,2,* Sujin Lee,1 Kira Skavinski,3 Solomon Liao,4 1Department of Physical Medicine and Rehabilitation, University of California Irvine, Orange, CA, 2Department of Immunology, University of Connecticut School of Medicine, Farmington, CT, 3Department of Palliative Medicine, University of California San Diego, La Jolla, 4Department of Palliative Medicine, University of California Irvine, Orange, CA, USA *These authors contributed equally to this work Objective: Ehlers–Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers–Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative. Case report: A 27-year-old Caucasian female with a history of Ehlers–Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7–8 out of 10 to 0–3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs. Conclusion: Ketamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of

  19. Predicting chronic stinger syndrome using the mean subaxial space available for the cord index.

    Science.gov (United States)

    Greenberg, Jared; Leung, Dan; Kendall, Jenny

    2011-05-01

    A 21-year-old division I collegiate football player who had a history of several stingers presented with 5 days of persistent left neck and shoulder pain associated with paresthesias and upper extremity weakness. His symptoms began immediately during a game when he was struck on the right side of his helmet, which induced a compression-extension mechanism of injury to his neck. Clinical and electrodiagnostic evaluation was consistent with a left C5 radiculopathy, but magnetic resonance imaging of the cervical spine yielded normal results. The mean subaxial cervical space available for the cord (MSCSAC) index is a novel tool to predict chronic stinger syndrome. It is calculated by subtracting the sagittal diameter of the spinal cord from the disc-level sagittal diameter of the spinal canal at levels C3 through C6 and then averaging these values. A cutoff of < 4.3 mm has been shown to predict a greater-than-13-fold increase in risk of developing chronic stinger syndrome. This patient had a MSCSAC index of 3.2 mm, which correlated with his history of multiple stingers. The MSCSAC index may be a useful tool to help counsel athletes on the risk of developing future stingers, although more extensive research on this measurement tool is indicated.

  20. Fungal infections of the central nervous system: The clinical syndromes

    Directory of Open Access Journals (Sweden)

    Murthy J.M.K

    2007-01-01

    Full Text Available Fungal infections of the central nervous system (CNS are being increasingly diagnosed both in immunocompromised and immunocompetent individuals. Sinocranial aspergillosis is more frequently described from countries with temperate climates, more often in otherwise immunocompetent individuals. The clinical syndromes with which fungal infections of the CNS can present are protean and can involve most part of the neuroaxis. Certain clinical syndromes are specific for certain fungal infections. The rhinocerebral form is the most common presenting syndrome with zygomycosis and skull-base syndromes are often the presenting clinical syndromes in patients with sinocranial aspergillosis. Subacute and chronic meningitis in patients with HIV infection is more likely to be due to cryptococcal infection. Early recognition of the clinical syndromes in an appropriate clinical setting is the first step towards achieving total cure in some of these infections.

  1. [Effects and indications of spinal cord stimulation on the vegetative syndrome].

    Science.gov (United States)

    Funahashi, K; Komai, N; Ogura, M; Kuwata, T; Nakai, M; Tsuji, N

    1989-10-01

    The effects of spinal cord stimulation (SCS) on the vegetative syndrome were studied in six patients. Factors affecting the results were mentioned with a view to establishing indications as to whether or not the SCS should be performed. "Persistent vegetative states" were thought to be identical with Ohta's "vegetative syndrome" which consists of eleven signs. Six of these signs--polyphasic cycle of waking and sleeping, urinary incontinence, being bedridden and being tube fed etc--were important criteria of the vegetative syndrome. SCS was thought to be effective if one or more of the 6 signs disappeared after SCS. SCS was performed at level from C2 to C4 with a frequency of 25 to 120 Hz, an intensity of 2.5 to 6 volts, a pulse duration of 0.3 to 0.5 msec. and a duration of 3 to 11 hours per day. Neurological signs, ABR, CT/MRI, EEG and the grade of the vegetative syndrome were estimated before and after SCS. In the course of SCS, 2 of the 6 patients recovered from the vegetative syndrome. Both had a localized lesion in the brain stem without a cerebral lesion on CT/MRI, with bilateral appearance of the fifth peak with prolonged latency and decreased amplitude of main peaks on ABR. The other 4 patients showed little or no improvement. They all had diffuse cerebral atrophy or low density areas on CT and almost normal ABR. One of these patients, who suffered a cerebral contusion leading to transtentorial herniation with unilateral cerebral contusion on CT and unilateral disappearance of the fifth peak on ABR, showed no recovery from the vegetative syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)

  2. Impact of spinal cord stimulation on sensory characteristics in complex regional pain syndrome type I - A randomized trial

    NARCIS (Netherlands)

    Kemler, MA; Reulen, JPH; Barendse, GAM; van Kleef, M; de Vet, HCW; van den Wildenberg, FAJM

    2001-01-01

    Background: A randomized trial was performed to assess the effect of spinal cord stimulation (SCS) on detection and pain thresholds for pressure, warmth, and cold and on the extent of mechanical hyperalgesia in patients with chronic complex regional pain syndrome type I. Methods: Fifty-four chronic

  3. Sjogrens Syndrome Presenting with Central Nervous System Involvement

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    Tülay Terzi

    2012-01-01

    Full Text Available Sjogren’s syndrome is a slowly progressive autoimmune disease. Neurological involvement occurs in approximately 20-25% cases in Sjogren’s syndrome. 87% of the neurological involvement is peripheral nervous system, almost 13% in the form of central nervous system involvement. Affected central nervous system may show similar clinical and radiological findings as in multiple sclerosis (MS. In this paper, a 43-year-old patient is discussed who was referred with the complaint of dizziness, there was MS- like lesions in brain imaging studies and was diagnosed with Sjogren’s syndrome. MS- like clinical and radiologic tables can be seen, albeit rarely in Sjogren’s syndrome. In these cases, early diagnosis and early treatment for the sjögren has a great importance for the prognosis of the disease.

  4. Paraneoplastic Syndromes of the Central Nervous System

    NARCIS (Netherlands)

    J.W.B. Moll (Wibe)

    1996-01-01

    textabstractIn recent years a continuous stream of new information on clinical, pathological and immunological aspects of paraneoplastic neurological syndromes has been published. In this survey, we will discuss current opinions on the value of anti-neuronal antibody detection for establishing a dia

  5. GABAergic responses of mammalian ependymal cells in the central canal neurogenic niche of the postnatal spinal cord

    OpenAIRE

    Corns, Laura F; Deuchars, Jim; Deuchars, Susan A

    2013-01-01

    The area surrounding the central canal of the postnatal mammalian spinal cord is a highly plastic region that exhibits many similarities to other postnatal neurogenic niches, such as the subventricular zone. Within this region, ependymal cells have been identified as neural stem cells however very little is known about their properties and how the local environment, including neurotransmitters, is capable of affecting them. The neurotransmitter GABA is present around the central canal and is ...

  6. Diaphragm pacers as a treatment for congenital central hypoventilation syndrome.

    Science.gov (United States)

    Chen, Maida Lynn; Tablizo, Mary Anne; Kun, Sheila; Keens, Thomas G

    2005-09-01

    Congenital central hypoventilation syndrome is a rare syndrome present from birth, and is defined as the failure of automatic control of breathing. All patients with congenital central hypoventilation syndrome require life-long ventilatory support during sleep, although approximately a third of patients require ventilatory support 24 h per day. Diaphragm pacers offer a modality of ventilatory support that affords congenital central hypoventilation syndrome patients with maximal mobility for full-time ventilatory patients, and they may allow for a more normal lifestyle in the appropriate patient. They may permit tracheostomy decannulation in those requiring only support during sleep. Diaphragm pacing entails surgical placement of an electrode onto the phrenic nerve, connected to a subcutaneous receiver. There is an external battery-operated transmitter and antenna placed on the skin over the receiver. The transmitter emits energy, similar to radio transmission, which is converted into an electrical current by the receiver. This stimulates the phrenic nerve resulting in a diaphragmatic contraction. Settings on the transmitter include respiratory rate and electrical voltage, and are adjusted to give enough tidal volume to allow for adequate oxygenation and ventilation. Therefore, diaphragm pacing is an attractive alternative mode of mechanically assisted ventilation for many patients with congenital central hypoventilation syndrome.

  7. [Peripheral, central and functional vertigo syndromes].

    Science.gov (United States)

    Strupp, M; Dieterich, M; Zwergal, A; Brandt, T

    2015-12-01

    Depending on the temporal course, three forms of vertigo syndrome can be differentiated: 1) vertigo attacks, e.g. benign paroxysmal positional vertigo (BPPV), Menière's disease and vestibular migraine, 2) acute spontaneous vertigo lasting for days, e.g. acute unilateral vestibulopathy, brainstem or cerebellar infarction and 3) symptoms lasting for months or years, e.g. bilateral vestibulopathy and functional vertigo. The specific therapy of the various syndromes is based on three principles: 1) physical treatment with liberatory maneuvers for BPPV and balance training for vestibular deficits, 2) pharmacotherapy, e.g. for acute unilateral vestibulopathy (corticosteroids) and Menière's disease (transtympanic administration of gentamicin or steroids and high-dose betahistine therapy); placebo-controlled pharmacotherapy studies are currently being carried out for acute unilateral vestibulopathy, vestibular paroxysmia, prophylaxis of BPPV, vestibular migraine, episodic ataxia type 2 and cerebellar ataxia; 3) psychotherapy for functional dizziness.

  8. Guillain-Barre syndrome: A possibility in a spinal cord injured patient

    Directory of Open Access Journals (Sweden)

    Jagatsinh Yogendrasinh

    2007-01-01

    Full Text Available A 28-year-old male had paraplegia as a result of fracture dislocation of T12/L1 six years ago. He was functioning independently until four weeks ago, when he started complaining of trunkal paraesthesia which later progressed to include the upper extremities. The initial diagnosis was that of posttraumatic syringomyelia (PTS. While awaiting the MRI scan he developed weakness of upper limbs. The weakness restricted his self-care activities including transfers. The MRI did not show any evidence of syringomyelia. Neurological consultation and assessment yielded provisional diagnosis of Guillain-Barre syndrome (GBS. The patient was treated with immunoglobulins and regained 90% of his previous neurological status. This case is reported to raise awareness among clinicians to include the possibility of the GBS in the differential diagnosis of progressive neurological loss on top of existing neurological deficiency in spinal cord injured patients.

  9. Umbilical cord blood and neonatal endothelin-1 levels in preterm newborns with and without respiratory distress syndrome

    Directory of Open Access Journals (Sweden)

    A.C.W. Benjamin

    2005-09-01

    Full Text Available Increased pulmonary vascular resistance in preterm newborn infants with respiratory distress syndrome is suggested, and endothelin-1 plays an important role in pulmonary vascular reactivity in newborns. We determined umbilical cord blood and neonatal (second sample levels of endothelin-1 in 18 preterm newborns with respiratory distress syndrome who had no clinical or echocardiographic diagnosis of pulmonary hypertension and 22 without respiratory distress syndrome (gestational ages: 31.4 ± 1.6 and 29.3 ± 2.3 weeks, respectively. Umbilical cord blood and a second blood sample taken 18 to 40 h after birth were used for endothelin-1 determination by enzyme immunoassay. Median umbilical cord blood endothelin-1 levels were similar in both groups (control: 10.9 and respiratory distress syndrome: 11.4 pg/mL and were significantly higher than in the second sample (control: 1.7 pg/mL and respiratory distress syndrome: 3.5 pg/mL, P < 0.001 for both groups. Median endothelin-1 levels in the second sample were significantly higher in children with respiratory distress syndrome than in control infants (P < 0.001. There were significant positive correlations between second sample endothelin-1 and Score for Neonatal Acute Physiology and Perinatal Extension II (r = 0.36, P = 0.02, and duration of mechanical ventilation (r = 0.64, P = 0.02. A slower decline of endothelin-1 from birth to 40 h of life was observed in newborns with respiratory distress syndrome when compared to controls. A significant correlation between neonatal endothelin-1 levels and some illness-severity signs suggests that endothelin-1 plays a role in the natural course of respiratory distress syndrome in preterm newborns.

  10. Decreased cerebrospinal fluid flow through the central canal of the spinal cord of rats immunologically deprived of Reissner's fibre.

    Science.gov (United States)

    Cifuentes, M; Rodríguez, S; Pérez, J; Grondona, J M; Rodríguez, E M; Fernández-Llebrez, P

    1994-01-01

    The subcommissural organ is an ependymal brain gland that secretes glycoproteins to the cerebrospinal fluid (CSF) of the third ventricle. They condense to form a fibre, Reissner's fibre (RF), that runs along the aqueduct and fourth ventricle and the central canal of the spinal cord. A single injection of an antibody against the secretory glycoproteins of RF into a lateral ventricle of adult rats results in animals permanently deprived of RF in the central canal and bearing a "short" RF extending only along the aqueduct and the fourth ventricle. These animals, together with untreated control animals were used to investigate the probable influence of RF in the circulation of CSF in the central canal of the spinal cord. For this purpose, two tracers, (horseradish peroxidase and rabbit immunoglobulin) were injected into the ventricular CSF. The animals were killed 13, 20, 60, 120 and 240 min after the injection, and the amount of the tracers was estimated in tissue sections obtained at proximal, medial and distal levels of the spinal cord. In rats deprived of RF, a significant decrease in the amount of tracers present in the central canal was observed at all experimental intervals, being more evident at 20 min after the injection of the tracers. This suggests that lacking a RF in the central canal decreases the bulk flow of CSF along the central canal. Turbulences of the CSF at the entrance of the central canal of RF-deprived rats might explain the inability of the regenerating RF to progress along the central canal, as well as the reduced flow of CSF in the central canal of these animals.

  11. Differential pain modulation properties in central neuropathic pain after spinal cord injury.

    Science.gov (United States)

    Gruener, Hila; Zeilig, Gabi; Laufer, Yocheved; Blumen, Nava; Defrin, Ruth

    2016-07-01

    It seems that central neuropathic pain (CNP) is associated with altered abilities to modulate pain; whereas dysfunction in descending pain inhibition is associated with the extent of chronic pain distribution, enhanced pain excitation is associated with the intensity of chronic pain. We investigated the hypothesis that CNP is associated with decreased descending pain inhibition along with increased neuronal excitability and that both traits are associated with spinothalamic tract (STT) damage. Chronic spinal cord injury subjects with CNP (n = 27) and without CNP (n = 23) and healthy controls (n = 20) underwent the measurement of pain adaptation, conditioned pain modulation (CPM), tonic suprathreshold pain (TSP), and spatial summation of pain above injury level. Central neuropathic pain subjects also underwent at and below-lesion STT evaluation and completed the questionnaires. Central neuropathic pain subjects showed decreased CPM and increased enhancement of TSP compared with controls. Among CNP subjects, the dysfunction of CPM and pain adaptation correlated positively with the number of painful body regions. The magnitude of TSP and spatial summation of pain correlated positively with CNP intensity. STT scores correlated with CNP intensity and with TSP, so that the more affected the STT below injury level, the greater the CNP and TSP magnitude. It seems that CNP is associated with altered abilities to modulate pain, whereas dysfunction in descending pain inhibition is associated with the extent of chronic pain distribution and enhanced pain excitation is associated with the intensity of chronic pain. Thus, top-down processes may determine the spread of CNP, whereas bottom-up processes may determine CNP intensity. It also seems that the mechanisms of CNP may involve STT-induced hyperexcitability. Future, longitudinal studies may investigate the timeline of this scenario.

  12. Spatial and temporal activation of spinal glial cells: role of gliopathy in central neuropathic pain following spinal cord injury in rats.

    Science.gov (United States)

    Gwak, Young S; Kang, Jonghoon; Unabia, Geda C; Hulsebosch, Claire E

    2012-04-01

    In the spinal cord, neuron and glial cells actively interact and contribute to neurofunction. Surprisingly, both cell types have similar receptors, transporters and ion channels and also produce similar neurotransmitters and cytokines. The neuroanatomical and neurochemical similarities work synergistically to maintain physiological homeostasis in the normal spinal cord. However, in trauma or disease states, spinal glia become activated, dorsal horn neurons become hyperexcitable contributing to sensitized neuronal-glial circuits. The maladaptive spinal circuits directly affect synaptic excitability, including activation of intracellular downstream cascades that result in enhanced evoked and spontaneous activity in dorsal horn neurons with the result that abnormal pain syndromes develop. Recent literature reported that spinal cord injury produces glial activation in the dorsal horn; however, the majority of glial activation studies after SCI have focused on transient and/or acute time points, from a few hours to 1 month, and peri-lesion sites, a few millimeters rostral and caudal to the lesion site. In addition, thoracic spinal cord injury produces activation of astrocytes and microglia that contributes to dorsal horn neuronal hyperexcitability and central neuropathic pain in above-level, at-level and below-level segments remote from the lesion in the spinal cord. The cellular and molecular events of glial activation are not simple events, rather they are the consequence of a combination of several neurochemical and neurophysiological changes following SCI. The ionic imbalances, neuroinflammation and alterations of cell cycle proteins after SCI are predominant components for neuroanatomical and neurochemical changes that result in glial activation. More importantly, SCI induced release of glutamate, proinflammatory cytokines, ATP, reactive oxygen species (ROS) and neurotrophic factors trigger activation of postsynaptic neuron and glial cells via their own receptors

  13. Acute Sheehan's syndrome presenting as central diabetes insipidus.

    Science.gov (United States)

    Robalo, Raquel; Pedroso, Célia; Agapito, Ana; Borges, Augusta

    2012-11-06

    Sheehan's syndrome occurs as a result of ischaemic pituitary necrosis due to severe postpartum haemorrhage. Improvements in obstetrical care have significantly reduced its incidence in developed countries, but postpartum pituitary infarction remains a common cause of hypopituitarism in developing countries. We report a case of severe postpartum haemorrhage followed by headache, central diabetes insipidus and failure to lactate, which prompted us to investigate and identify both anterior and posterior pituitary deficiency compatible with Sheehan's syndrome. A timely diagnosis allowed us to implement an adequate treatment and follow-up plan, which are known to improve clinical status and patient outcome.

  14. Masquerade Syndrome of Multicentre Primary Central Nervous System Lymphoma

    Directory of Open Access Journals (Sweden)

    Silvana Guerriero

    2011-01-01

    Full Text Available Purpose. In Italy we say that the most unlucky things can happen to physicians when they get sick, despite the attention of colleagues. To confirm this rumor, we report the sad story of a surgeon with bilateral vitreitis and glaucoma unresponsive to traditional therapies. Methods/Design. Case report. Results. After one year of steroidal and immunosuppressive therapy, a vitrectomy, and a trabeculectomy for unresponsive bilateral vitreitis and glaucoma, MRI showed a multicentre primary central nervous system lymphoma, which was the underlying cause of the masquerade syndrome. Conclusions. All ophthalmologists and clinicians must be aware of masquerade syndromes, in order to avoid delays in diagnosis.

  15. Belly dancer's myoclonus and chronic abdominal pain: pain-related dysinhibition of a spinal cord central pattern generator?

    Science.gov (United States)

    Tamburin, Stefano; Idone, Domenico; Zanette, Giampietro

    2007-07-01

    We report on a patient with segmental rhythmic myoclonus resembling belly dance. This patient developed the myoclonus in temporal and anatomical association with chronic abdominal pain. No structural or metabolic abnormalities were found. EMG recordings suggested the presence of a spinal cord central pattern generator (CPG). We hypothesize that pain-related spinal plasticity might have contributed to the hyperactivity of a spinal CPG, thus leading to the myoclonic jerks in our patient.

  16. Pre-engraftment syndrome after myeloablative dual umbilical cord blood transplantation: Risk factors and response to treatment

    OpenAIRE

    Kanda, Junya; KAYNAR, LEYLAGUL; Kanda, Yoshinobu; Prasad, Vinod K.; Parikh, Suhag H.; Lan, Lan; SHEN, TONG; Rizzieri, David A.; Long, Gwynn D.; Sullivan, Keith M.; Gasparetto, Cristina; Chute, John P.; Morris, Ashley; Winkel, Scott; McPherson, Jacalyn

    2013-01-01

    High fever and/or rash prior to neutrophil engraftment are frequently observed after umbilical cord blood (UCB) transplantation, and the condition is referred to as pre-engraftment syndrome (PES). Few studies have evaluated the risk factors for and treatment response to PES. Therefore, we retrospectively characterized PES in 57 consecutive engrafted patients (≥12 years old) who received myeloablative dual UCB transplantation. All patients received TBI (≥13.2Gy)-based myeloablative conditionin...

  17. Symptomatic epidural lipomatosis of the spinal cord in a child: MR demonstration of spinal cord injury

    Energy Technology Data Exchange (ETDEWEB)

    Munoz, Alberto [Department of Radiology, Section of Neuroradiology, 505 Parnassus Av, L-371, University of California-San Francisco, CA 94143-0628 (United States); Servicio de Radiodiagnostico, Seccion de Neurorradiologia, Hospital Universitario ' ' 12 de Octubre' ' , 28040 Madrid (Spain); Barkovich, James A. [Department of Radiology, Section of Neuroradiology, 505 Parnassus Av, L-371, University of California-San Francisco, CA 94143-0628 (United States); Mateos, Fernando; Simon, Rogelio [Seccion de Neurpediatria, Servicio de Neurologia, Hospital Universitario ' ' 12 de Octubre' ' , 28041 Madrid (Spain)

    2002-12-01

    We report a case of symptomatic epidural lipomatosis in an 8-year-old girl with Cushing's syndrome secondary to longstanding high-dose steroid therapy for Crohn's disease. MR imaging of the spine revealed massive diffuse epidural fat compressing the entire spinal cord with T2 prolongation in the central gray matter of the cord suggesting ischemic myelopathy. This finding has not been previously demonstrated on imaging. A proposed mechanism underlying these findings is discussed. (orig.)

  18. Ependyma of the central canal of the rat spinal cord: a light and transmission electron microscopic study.

    Science.gov (United States)

    Bruni, J E; Reddy, K

    1987-06-01

    Ependymal cells of the rat central canal were examined with a view to identifying features that distinguish them regionally and from their counterparts elsewhere in the ventricular system. The results revealed that the lining consisted for the most part of a pseudostratified layer of uniformly organised cuboidal to columnar ependymal cells present in largest numbers in lumbar and sacral segments and in the conus. Two cell variants were identified on the basis of the presence or absence of a radially directed cytoplasmic process originating from the base of the cell. The tanycytic form of ependymal cell was encountered along the entire length of the central canal but with increased frequency in caudalmost segments. Ependymal cells were largely similar in ultrastructural appearance along the length of the cord. Although they were also similar in appearance and orientation to their counterparts in the ventricles they did exhibit some unique features. The most notable were the prominent junctional complexes and associated filaments present along the lateral border of the cells near their apex and the abundance of intermediate filaments in tanycytes. The central canal of the filum differed most markedly from other segments of the cord and resembled in structure the primitive ependymal tube of the caudal cord in lower vertebrates. Ependymal cells of the cord were not sufficiently dissimilar morphologically from their counterparts in the ventricles to account for differences in proliferative capacity in response to localised injury. A factor that merits further study is the difference in numbers of tanycyte ependymal cells in the two locations for they may be the reactive elements that proliferate in response to injury.

  19. Ascending central canal dilation and progressive ependymal disruption in a contusion model of rodent chronic spinal cord injury

    Directory of Open Access Journals (Sweden)

    Keirstead Hans S

    2007-09-01

    Full Text Available Abstract Background Chronic spinal cord injury (SCI can lead to an insidious decline in motor and sensory function in individuals even years after the initial injury and is accompanied by a slow and progressive cytoarchitectural destruction. At present, no pathological mechanisms satisfactorily explain the ongoing degeneration. Methods Adult female Sprague-Dawley rats were anesthetized laminectomized at T10 and received spinal cord contusion injuries with a force of 250 kilodynes using an Infinite Horizon Impactor. Animals were randomly distributed into 5 groups and killed 1 (n = 4, 28 (n = 4, 120 (n = 4, 450 (n = 5, or 540 (n = 5 days after injury. Morphometric and immunohistochemical studies were then performed on 1 mm block sections, 6 mm cranial and 6 mm caudal to the lesion epicenter. The SPSS 11.5 t test was used to determine differences between quantitative measures. Results Here, we document the first report of an ascending central canal dilation and progressive ependymal disruption cranial to the epicenter of injury in a contusion model of chronic SCI, which was characterized by extensive dural fibrosis and intraparenchymal cystic cavitation. Expansion of the central canal lumen beyond a critical diameter corresponded with ependymal cell ciliary loss, an empirically predictable thinning of the ependymal region, and a decrease in cell proliferation in the ependymal region. Large, aneurysmal dilations of the central canal were accompanied by disruptions in the ependymal layer, periependymal edema and gliosis, and destruction of the adjacent neuropil. Conclusion Cells of the ependymal region play an important role in CSF homeostasis, cellular signaling and wound repair in the spinal cord. The possible effects of this ascending pathology on ependymal function are discussed. Our studies suggest central canal dilation and ependymal region disruption as steps in the pathogenesis of chronic SCI, identify central canal dilation as a marker of

  20. GABAergic responses of mammalian ependymal cells in the central canal neurogenic niche of the postnatal spinal cord.

    Science.gov (United States)

    Corns, Laura F; Deuchars, Jim; Deuchars, Susan A

    2013-10-11

    The area surrounding the central canal of the postnatal mammalian spinal cord is a highly plastic region that exhibits many similarities to other postnatal neurogenic niches, such as the subventricular zone. Within this region, ependymal cells have been identified as neural stem cells however very little is known about their properties and how the local environment, including neurotransmitters, is capable of affecting them. The neurotransmitter GABA is present around the central canal and is known to affect cells within other postnatal neurogenic niches. This study used whole cell patch clamp electrophysiology and intracellular dye-loading in in vitro Wistar rat spinal cord slices to characterise ependymal cells and their ability to respond to GABA. Ependymal cells were defined by their passive response properties and low input resistances. Extensive dye-coupling was observed between ependymal cells; this was confirmed as gap junction coupling using the gap junction blocker, 18β-glycyrrhetinic acid, which significantly increased the input resistance of ependymal cells. GABA depolarised all ependymal cells tested; the partial antagonism of this response by bicuculline and gabazine indicates that GABA(A) receptors contribute to this response. A lack of effect by baclofen suggests that GABA(B) receptors do not contribute to the GABAergic response. The ability of ependymal cells to respond to GABA suggests that GABA could be capable of influencing the proliferation and differentiation of cells within the neurogenic niche of the postnatal spinal cord.

  1. Preclinical evidence supporting the clinical development of central pattern generator-modulating therapies for chronic spinal cord-injured patients

    Directory of Open Access Journals (Sweden)

    Pierre eGuertin

    2014-05-01

    Full Text Available Ambulation or walking is one of the main gaits of locomotion. In terrestrial animals, it may be defined as a series of rhythmic and bilaterally coordinated movement of the limbs which creates a forward movement of the body. This applies regardless of the number of limbs - from arthropods with six or more limbs to bipedal primates. These fundamental similarities among species may explain why comparable neural systems and cellular properties have been found, thus far, to control in similar ways locomotor rhythm generation in most animal models. The aim of this article is to provide a comprehensive review of the known structural and functional features associated with central nervous system (CNS networks that are involved in the control of ambulation and other stereotyped motor patterns - specifically Central Pattern Generators (CPGs that produce basic rhythmic patterned outputs for locomotion, micturition, ejaculation, and defecation. Although there is compelling evidence of their existence in humans, CPGs have been most studied in reduced models including in vitro isolated preparations, genetically-engineered mice and spinal cord-transected animals. Compared with other structures of the CNS, the spinal cord is generally considered as being well-preserved phylogenetically. As such, most animal models of SCI should be considered as valuable tools for the development of novel pharmacological strategies aimed at modulating spinal activity and restoring corresponding functions in chronic spinal cord-injured patients.

  2. An Unusual Presentation of Adult Tethered Cord Syndrome Associated with Severe Chest and Upper Back Pain

    Directory of Open Access Journals (Sweden)

    Shotaro Kanda

    2015-01-01

    Full Text Available Adult tethered cord syndrome (ATCS is a rare entity that usually presents with multiple neurological symptoms, including lower extremity pain, backache, lower extremity muscle weakness, and bowel/bladder disturbances. Prompt surgical treatment is often necessary to avoid permanent sequelae. We report a 63-year-old man with sudden-onset severe right chest and upper back pain, followed by urinary retention. His initial workup included computed tomography of the abdomen and pelvis, which showed a presacral mass. His symptom-driven neurological workup focused on the cervical and thoracic spine, the results of which were normal. Pelvic radiographs and magnetic resonance imaging of the lumbosacral spine showed spina bifida occulta, meningocele, and presacral masses consistent with a teratomatous tumor. His symptoms, except for urinary retention, improved dramatically with surgical treatment. The excised specimen contained a teratomatous lesion plus an organized hematoma. Hematoma formation was suspected as the trigger of his sudden-onset right chest and upper back pain.

  3. Primary and secondary tethered cord syndrome. MRT diagnosis and comparison with operative findings in 40 patients. Das primaere und sekundaere Tethered Cord Syndrom. Kernspintomographische Diagnostik und Vergleich mit operativen Befunden bei 40 Patienten

    Energy Technology Data Exchange (ETDEWEB)

    Reichel, M.; Hamm, B.; Wolf, K.J. (Radiologische Klinik, Abt. fuer Radiologische Diagnostik und Nuklearmedizin, Freie Univ. Berlin (Germany)); Weigel, K. (Neurochirurgische Klinik, Universitaets-Klinikum Steglitz, Freie Univ. Berlin (Germany))

    1992-08-01

    The MRT findings in 40 patients with the tethered cord syndrome (TCS) have been compared with the operative findings. In cases of primary TCS (17 patients), MRT showed a low conus medullaris in 15 cases and this was confirmed surgically in 14. Other abnormal findings were lipomas (MRT 12/operatively 13), diastematomyelia (MRT 2/operatively 2) and thickened filum terminale (MRT 6/operatively 3). In secondary TCS (23 patients) postoperatively adhesions were prominent (MRT 16/operatively 23) while low position of the conus was found by MRT in 19 and surgically in 18 patients. Other findings were lipomas (MRT 5/operatively 6), epidermoids (MRT 2/operatively 2) and diastematomyelia (MRT 2/operatively 0). Agreement between MRT and operative findings regarding fixation of the spinal cord is closer in primary TCS (15/17, 88%) than in secondary TCS (16/23, 69%). (orig.).

  4. Spinal cord stimulation for treatment of pain in a patient with post thoracotomy pain syndrome.

    Science.gov (United States)

    Graybill, Jordan; Conermann, Till; Kabazie, Abraham J; Chandy, Sunil

    2011-01-01

    Post Thoracotomy Pain Syndrome (PTPS) is defined as pain that occurs or persists in the area of the thoracotomy incision for at least 2 months following the initial procedure.  The true incidence of PTPS is hard to define as literature reports a wide range of occurrence from 5% to 90%.  Thoracotomy is associated with a high risk of severe chronic postoperative pain.  Presenting symptoms include both neuropathic pain in the area of the incision, as well as myofascial pain commonly in the ipsilateral scapula and shoulder.  Pain management can be challenging in these patients.  Multiple treatments have been described including conservative treatments with oral nonsteroidal anti-inflammatory drugs (NSAIDs); topically applied, peripherally acting drugs; neuromodulating agents; physical therapy; transcutaneous electrical nerve stimulation as well as more invasive treatments including intercostal nerve blocks, trigger point steroid injections, epidural steroid injections, radiofrequency nerve ablation, cryoablation, and one case report of spinal cord stimulation.  Unfortunately, a portion of these patients will have persistent pain in spite of multiple treatment modalities, and in some cases will experience worsening of pain. This case report describes the novel utility and complete resolution of symptoms with spinal cord stimulation (SCS) in treatment of a patient with persistent PTPS. In the operating room, a percutaneous octet electrode lead was placed using sterile technique under fluoroscopic guidance and loss-of-resistance technique.  The octet electrode lead was subsequently advanced with the aid of fluoroscopy to the level of the T3 superior endplate just right of midline.  The patient's pain distribution was captured optimally with stimulation at this level.  With the assistance of a neurosurgeon, the lead was anchored, tunneled, and connected to a generator, which was implanted over the right iliac crest.  The patient tolerated the procedure well with

  5. Central pain.

    Science.gov (United States)

    Singh, Supreet

    2014-12-01

    Questions from patients about pain conditions and analgesic pharmacotherapy and responses from authors are presented to help educate patients and make them more effective self-advocates. The topic addressed in this issue is central pain, a neuropathic pain syndrome caused by a lesion in the brain or spinal cord that sensitizes one's perception of pain. It is a debilitating condition caused by various diseases such as multiple sclerosis, strokes, spinal cord injuries, or brain tumors. Varied symptoms and the use of pharmacological medicines and nonpharmacological therapies will be addressed.

  6. An electron microscopic study of the development of the ependyma of the central canal of the mouse spinal cord.

    OpenAIRE

    Sturrock, R R

    1981-01-01

    The central canal of the adult mouse spinal cord is lined for most of its extent by ependymal cells which are rich in microfilaments and whose apical surface is covered with matted, broad microvilli. The canal itself is filled with amorphous material containing glycogen granules. Two forms of this material are present, a dark form rich in glycogen, and a light form containing a few glycogen granules. Each type appears to be surrounded by a membrane. The upper cervical region, however, has a l...

  7. [Central hemodynamic changes in children reoperated on for congenital spinal cord hernia using balanced anesthesia based on midasolam and propofol].

    Science.gov (United States)

    Diordiev, A V; Kontakevich, M M; Il'in, A V; Kusakin, V V; Ostreĭkov, I F

    2005-01-01

    The central hemodynamics was studied and analyzed in 51 patients reoperated on for congenital spinal cord hernia under balanced anesthesia based on midasolam and proforol. The procedure for anesthesiological provision of replastic repair of operated spinal hernia, which is based on propofol of bolus administration of midasolam with a hypnotic appliance, was found to cause no negative hemodynamic effects. When the benzodiazepine antagonist flumazenyl is used, the interval between the end of surgery and tracheal extubation is virtually identical in the propofol and midasolam groups since the intravenous injection of flumazenyl induces a drastic awakening effect.

  8. Small for Gestational Age and Magnesium in Cord Blood Platelets: Intrauterine Magnesium Deficiency May Induce Metabolic Syndrome in Later Life

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    Junji Takaya

    2011-01-01

    Full Text Available Magnesium deficiency in pregnancy frequently occurs because of inadequate or low intake of magnesium. Magnesium deficiency during pregnancy can induce not only maternal and fetal nutritional problems, but also consequences that might last in offspring throughout life. Many epidemiological studies have disclosed that small for gestational age (SGA is associated with an increased risk of insulin resistance in adult life. We reported that intracellular magnesium of cord blood platelets is lower in SGA groups than that in appropriate for gestational age groups, suggesting that intrauterine magnesium deficiency may result in SGA. Taken together, intrauterine magnesium deficiency in the fetus may lead to or at least program insulin resistance after birth. In this review, we propose that intrauterine magnesium deficiency may induce metabolic syndrome in later life. We discuss the potential contribution of aberrant magnesium regulation to SGA and to the pathogenesis of metabolic syndrome.

  9. Congenital central hypoventilation syndrome: diagnostic and management challenges

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    Kasi AS

    2016-08-01

    Full Text Available Ajay S Kasi,1 Iris A Perez,1,2 Sheila S Kun,1 Thomas G Keens1,2 1Division of Pediatric Pulmonology and Sleep Medicine, Children’s Hospital Los Angeles, 2Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA Abstract: Congenital central hypoventilation syndrome (CCHS is a rare genetic disorder with failure of central control of breathing and of the autonomic nervous system function due to a mutation in the paired-like homeobox 2B (PHOX2B gene. Affected patients have absent or negligible ventilatory sensitivity to hypercapnia and hypoxemia, and they do not exhibit signs of respiratory distress when challenged with hypercarbia or hypoxia. The diagnosis of CCHS must be confirmed with PHOX2B gene mutation. Generally, the PHOX2B mutation genotype can aid in anticipating the severity of the phenotype. They require ventilatory support for life. Home assisted ventilation options include positive pressure ventilation via tracheostomy, noninvasive positive pressure ventilation, and diaphragm pacing via phrenic nerve stimulation, but each strategy has its associated limitations and challenges. Since all the clinical manifestations of CCHS may not manifest at birth, periodic monitoring and early intervention are necessary to prevent complications and improve outcome. Life-threatening arrhythmias can manifest at different ages and a normal cardiac monitoring study does not exclude future occurrences leading to the dilemma of timing and frequency of cardiac rhythm monitoring and treatment. Given the rare incidence of CCHS, most health care professionals are not experienced with managing CCHS patients, particularly those with diaphragm pacers. With early diagnosis and advances in home mechanical ventilation and monitoring strategies, many CCHS children are surviving into adulthood presenting new challenges in their care. Keywords: congenital central hypoventilation syndrome, PHOX2B, home mechanical ventilation, diaphragm

  10. Acrolein contributes to TRPA1 up-regulation in peripheral and central sensory hypersensitivity following spinal cord injury.

    Science.gov (United States)

    Park, Jonghyuck; Zheng, Lingxing; Acosta, Glen; Vega-Alvarez, Sasha; Chen, Zhe; Muratori, Breanne; Cao, Peng; Shi, Riyi

    2015-12-01

    Acrolein, an endogenous aldehyde, has been shown to be involved in sensory hypersensitivity after rat spinal cord injury (SCI), for which the pathogenesis is unclear. Acrolein can directly activate a pro-algesic transient receptor protein ankyrin 1 (TRPA1) channel that exists in sensory neurons. Both acrolein and TRPA1 mRNA are elevated post SCI, which contributes to the activation of TRPA1 by acrolein and consequently, neuropathic pain. In the current study, we further showed that, post-SCI elevation of TRPA1 mRNA exists not only in dorsal root ganglias but also in both peripheral (paw skin) and central endings of primary afferent nerves (dorsal horn of spinal cord). This is the first indication that pain signaling can be over-amplified in the peripheral skin by elevated expressions of TRPA1 following SCI, in addition over-amplification previously seen in the spinal cord and dorsal root ganglia. Furthermore, we show that acrolein alone, in the absence of physical trauma, could lead to the elevation of TRPA1 mRNA at various locations when injected to the spinal cord. In addition, post-SCI elevation of TRPA1 mRNA could be mitigated using acrolein scavengers. Both of these attributes support the critical role of acrolein in elevating TRPA1 expression through gene regulation. Taken together, these data indicate that acrolein is likely a critical causal factor in heightening pain sensation post-SCI, through both the direct binding of TRPA1 receptor, and also by boosting the expression of TRPA1. Finally, our data also further support the notion that acrolein scavenging may be an effective therapeutic approach to alleviate neuropathic pain after SCI. We propose that the trauma-mediated elevation of acrolein causes neuropathic pain through at least two mechanisms: acrolein stimulates the production of transient receptor protein ankyrin 1 (TRPA1) in both central and peripheral locations, and it activates TRPA1 channels directly. Therefore, acrolein appears to be a critical

  11. Selective adenosine A2A receptor agonists and antagonists protect against spinal cord injury through peripheral and central effects

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    Esposito Emanuela

    2011-04-01

    Full Text Available Abstract Background Permanent functional deficits following spinal cord injury (SCI arise both from mechanical injury and from secondary tissue reactions involving inflammation. Enhanced release of adenosine and glutamate soon after SCI represents a component in the sequelae that may be responsible for resulting functional deficits. The role of adenosine A2A receptor in central ischemia/trauma is still to be elucidated. In our previous studies we have demonstrated that the adenosine A2A receptor-selective agonist CGS21680, systemically administered after SCI, protects from tissue damage, locomotor dysfunction and different inflammatory readouts. In this work we studied the effect of the adenosine A2A receptor antagonist SCH58261, systemically administered after SCI, on the same parameters. We investigated the hypothesis that the main action mechanism of agonists and antagonists is at peripheral or central sites. Methods Spinal trauma was induced by extradural compression of SC exposed via a four-level T5-T8 laminectomy in mouse. Three drug-dosing protocols were utilized: a short-term systemic administration by intraperitoneal injection, a chronic administration via osmotic minipump, and direct injection into the spinal cord. Results SCH58261, systemically administered (0.01 mg/kg intraperitoneal. 1, 6 and 10 hours after SCI, reduced demyelination and levels of TNF-α, Fas-L, PAR, Bax expression and activation of JNK mitogen-activated protein kinase (MAPK 24 hours after SCI. Chronic SCH58261 administration, by mini-osmotic pump delivery for 10 days, improved the neurological deficit up to 10 days after SCI. Adenosine A2A receptors are physiologically expressed in the spinal cord by astrocytes, microglia and oligodendrocytes. Soon after SCI (24 hours, these receptors showed enhanced expression in neurons. Both the A2A agonist and antagonist, administered intraperitoneally, reduced expression of the A2A receptor, ruling out the possibility that the

  12. Successful management of aortic thrombi resulting in spinal cord infarction in a patient with antiphospholipid antibody syndrome and acute cholecystitis

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    Izumi M

    2011-12-01

    Full Text Available Manabu Izumi, Shoko Teraoka, Keisuke Yamashita, Kenji Matsumoto, Tomohiro Muronoi, Yoshimitsu Izawa, Chikara Yonekawa, Masaki Ano, Masayuki SuzukawaDepartment of Emergency and Critical Care Medicine, Jichi Medical University, Tochigi, JapanAbstract: A 74-year-old man with coronary artery disease was suffering from acute nonobstructive cholecystitis and was admitted to a nearby hospital. Dual antiplatelet (aspirin and ticlopidine therapy was discontinued before preparation for surgical resection of the gall bladder. During his time in hospital he was aware of lumbar pain and weakness in both legs. He was transferred to our hospital for further evaluation and therapy. Diffuse intra-aortic thrombi were revealed by computed tomography with contrast media, and magnetic resonance imaging showed spinal cord infarction. However, computed tomography scans of the descending aorta obtained 4 months before admission exhibited no signs of atherosclerotic plaques or intra-aortic thrombi. Laboratory data suggest that antiphospholipid antibody syndrome might have caused these acute multiple intra-arterial thrombi. By restarting dual antiplatelet therapy and increasing the dose of heparin (from 10,000 IU/day to 15,000 IU/day we successfully managed the patient's clinical condition and symptoms. It is important to understand that stopping antiplatelet therapy may rapidly grow thrombi in patients with a hypercoagulative state.Keywords: intra-aortic thrombus, antiphospholipid antibody syndrome, spinal cord infarction

  13. Central neurogenic diabetes insipidus, syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome in traumatic brain injury.

    Science.gov (United States)

    John, Cynthia A; Day, Michael W

    2012-04-01

    Central neurogenic diabetes insipidus, syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome are secondary events that affect patients with traumatic brain injury. All 3 syndromes affect both sodium and water balance; however, they have differences in pathophysiology, diagnosis, and treatment. Differentiating between hypernatremia (central neurogenic diabetes insipidus) and the 2 hyponatremia syndromes (syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome) is critical for preventing worsening neurological outcomes in patients with head injuries.

  14. Stenosis of central canal of spinal cord in man: incidence and pathological findings in 232 autopsy cases.

    Science.gov (United States)

    Milhorat, T H; Kotzen, R M; Anzil, A P

    1994-04-01

    The central canal of the spinal cord is generally regarded as a vestigial structure that is obliterated after birth in 70% to 80% of the general population. This report describes the first detailed histological study of the human central canal in 232 subjects ranging in age from 6 weeks' gestation to 92 years. Whole spinal cords were harvested at autopsy and sectioned serially from the conus medullaris to the upper medulla. Histological findings and morphometric analysis of the cross-sectional luminal area were used to grade stenosis at seven levels of the canal. Varying grades of stenosis were present at one or more levels in none (0%) of 60 fetuses, one (3%) of 34 infants, three (18%) of 17 children, 21 (88%) of 24 adolescents and young adults, 67 (96%) of 70 middle-aged adults, and all 27 adults aged 65 years or older (100%). The stenotic process was most pronounced in the thoracic segments of the canal and involved more levels with higher grades of stenosis in older individuals. Histological findings consisted of disorganization of the ependymal epithelium, formation of ependymal rosettes or microcanals, proliferation of subependymal gliovascular buds, and intracanalicular gliosis. These features are consistent with a pathological lesion involving ependymal injury and scarring and are less compatible with an involutional or degenerative process. Stenosis of the central canal probably influences the anatomical features of syringomyelia and may account for variations in cavity formation such as the prevalence of holocord syrinxes in children, the formation of focal and paracentral syrinxes in adults, and the rare incidence of syrinx formation in many older individuals with acquired lesions known to produce syringomyelia.

  15. Central canal ependymal cells proliferate extensively in response to traumatic spinal cord injury but not demyelinating lesions.

    Science.gov (United States)

    Lacroix, Steve; Hamilton, Laura K; Vaugeois, Alexandre; Beaudoin, Stéfanny; Breault-Dugas, Christian; Pineau, Isabelle; Lévesque, Sébastien A; Grégoire, Catherine-Alexandra; Fernandes, Karl J L

    2014-01-01

    The adult mammalian spinal cord has limited regenerative capacity in settings such as spinal cord injury (SCI) and multiple sclerosis (MS). Recent studies have revealed that ependymal cells lining the central canal possess latent neural stem cell potential, undergoing proliferation and multi-lineage differentiation following experimental SCI. To determine whether reactive ependymal cells are a realistic endogenous cell population to target in order to promote spinal cord repair, we assessed the spatiotemporal dynamics of ependymal cell proliferation for up to 35 days in three models of spinal pathologies: contusion SCI using the Infinite Horizon impactor, focal demyelination by intraspinal injection of lysophosphatidylcholine (LPC), and autoimmune-mediated multi-focal demyelination using the active experimental autoimmune encephalomyelitis (EAE) model of MS. Contusion SCI at the T9-10 thoracic level stimulated a robust, long-lasting and long-distance wave of ependymal proliferation that peaked at 3 days in the lesion segment, 14 days in the rostral segment, and was still detectable at the cervical level, where it peaked at 21 days. This proliferative wave was suppressed distal to the contusion. Unlike SCI, neither chemical- nor autoimmune-mediated demyelination triggered ependymal cell proliferation at any time point, despite the occurrence of demyelination (LPC and EAE), remyelination (LPC) and significant locomotor defects (EAE). Thus, traumatic SCI induces widespread and enduring activation of reactive ependymal cells, identifying them as a robust cell population to target for therapeutic manipulation after contusion; conversely, neither demyelination, remyelination nor autoimmunity appears sufficient to trigger proliferation of quiescent ependymal cells in models of MS-like demyelinating diseases.

  16. Central canal ependymal cells proliferate extensively in response to traumatic spinal cord injury but not demyelinating lesions.

    Directory of Open Access Journals (Sweden)

    Steve Lacroix

    Full Text Available The adult mammalian spinal cord has limited regenerative capacity in settings such as spinal cord injury (SCI and multiple sclerosis (MS. Recent studies have revealed that ependymal cells lining the central canal possess latent neural stem cell potential, undergoing proliferation and multi-lineage differentiation following experimental SCI. To determine whether reactive ependymal cells are a realistic endogenous cell population to target in order to promote spinal cord repair, we assessed the spatiotemporal dynamics of ependymal cell proliferation for up to 35 days in three models of spinal pathologies: contusion SCI using the Infinite Horizon impactor, focal demyelination by intraspinal injection of lysophosphatidylcholine (LPC, and autoimmune-mediated multi-focal demyelination using the active experimental autoimmune encephalomyelitis (EAE model of MS. Contusion SCI at the T9-10 thoracic level stimulated a robust, long-lasting and long-distance wave of ependymal proliferation that peaked at 3 days in the lesion segment, 14 days in the rostral segment, and was still detectable at the cervical level, where it peaked at 21 days. This proliferative wave was suppressed distal to the contusion. Unlike SCI, neither chemical- nor autoimmune-mediated demyelination triggered ependymal cell proliferation at any time point, despite the occurrence of demyelination (LPC and EAE, remyelination (LPC and significant locomotor defects (EAE. Thus, traumatic SCI induces widespread and enduring activation of reactive ependymal cells, identifying them as a robust cell population to target for therapeutic manipulation after contusion; conversely, neither demyelination, remyelination nor autoimmunity appears sufficient to trigger proliferation of quiescent ependymal cells in models of MS-like demyelinating diseases.

  17. Catastrophic Antiphospholipid Syndrome Presenting as Bilateral Central Retinal Artery Occlusions

    Directory of Open Access Journals (Sweden)

    Steven S. Saraf

    2015-01-01

    Full Text Available A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial “boxcarring,” retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient’s vision improved and remained stable at 20/200 and 20/80.

  18. Hippocampal volume reduction in congenital central hypoventilation syndrome.

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    Paul M Macey

    Full Text Available Children with congenital central hypoventilation syndrome (CCHS, a genetic disorder characterized by diminished drive to breathe during sleep and impaired CO(2 sensitivity, show brain structural and functional changes on magnetic resonance imaging (MRI scans, with impaired responses in specific hippocampal regions, suggesting localized injury.We assessed total volume and regional variation in hippocampal surface morphology to identify areas affected in the syndrome. We studied 18 CCHS (mean age+/-std: 15.1+/-2.2 years; 8 female and 32 healthy control (age 15.2+/-2.4 years; 14 female children, and traced hippocampi on 1 mm(3 resolution T1-weighted scans, collected with a 3.0 Tesla MRI scanner. Regional hippocampal volume variations, adjusted for cranial volume, were compared between groups based on t-tests of surface distances to the structure midline, with correction for multiple comparisons. Significant tissue losses emerged in CCHS patients on the left side, with a trend for loss on the right; however, most areas affected on the left also showed equivalent right-sided volume reductions. Reduced regional volumes appeared in the left rostral hippocampus, bilateral areas in mid and mid-to-caudal regions, and a dorsal-caudal region, adjacent to the fimbria.The volume losses may result from hypoxic exposure following hypoventilation during sleep-disordered breathing, or from developmental or vascular consequences of genetic mutations in the syndrome. The sites of change overlap regions of abnormal functional responses to respiratory and autonomic challenges. Affected hippocampal areas have roles associated with memory, mood, and indirectly, autonomic regulation; impairments in these behavioral and physiological functions appear in CCHS.

  19. Herpesvirus-6 encephalitis complicated by Wernicke-Korsakoff syndrome in a pediatric recipient of unrelated cord blood transplantation.

    Science.gov (United States)

    Carvajal, E; Verdeguer, A; Fernández, J M; Cañete, A; Castel, V

    2001-12-01

    A 10-year-old girl with M2 acute myeloid leukemia underwent an unrelated cord blood transplantation in refractory first relapse. On day +13, after 48 hours with fever, she showed a measles-like rash, and on day +15, she began experiencing neurologic symptoms (headache, tremors, weakness, nystagmus, mild confusion, speaking, taste, and behavior disturbances, and focal seizures). She also had amnesia for recent events with disability to learn, mimicking Wernicke-Korsakoff syndrome. Computed tomography of the brain and cerebrospinal fluid (CSF) and electroencephalogram were nonspecific. We found human herpesvirus 6 (HHV-6) DNA in CSF and cytomegalovirus in bronchoalveolar lavage using polymerase chain reaction techniques. Treatment with ganciclovir and foscarnet was effective, with total resolution of symptoms.

  20. Central versus peripheral cardiovascular risk in metabolic syndrome

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    Heather eEdgell

    2012-02-01

    Full Text Available Individuals with metabolic syndrome (MetS; i.e. 3 of 5 of the following risk factors (RFs: elevated blood pressure, waist circumference, triglycerides, blood glucose or reduced HDL are thought to be prone to serious cardiovascular disease and there is debate as to whether the disease begins in the peripheral vasculature or centrally. This study investigates hemodynamics, cardiac function/morphology, as well as mechanical properties of the central (heart, carotid artery and peripheral (total peripheral resistance, forearm vascular bed vasculature in individuals without (1-2 RFs; n=28, or with (≥3 RFs; n=46 MetS. After adjustments for statin and blood pressure medication use, those with MetS had lower mitral valve E/A ratios (<3 RFs: 1.24±0.07; ≥3 RFs: 1.01±0.04; P=0.025, and higher total peripheral resistance index (<3 RFs: 48±2 mmHg/L/min/m2; ≥3 RFs: 53±2 mmHg/L/min/m2; P=0.04. There were no differences in heart size, carotid artery measurements, cardiovagal baroreflex sensitivity, pulse wave velocity, stroke volume index, or cardiac output index due to MetS after adjustments for statin and blood pressure medication use. In a separate analysis, the use of statins was associated with increased inertia in the brachial vascular bed, increased HbA1c and decreased LDL cholesterol. The independent use of anti-hypertensive medication was associated with decreased predicted VO2max, triglycerides, diastolic blood pressure, interventricular septum thickness, calculated left ventricle mass, left ventricle posterior wall thickness, and left ventricle pre-ejection period, but increased carotid stiffness, HDL cholesterol, and heart rate. These data imply that both a central cardiac effect and a peripheral effect of vascular resistance are expressed in MetS. These data also indicate that variance in between-group responses due to pharmacological treatments are important factors to consider in studying cardiovascular changes in these individuals.

  1. Central sensitization in spinal cord injured humans assessed by reflex receptive fields

    DEFF Research Database (Denmark)

    Biurrun Manresa, José Alberto; Finnerup, Nanna Brix; Johannesen, Inger Lauge

    2014-01-01

    after an intramuscular injection of capsaicin in the foot sole in order to induce central sensitization. RESULTS: Both groups presented RRF expansion and lowered NWR thresholds immediately after capsaicin injection, reflected by the enlargement of RRF sensitivity areas and RRF probability areas....... Moreover, the topography of the RRF sensitivity and probability areas were significantly different in SCI volunteers compared to NI volunteers in terms of size and shape. CONCLUSIONS: SCI volunteers can develop central sensitization, despite adaptive/maladaptive changes in synaptic plasticity and lack...

  2. The nature and course of sensory changes following spinal cord injury: predictive properties and implications on the mechanism of central pain.

    Science.gov (United States)

    Zeilig, Gabi; Enosh, Shavit; Rubin-Asher, Deborah; Lehr, Benjamin; Defrin, Ruth

    2012-02-01

    Central pain below the injury level after spinal cord injury is excruciating, chronic and resistive to treatment. Animal studies suggest that pretreatment may prevent central pain, but to date there are no measures to predict its development. Our aim was to monitor changes in the sensory profile below the lesion prior to the development of below-level central pain in order to search for a parameter that could predict its risk and to further explore its pathophysiology. Thirty patients with spinal cord injury and 27 healthy controls underwent measurement of warm, cold, heat-pain and touch thresholds as well as graphaesthesia, allodynia, hyperpathia and wind-up pain in intact region and in the shin and feet (below level). Patients were tested at 2-4 weeks, 1-2.5 months and 2.5-6 months after the injury or until central pain had developed. At the end of the follow-up, 46% of patients developed below-level central pain. During the testing periods, individuals who eventually developed central pain had higher thermal thresholds than those who did not and displayed high rates of abnormal sensations (allodynia and hyperpathia), which gradually increased with time until central pain developed. Logistic regressions revealed that the best predictor for the risk of below-level central pain was allodynia in the foot in the second testing session with a 77% probability (90.9% confidence). The results suggest that neuronal hyperexcitability, which may develop consequent to damage to spinothalamic tracts, precedes central pain. Furthermore, it appears that below-level central pain develops after a substantial build-up of hyperexcitability. To the best of our knowledge, this is the first systematic report establishing that neuronal hyperexcitability precedes central pain. Predicting the risk for central pain can be utilized to initiate early treatment in order to prevent its development.

  3. Mild moxibustion decreases the expression of prokineticin 2 and prokineticin receptor 2 in the colon and spinal cord of rats with irritable bowel syndrome.

    Science.gov (United States)

    Zhou, Cili; Zhao, Jimeng; Wu, Luyi; Huang, Renjia; Shi, Yin; Wang, Xiaomei; Liao, Wen; Hong, Jue; Liu, Shimin; Wu, Huangan

    2014-01-01

    It has been proven that prokineticin 2 (PK2) and its receptor PKR2 play an important role in hyperalgesia, while mild moxibustion can relieve visceral hypersensitivity in a rat model of irritable bowel syndrome (IBS). The goal of the present study was to determine the effects of mild moxibustion on the expression of PK2 and PKR2 in colon and spinal cord in IBS rat model, which was induced by colorectal distension using inflatable balloons. After mild moxibustion treatment, abdominal withdrawal reflex (AWR) scores were assessed by colorectal distension; protein and mRNA expression of PK2 and PKR2 in rat colon and spinal cord was determined by immunohistochemistry and fluorescence quantitative PCR. Compared with normal rats, the AWR scores of rats and the expressions of PK2/PKR2 proteins and mRNAs in colon and spinal cord tissue were significantly increased in the model group; compared with the model group, the AWR scores of rats and the expressions of PK2/PKR2 proteins and mRNAs in colon and spinal cord tissue were significantly decreased in the mild moxibustion group. These findings suggest that the analgesia effect of mild moxibustion may be associated with the reduction of the abnormally increased expression of the PK2/PKR2 proteins and mRNAs in the colon and spinal cord.

  4. Mild Moxibustion Decreases the Expression of Prokineticin 2 and Prokineticin Receptor 2 in the Colon and Spinal Cord of Rats with Irritable Bowel Syndrome

    Directory of Open Access Journals (Sweden)

    Cili Zhou

    2014-01-01

    Full Text Available It has been proven that prokineticin 2 (PK2 and its receptor PKR2 play an important role in hyperalgesia, while mild moxibustion can relieve visceral hypersensitivity in a rat model of irritable bowel syndrome (IBS. The goal of the present study was to determine the effects of mild moxibustion on the expression of PK2 and PKR2 in colon and spinal cord in IBS rat model, which was induced by colorectal distension using inflatable balloons. After mild moxibustion treatment, abdominal withdrawal reflex (AWR scores were assessed by colorectal distension; protein and mRNA expression of PK2 and PKR2 in rat colon and spinal cord was determined by immunohistochemistry and fluorescence quantitative PCR. Compared with normal rats, the AWR scores of rats and the expressions of PK2/PKR2 proteins and mRNAs in colon and spinal cord tissue were significantly increased in the model group; compared with the model group, the AWR scores of rats and the expressions of PK2/PKR2 proteins and mRNAs in colon and spinal cord tissue were significantly decreased in the mild moxibustion group. These findings suggest that the analgesia effect of mild moxibustion may be associated with the reduction of the abnormally increased expression of the PK2/PKR2 proteins and mRNAs in the colon and spinal cord.

  5. Dynamic Changes in Local Protein Synthetic Machinery in Regenerating Central Nervous System Axons after Spinal Cord Injury

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    Rahul Sachdeva

    2016-01-01

    Full Text Available Intra-axonal localization of mRNAs and protein synthesis machinery (PSM endows neurons with the capacity to generate proteins locally, allowing precise spatiotemporal regulation of the axonal response to extracellular stimuli. A number of studies suggest that this local translation is a promising target to enhance the regenerative capacity of damaged axons. Using a model of central nervous system (CNS axons regenerating into intraspinal peripheral nerve grafts (PNGs we established that adult regenerating CNS axons contain several different mRNAs and protein synthetic machinery (PSM components in vivo. After lower thoracic level spinal cord transection, ascending sensory axons regenerate into intraspinal PNGs but axon growth is stalled when they reach the distal end of the PNG (3 versus 7 weeks after grafting, resp.. By immunofluorescence with optical sectioning of axons by confocal microscopy, the total and phosphorylated forms of PSMs are significantly lower in stalled compared with actively regenerating axons. Reinjury of these stalled axons increased axonal localization of the PSM proteins, indicative of possible priming for a subcellular response to axotomy. These results suggest that axons downregulate protein synthetic capacity as they cease growing, yet they retain the ability to upregulate PSM after a second injury.

  6. Should waist circumference be replaced by index of central obesity (ICO) in definition of metabolic syndrome?

    Science.gov (United States)

    Parikh, R; Mohan, V; Joshi, S

    2012-01-01

    Waist circumference has been widely used as the parameter of central obesity in defining metabolic syndrome. Global consensus definition of metabolic syndrome has suggested various race- and gender-specific cutoffs of waist circumference for quantifying central obesity. We have earlier proposed that using index of central obesity (ICO), the need for race- and gender-specific cutoffs may be obviated. We propose that waist circumference be supplanted with index of central obesity in all definitions of metabolic syndrome. Using index of central obesity a common cutoff of 0.5 applicable across races and genders might be obtained. Moreover, it will enhance the sensitivity of definition by diagnosing subjects who are shorter than general population. Among 258 male and 242 female diabetic patients, subjects with metabolic syndrome (defined as per gender-specific waist circumference cutoffs), were found to have a common lower range of index of central obesity suggesting that a common cutoff of waist circumference may be obtained. In another study from National Health and Nutrition Examination Survey database, replacing index of central obesity with waist circumference was found to enhance the specificity and sensitivity of definition of metabolic syndrome.

  7. Síndrome de Klippel-Trenaunay-Parkes-Weber com angiomatose medular Klippel-Trénaunay-Parkes-Weber syndrome with spinal cord angioma: a case report

    Directory of Open Access Journals (Sweden)

    James Pitagoras de Mattos

    1975-09-01

    Full Text Available É relatado um caso de síndrome de Klippel-Trénaunay-Parkes-Weber associada a angiomatose medular. O autor salienta ter encontrado na literatura somente uma referência com tal associação.A case of Klippel-Trénaunay-Parkes-Weber syndrome associated with spinal cord angioma is reported. The author points out that it was found only one reference in literature with such association.

  8. Functional and metabolic changes in the brain in neuropathic pain syndrome against the background of chronic epidural electrostimulation of the spinal cord.

    Science.gov (United States)

    Sufianov, A A; Shapkin, A G; Sufianova, G Z; Elishev, V G; Barashin, D A; Berdichevskii, V B; Churkin, S V

    2014-08-01

    Changes in functional and metabolic activities of the brain were evaluated by EEG and positron-emission/computer tomography with 18F-fluorodeoxyglucose in patients with neuropathic pain syndrome previous to and 3 months after implantation of a system for chronic epidural spinal cord stimulation. In most cases, the use of a nerve stimulator was followed by alleviation of neuropathic pain and partial normalization of functional and metabolic activities of brain structures responsible for pain perception, emotiogenic, behavioral, and autonomic responses.

  9. Cellular organization of the central canal ependymal zone, a niche of latent neural stem cells in the adult mammalian spinal cord.

    Science.gov (United States)

    Hamilton, L K; Truong, M K V; Bednarczyk, M R; Aumont, A; Fernandes, K J L

    2009-12-15

    A stem cell's microenvironment, or "niche," is a critical regulator of its behaviour. In the adult mammalian spinal cord, central canal ependymal cells possess latent neural stem cell properties, but the ependymal cell niche has not yet been described. Here, we identify important similarities and differences between the central canal ependymal zone and the forebrain subventricular zone (SVZ), a well-characterized niche of neural stem cells. First, direct immunohistochemical comparison of the spinal cord ependymal zone and the forebrain SVZ revealed distinct patterns of neural precursor marker expression. In particular, ependymal cells in the spinal cord were found to be bordered by a previously uncharacterized sub-ependymal layer, which is relatively less elaborate than that of the SVZ and comprised of small numbers of astrocytes, oligodendrocyte progenitors and neurons. Cell proliferation surrounding the central canal occurs in close association with blood vessels, but unlike in the SVZ, involves mainly ependymal rather than sub-ependymal cells. These proliferating ependymal cells typically self-renew rather than produce transit-amplifying progenitors, as they generate doublets of progeny that remain within the ependymal layer and show no evidence of a lineage relationship to sub-ependymal cells. Interestingly, the dorsal pole of the central canal was found to possess a sub-population of tanycyte-like cells that express markers of both ependymal cells and neural precursors, and their presence correlates with higher numbers of dorsally proliferating ependymal cells. Together, these data identify key features of the spinal cord ependymal cell niche, and suggest that dorsal ependymal cells possess the potential for stem cell activity. This work provides a foundation for future studies aimed at understanding ependymal cell regulation under normal and pathological conditions.

  10. Central nervous system involvement in primary Sjogren`s syndrome manifesting as multiple sclerosis.

    Science.gov (United States)

    Liu, Jing-Yao; Zhao, Teng; Zhou, Chun-Kui

    2014-04-01

    Central nervous system symptoms in patients with primary Sjogren`s syndrome are rare. They can present as extraglandular manifestations and require a differential diagnosis from multiple sclerosis. Due to a variety of presentations, Sjogren`s syndrome with neurologic involvement may be difficult to diagnose. Here, we report a case of a 75-year-old woman who was first diagnosed with multiple sclerosis in 2010, but who was subsequently diagnosed with primary Sjogren`s syndrome 2 years later after showing signs of atypical neurologic manifestations. Therefore, primary Sjogren`s syndrome should be suspected in patients who present with atypical clinical and radiologic neurologic manifestations.

  11. Dorsal border periaqueductal gray neurons project to the area directly adjacent to the central canal ependyma of the C4-T8 spinal cord in the cat.

    Science.gov (United States)

    Mouton, L J; Kerstens, L; Van der Want, J; Holstege, G

    1996-11-01

    In a previous study horseradish peroxidase (HRP) injections in the upper thoracic and cervical spinal cord revealed some faintly labeled small neurons at the dorsal border of the periaqueductal gray (PAG). The present light microscopic and electronmicroscopic tracing study describes the precise location of these dorsal border PAG-spinal neurons and their terminal organization. Wheat germ agglutinin-conjugated HRP (WGA-HRP) injections into cervical and upper thoracic spinal segments resulted in several hundreds of small retrogradely labeled neurons at the dorsal border of the ipsilateral caudal PAG. These neurons were not found after injections in more caudal segments. WGA-HRP injections in the dorsal border PAG region surprisingly resulted in anterogradely labeled fibers terminating in the area dorsally and laterally adjoining the central canal ependyma of the C4-T8 spinal cord. No anterogradely labeled fibers were found more caudal in the spinal cord. The labeled fibers found in the upper cervical cord were not located in the area immediately adjoining the ependymal layer of the central canal, but in the lateral part of laminae VI, VII and VIII and in area X bilaterally. Electronmicroscopic results of one case show that the dorsal border PAG-spinal neurons terminate in the neuropil of the subependymal area and in the vicinity of the basal membranes of capillaries located laterally to the central canal. The terminal profiles contain electron-lucent and densecored vesicles, suggesting a heterogeneity of possible transmitters. A striking observation was the lack of synaptic contacts, suggesting nonsynaptic release from the profiles. The function of the dorsal border PAG-spinal projection is unknown, but considering the termination pattern of the dorsal border PAG neurons on the capillaries the intriguing similarity between this projection system and the hypothalamohypophysial system is discussed.

  12. Successful management of aortic thrombi resulting in spinal cord infarction in a patient with antiphospholipid antibody syndrome and acute cholecystitis.

    Science.gov (United States)

    Izumi, Manabu; Teraoka, Shoko; Yamashita, Keisuke; Matsumoto, Kenji; Muronoi, Tomohiro; Izawa, Yoshimitsu; Yonekawa, Chikara; Ano, Masaki; Suzukawa, Masayuki

    2011-01-01

    A 74-year-old man with coronary artery disease was suffering from acute nonobstructive cholecystitis and was admitted to a nearby hospital. Dual antiplatelet (aspirin and ticlopidine) therapy was discontinued before preparation for surgical resection of the gall bladder. During his time in hospital he was aware of lumbar pain and weakness in both legs. He was transferred to our hospital for further evaluation and therapy. Diffuse intra-aortic thrombi were revealed by computed tomography with contrast media, and magnetic resonance imaging showed spinal cord infarction. However, computed tomography scans of the descending aorta obtained 4 months before admission exhibited no signs of atherosclerotic plaques or intra-aortic thrombi. Laboratory data suggest that antiphospholipid antibody syndrome might have caused these acute multiple intra-arterial thrombi. By restarting dual antiplatelet therapy and increasing the dose of heparin (from 10,000 IU/day to 15,000 IU/day) we successfully managed the patient's clinical condition and symptoms. It is important to understand that stopping antiplatelet therapy may rapidly grow thrombi in patients with a hypercoagulative state.

  13. Mass psychogenic illness: psychological predisposition and iatrogenic pseudo-vocal cord dysfunction and pseudo-reactive airways disease syndrome.

    Science.gov (United States)

    Staudenmayer, Herman; Christopher, Kent L; Repsher, Lawrence; Hill, Ronald H

    2011-06-01

    A multidisciplinary team assessed five patients who alleged chronic medically unexplained multiorgan system symptoms described by idiopathic environmental intolerance allegedly triggered by exposure to solvents used in membrane roofing repair work on an office building. The event precipitated an incident of mass psychogenic illness (MPI). Treating physicians diagnosed irritant-associated vocal cord dysfunction (IVCD) and reactive airways disease syndrome (RADS) resulting from exposure. The authors conducted medical, psychological, and industrial hygiene evaluations. Air monitoring data for total volatile organic compounds obtained during the 2-day exposure period, measurements of emissions during membrane roofing repair at a similar site, mathematical modeling of air contaminant concentrations, and injection of tracer gas into the incident building revealed exposure levels well below those doses anticipated to cause clinical symptoms. There was no objective medical evidence validating symptoms. Review of the medical records indicated that the video laryngoscopy data, pulmonary function tests, and medical examinations relied upon by the treating physicians were inconsistent with published criteria for IVCD and RADS. Psychological evaluation identified defensiveness and self-serving misrepresentations of exaggerated health concerns associated with somatization and malingering. Each case had personality traits associated with at least one personality disorder. Social histories identified premorbid life events and stressors associated with distress. This is the first study to assess psychological predisposition, social interaction among the plaintiffs, and iatrogenic reinforcement of beliefs by diagnoses of pseudo-disorders associated with patient misrepresentation of exaggerated health concerns in an incident of MPI.

  14. Evidence-based and heuristic approaches for customization of care in cardiometabolic syndrome after spinal cord injury.

    Science.gov (United States)

    Nash, Mark S; Cowan, Rachel E; Kressler, Jochen

    2012-09-01

    Component and coalesced health risks of the cardiometabolic syndrome (CMS) are commonly reported in persons with spinal cord injuries (SCIs). These CMS hazards are also co-morbid with physical deconditioning and elevated pro-atherogenic inflammatory cytokines, both of which are common after SCI and worsen the prognosis for all-cause cardiovascular disease. This article describes a systematic procedure for individualized CMS risk assessment after SCI, and emphasizes evidence-based and intuition-centered countermeasures to disease. A unified approach will propose therapeutic lifestyle intervention as a routine plan for aggressive primary prevention in this risk-susceptible population. Customization of dietary and exercise plans then follow, identifying shortfalls in diet and activity patterns, and ways in which these healthy lifestyles can be more substantially embraced by both stakeholders with SCI and their health care providers. In cases where lifestyle intervention utilizing diet and exercise is unsuccessful in countering risks, available pharmacotherapies and a preferred therapeutic agent are proposed according to authoritative standards. The over-arching purpose of the monograph is to create an operational framework in which existing evidence-based approaches or heuristic modeling becomes best practice. In this way persons with SCI can lead more active and healthy lives.

  15. Ascending central canal dilation and progressive ependymal disruption in a contusion model of rodent chronic spinal cord injury

    OpenAIRE

    Keirstead Hans S; Nistor Gabriel; Radojicic Milan

    2007-01-01

    Abstract Background Chronic spinal cord injury (SCI) can lead to an insidious decline in motor and sensory function in individuals even years after the initial injury and is accompanied by a slow and progressive cytoarchitectural destruction. At present, no pathological mechanisms satisfactorily explain the ongoing degeneration. Methods Adult female Sprague-Dawley rats were anesthetized laminectomized at T10 and received spinal cord contusion injuries with a force of 250 kilodynes using an In...

  16. Investigation of the spinal cord as a natural receptor antenna for incident electromagnetic waves and possible impact on the central nervous system.

    Science.gov (United States)

    Balaguru, Sevaiyan; Uppal, Rajan; Vaid, Ravinder Pal; Kumar, Balasubramaniam Preetham

    2012-06-01

    The effects of electromagnetic field (EMF) exposure on biological systems have been studied for many years, both as a source of medical therapy and also for potential health risks. In particular, the mechanisms of EMF absorption in the human or animal body is of medical/engineering interest, and modern modelling techniques, such as the Finite Difference Time Domain (FDTD), can be utilized to simulate the voltages and currents induced in different parts of the body. The simulation of one particular component, the spinal cord, is the focus of this article, and this study is motivated by the fact that the spinal cord can be modelled as a linear conducting structure, capable of generating a significant amount of voltage from incident EMF. In this article, we show, through a FDTD simulation analysis of an incoming electromagnetic field (EMF), that the spinal cord acts as a natural antenna, with frequency dependent induced electric voltage and current distribution. The multi-frequency (100-2400 MHz) simulation results show that peak voltage and current response is observed in the FM radio range around 100 MHz, with significant strength to potentially cause changes in the CNS. This work can contribute to the understanding of the mechanism behind EMF energy leakage into the CNS, and the possible contribution of the latter energy leakage towards the weakening of the blood brain barrier (BBB), whose degradation is associated with the progress of many diseases, including Acquired Immuno-Deficiency Syndrome (AIDS).

  17. Isolated Richter's syndrome in central nervous system: case report Sindrome de Richter isolada em sistema nervoso central: relato de caso

    Directory of Open Access Journals (Sweden)

    Lucilene S.R. Resende

    2005-06-01

    Full Text Available Diffuse large cell non Hodgkin's lymphoma associated with chronic lymphoid leukemia (CLL, or Richter's syndrome, is a rare and serious complication. Isolated Richter's syndrome in the central nervous system is very rare; only 12 cases have been reported. We describe a 74-year-old patient with diffuse large cell non Hodgkin's lymphoma in the right frontal region with the appearance of multiform glioblastoma.Linfoma não Hodgkin difuso de grandes células em paciente portador de leucemia linfóide crônica (LLC, ou síndrome de Richter, é complicação rara e grave nesta leucemia. Síndrome de Richter isolada no sistema nervoso central é muito rara, tendo sido encontrados apenas 12 casos descritos. Descrevemos paciente de 74 anos, que apresentou linfoma não Hodgkin difuso de grandes células em região frontal direita, simulando glioblastoma multiforme.

  18. Myelodysplastic syndrome complicated by central diabetes insipidus and cerebral salt wasting syndrome with peculiar change in magnetic resonance images.

    Science.gov (United States)

    Sano, Soichi; Yamagami, Keiko; Morikawa, Takashi; Yoshioka, Katsunobu

    2010-01-01

    Central diabetes insipidus (CDI) could occurs in patients with myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML), because of infiltration of leukemic cells into the neurohypophysis or some other reason and it is closely associated with abnormalities of chromosome 7. We report a case of MDS with abnormalities of chromosome 7, presenting as CDI followed by deterioration of polyuria and hyponatremia with a decreased extracellular fluid volume. Magnetic resonance imaging (MRI) revealed symmetrically enhanced lesions in the hypothalamus. Fludrocortisone treatment normalized his serum sodium level and cerebral salt wasting syndrome (CSWS) was suspected.

  19. Bilateral widespread mechanical pain sensitivity in carpal tunnel syndrome: evidence of central processing in unilateral neuropathy.

    Science.gov (United States)

    Fernández-de-las-Peñas, César; de la Llave-Rincón, Ana Isabel; Fernández-Carnero, Josué; Cuadrado, María Luz; Arendt-Nielsen, Lars; Pareja, Juan A

    2009-06-01

    The aim of this study was to investigate whether bilateral widespread pressure hypersensitivity exists in patients with unilateral carpal tunnel syndrome. A total of 20 females with carpal tunnel syndrome (aged 22-60 years), and 20 healthy matched females (aged 21-60 years old) were recruited. Pressure pain thresholds were assessed bilaterally over median, ulnar, and radial nerve trunks, the C5-C6 zygapophyseal joint, the carpal tunnel and the tibialis anterior muscle in a blinded design. The results showed that pressure pain threshold levels were significantly decreased bilaterally over the median, ulnar, and radial nerve trunks, the carpal tunnel, the C5-C6 zygapophyseal joint, and the tibialis anterior muscle in patients with unilateral carpal tunnel syndrome as compared to healthy controls (all, P < 0.001). Pressure pain threshold was negatively correlated to both hand pain intensity and duration of symptoms (all, P < 0.001). Our findings revealed bilateral widespread pressure hypersensitivity in subjects with carpal tunnel syndrome, which suggest that widespread central sensitization is involved in patients with unilateral carpal tunnel syndrome. The generalized decrease in pressure pain thresholds associated with pain intensity and duration of symptoms supports a role of the peripheral drive to initiate and maintain central sensitization. Nevertheless, both central and peripheral sensitization mechanisms are probably involved at the same time in carpal tunnel syndrome.

  20. Combined central diabetes insipidus and cerebral salt wasting syndrome in children.

    Science.gov (United States)

    Lin, Jainn-Jim; Lin, Kuang-Lin; Hsia, Shao-Hsuan; Wu, Chang-Teng; Wang, Huei-Shyong

    2009-02-01

    Central diabetes insipidus, a common consequence of acute central nervous system injury, causes hypernatremia; cerebral salt wasting syndrome can cause hyponatremia. The two conditions occurring simultaneous are rarely described in pediatric patients. Pediatric cases of combined diabetes insipidus and cerebral salt wasting after acute central nervous system injury between January 2000 and December 2007 were retrospectively reviewed, and clinical characteristics were systemically assessed. Sixteen patients, aged 3 months to 18 years, met study criteria: 11 girls and 5 boys. The most common etiologies were severe central nervous system infection (n = 7, 44%) and hypoxic-ischemic event (n = 4, 25%). In 15 patients, diabetes insipidus was diagnosed during the first 3 days after acute central nervous system injury. Onset of cerebral salt wasting syndrome occurred 2-8 days after the onset of diabetes insipidus. In terms of outcome, 13 patients died (81%) and 3 survived under vegetative status (19%). Central diabetes insipidus and cerebral salt wasting syndrome may occur after acute central nervous system injury. A combination of both may impede accurate diagnosis. Proper differential diagnoses are critical, because the treatment strategy for each entity is different.

  1. Non-stroke Central Neurologic Manifestations in Antiphospholipid Syndrome.

    Science.gov (United States)

    Yelnik, Cécile M; Kozora, Elizabeth; Appenzeller, Simone

    2016-02-01

    Thrombotic manifestations of antiphospholipid syndrome (APS) are well known, and various non-stroke neuro-psychiatric manifestations (NPMs) have also been consistently described, but their place in APS remains unclear. Some syndromes, such as migraine or cognitive dysfunction, are frequently described in APS, whereas others, like seizure, multiple sclerosis-like symptoms, transverse myelitis, movement disorders, or psychiatric symptoms, are rarely found. Overlap with other autoimmune diseases, in particular with systemic lupus erythematosus, the lack of large sample size prospective studies, and discrepancies in antiphospholipid antibody (aPL) determinations complicate the study of the relationship between those disorders and aPL/APS. This review article aimed to summarize updated data on pathophysiologic, epidemiologic, and radiologic findings about non-stroke NPM described in primary APS and aPL-positive patients without overlap of other autoimmune diseases.

  2. The Riley-Day syndrome. Familial dysautonomy, central autonomic dysfunction.

    Science.gov (United States)

    François, J

    1977-01-01

    The Riley-Day syndrome is characterized by a dysfunction of the autonomous nervous system, sensory disturbances, neurological disorders, psychical anomalies and important ophthalmological symptoms, such as absence of tears, corneal anaesthesia, keratinized conjunctiva and cornea; myosis after instillation of methacholine. The diagnosis is based on the absence of fungiform papillae of the tongue and the absence of reaction after intradermic injection of histamine. The inheritance is autosomal recessive. The disease results probably from an enzymatic insufficiency.

  3. Comparison of the metabolic parameters and androgen level of umbilical cord blood in newborns of mothers with polycystic ovary syndrome and controls

    Directory of Open Access Journals (Sweden)

    Ferdous Mehrabian

    2012-01-01

    Full Text Available Background: This study aimed to assess the metabolic parameters and androgen concentration in the cord blood of newborns of mothers with polycystic ovary syndrome (PCOS in comparison with controls. Materials and Methods: This cross-sectional study was conducted in 2010-2011 in Isfahan, Iran. Biochemical tests were conducted on 40 infants, born from singleton pregnancies in women with PCOS and an equal number of controls. Results: The mean weight gain during pregnancy was higher in women with PCOS than in controls (16.02 ± 4.39 vs. 9.10 ± 2.20 kg, respectively, P < 0.0001. The mean birth weight was lower in newborns of mothers with PCOS than in controls (2905.25 ± 415.59 vs. 3223.25 ± 425.02 vs. grams, respectively, P = 0.001. The mean testosterone was higher in cord blood of newborns of PCOS women than in controls (5.58 ± 3.20 vs. 2.28 ± 0.62 pg/ml, P < 0.0001. Triglycerides and LDL-C were lower in cord blood of newborns, born from PCOS women than in controls (P = 0.001. The birth weight of the newborns of PCOS mothers was negatively correlated to free testosterone of cord blood (R = -0. 26, P = 0.04. Conclusion: The metabolic aberration in PCOS might influence fetal birth weight and cord blood lipid profile. These disorders may be caused by an exposure to elevated testosterone level during fetal life. The offspring of PCOS women may be at higher risk for chronic diseases in later life. The clinical impact of our findings should be confirmed in future longitudinal studies.

  4. Combined central retinal artery and vein occlusion in Churg-Strauss syndrome

    DEFF Research Database (Denmark)

    Hamann, Steffen; Johansen, Sven; Hamann, Steffen Ellitsgaard

    2006-01-01

    PURPOSE: To describe a rare case of Churg-Strauss syndrome presenting with severe visual loss due to a combined central retinal vein and artery occlusion. METHODS: A 42-year old man with a medical history of asthma and blood hypereosinophilia developed a sudden loss of vision in his right eye. We...... describe the clinical features and evolution of the case after treatment. RESULTS: A combined occlusion of the central retinal artery and central retinal vein was diagnosed by the funduscopic appearance of retinal whitening, macular cherry-red spot, papilloedema, retinal haemorrhages in all four quadrants...... the vascular occlusion and experienced no visual improvement. CONCLUSION: Combined central retinal artery and vein occlusion can occur in Churg-Strauss syndrome. We suggest that regional vasculitis may be the pathological mechanism underlying the vascular occlusions observed in our case. The condition carries...

  5. Chiari malformation and central sleep apnea syndrome: efficacy of treatment with adaptive servo-ventilation

    Directory of Open Access Journals (Sweden)

    Jorge Marques do Vale

    2014-10-01

    Full Text Available The Chiari malformation type I (CM-I has been associated with sleep-disordered breathing, especially central sleep apnea syndrome. We report the case of a 44-year-old female with CM-I who was referred to our sleep laboratory for suspected sleep apnea. The patient had undergone decompressive surgery 3 years prior. An arterial blood gas analysis showed hypercapnia. Polysomnography showed a respiratory disturbance index of 108 events/h, and all were central apnea events. Treatment with adaptive servo-ventilation was initiated, and central apnea was resolved. This report demonstrates the efficacy of servo-ventilation in the treatment of central sleep apnea syndrome associated with alveolar hypoventilation in a CM-I patient with a history of decompressive surgery.

  6. Involvement of central serotonergic systems in dextromethorphan-induced behavioural syndrome in rats.

    Science.gov (United States)

    Gaikwad, R V; Gaonkar, R K; Jadhav, S A; Thorat, V M; Jadhav, J H; Balsara, J J

    2005-07-01

    Dextromethorphan, a noncompetitive blocker of the N-methyl-D-aspartate (NMDA) type of glutamate receptor, at 45, 60 and 75 mg/kg, ip doses induced a behavioural syndrome characterised by reciprocal forepaw treading, lateral head-weaving, hind-limb abduction and flat body posture. Such type of behavioural syndrome is induced by 8-hydroxy-2- (di-n-propylamino) tetralin (8-OH-DPAT) by directly stimulating the central postsynaptic 5-hydroxytryptamine (5-HT, serotonin) receptors of the 5-HT1A type. Pretreatment with buspirone (5, 10 mg/kg, ip) and l-propranolol (10, 20 mg/kg, ip) antagonised the behavioural syndrome induced by 8-OH-DPAT and dextromethorphan. Pretreatment with p-chlorophenylalanine (100 mg/kg/day x 4 days) antagonised the behavioural syndrome induced by dextromethorphan and dexfenfluramine but had no significant effect on 8-OH-DPAT induced behavioural syndrome. This indicates that dextromethorphan induces the behavioural syndrome by releasing 5-HT from serotonergic neurons with resultant activation of the postsynaptic 5-HT1A receptors by the released 5-HT. Pretreatment with fluoxetine (10 mg/kg, ip) significantly potentiated the behavioural syndrome induced by dextromethorphan and 5-hydroxytryptophan but significantly antagonised dexfenfluramine induced behavioural syndrome. This indicates that dextromethorphan releases 5-HT by a mechanism which differs from that of dexfenfluramine. Dextromethorphan may be releasing 5-HT by blocking the NMDA receptors and thereby counteracting the inhibitory influence of l-glutamate on 5-HT release.

  7. Central obesity measurements predict metabolic syndrome in a retrospective cohort study of postmenopausal women

    Directory of Open Access Journals (Sweden)

    Manuel Rosety-Rodríguez

    2013-12-01

    Full Text Available Introduction: The various diagnostic classifications in the literature concur as regards the important role of abdominal obesity in the onset and progression of metabolic syndrome. Accordingly, this study was aimed at clarifying whether central obesity measurements assessed by dual X-ray absorptiometry (DXA may predict metabolic syndrome in Spanish postmenopausal women. Material and methods: This historical cohort study included a total of 1326 postmenopausal women aged > 45 years old who had routinely undergone DXA to measure their bone mineral density between january 2006 and january 2011. The regions of interest (ROI envisaged in our study by using DXA were the lumbar regions L1-L4 and L4-L5. At the same time, they underwent a complete medical examination including personal medical history assessment, biochemical blood analysis, blood pressure measurement and anthropometrical evaluation. Metabolic syndrome was diagnosed attending to the criteria established by National Cholesterol Education Program Adult Treatment Panel III (NECP-ATP-III. Results: During the observation period, 537 women, representing 40.5% of the total studied, met the diagnostic criteria for metabolic syndrome. L1-L4 and L4-L5 abdominal fat mass determinations were associated with the development of metabolic syndrome in all regression models tested, showing an increasing gradient from the lowest to highest quintile. Conclusion: Central adiposity measurements assessed by DXA, especially L1-L4 region of interest, could be considered a powerful predictor of metabolic syndrome in postmenopausal women.

  8. Nociceptive transmission and modulation via P2X receptors in central pain syndrome.

    Science.gov (United States)

    Kuan, Yung-Hui; Shyu, Bai-Chuang

    2016-05-26

    Painful sensations are some of the most frequent complaints of patients who are admitted to local medical clinics. Persistent pain varies according to its causes, often resulting from local tissue damage or inflammation. Central somatosensory pathway lesions that are not adequately relieved can consequently cause central pain syndrome or central neuropathic pain. Research on the molecular mechanisms that underlie this pathogenesis is important for treating such pain. To date, evidence suggests the involvement of ion channels, including adenosine triphosphate (ATP)-gated cation channel P2X receptors, in central nervous system pain transmission and persistent modulation upon and following the occurrence of neuropathic pain. Several P2X receptor subtypes, including P2X2, P2X3, P2X4, and P2X7, have been shown to play diverse roles in the pathogenesis of central pain including the mediation of fast transmission in the peripheral nervous system and modulation of neuronal activity in the central nervous system. This review article highlights the role of the P2X family of ATP receptors in the pathogenesis of central neuropathic pain and pain transmission. We discuss basic research that may be translated to clinical application, suggesting that P2X receptors may be treatment targets for central pain syndrome.

  9. Sudden Unexplained Nocturnal Death Syndrome in Central China (Hubei)

    Science.gov (United States)

    Chen, Zhenglian; Mu, Jiao; Chen, Xinshan; Dong, Hongmei

    2016-01-01

    Abstract A retrospective study was conducted at Tongji Forensic Medical Center in Hubei (TFMCH) from 1999 to 2014. Forty-nine cases of sudden unexplained nocturnal death syndrome (SUNDS) were collected. The SUNDS rate was 1.0% in the total number of cases, in which an incidence was fluctuating over the years. Interestingly, April and January, and 3:00 to 6:00 am were the peak months and times of death. Among the decedents, farmers and migrant workers accounted for 67.3%. The syndrome predominantly attacked males in their 30s. One victim had sinus tachycardia. Thirteen victims (26.5%) were witnessed and had abnormal symptoms near death. Macroscopically, compared to sudden noncardiac deaths, the weights of brain, heart, and lungs had no statistical difference in SUNDS. Microscopically, the incidence of lung edema (45 cases, 91.8%) was significantly higher in SUNDS group than in the control group (27 cases, 55.1%). 82.9% of 35 SUNDS cases examined displayed minor histological anomalies of the cardiac conduction system (CCS), including mild or moderate fatty, fibrous or fibrofatty tissue replacement, insignificant stenosis of node artery, and punctate hemorrhage in the node area. These findings suggested that minor CCS abnormalities might be the substrates for some SUNDS deaths. Therefore, SUNDS victims might suffer ventricular fibrillation and acute cardiopulmonary failure before death. Further in-depth studies are needed to unveil the underlying mechanisms of SUNDS. PMID:26945374

  10. Catheter-directed Thrombolysis in Acute Superior Vena Cava Syndrome Caused by Central Venous Catheters.

    Science.gov (United States)

    Cui, Jie; Kawai, Tasuo; Irani, Zubin

    2015-01-01

    Indwelling central venous catheters have been reported to increase the risk of superior venous cava (SVC) syndrome. This case report describes the development of acute SVC syndrome in a 28-year-old woman with end-stage renal disease implanted with a left-side hemodialysis reliable outflow graft and a right-side double lumen hemodialysis catheter via internal jugular veins. Her symptoms were not alleviated after catheter removal and systemic anticoagulation therapy. She was eventually treated with catheter-directed thrombolysis and a predischarge computer tomographic venogram on postthrombolytic procedure day 7 showed patent central veins and patient remained asymptomatic. This case demonstrates that catheter-directed thrombolysis can be safely employed to treat refractory catheter-induced acute SVC syndrome in end-stage renal disease patients.

  11. The primary Maxillary Central Incisor in the Solitary Median Maxillary Central Incisor syndrome

    DEFF Research Database (Denmark)

    Kjaer, I; Balslev-Olesen, M

    2012-01-01

    Solitary Median Maxillary Central Incisor (SMMCI) is a developmental anomaly in the permanent dentition with one single central incisor in the maxilla, positioned exactly in the midline. This condition has been associated with extra- and intraoral malformations in the frontonasal segment of the c...

  12. Cerebral salt wasting syndrome in children with acute central nervous system injury.

    Science.gov (United States)

    Jiménez, Raquel; Casado-Flores, Juan; Nieto, Monserrat; García-Teresa, María Angeles

    2006-10-01

    The purpose of this investigation was to describe the causes, clinical pattern, and treatment of cerebral salt wasting syndrome in children with acute central nervous system injury. This retrospective study focused on patientscerebral salt wasting syndrome, over a period of 7 years, in the pediatric intensive care unit of a tertiary care hospital. Selection criteria included evidence of hyponatremia (serum sodium120 mEq/L), and volume depletion. Fourteen patients were identified with cerebral salt wasting syndrome, 12 after a neurosurgical procedure (8 brain tumor, 4 hydrocephalus) and 2 after severe brain trauma. In 11 patients the cerebral salt wasting syndrome was diagnosed during the first 48 hours of admission. Prevalence of cerebral salt wasting syndrome in neurosurgical children was 11.3/1000 surgical procedures. The minimum sodium was 122+/-7 mEq/L, the maximum urine osmolarity 644+/-59 mOsm/kgH2O. The maximum sodium supply was 1 mEq/kg/h (range, 0.1-2.4). The mean duration of cerebral salt wasting syndrome was 6+/-5 days (range 1-9). In conclusion, cerebral salt wasting syndrome can complicate the postoperative course of children with brain injury; it is frequently present after surgery for brain tumors and hydrocephalus and in patients with severe head trauma. Close monitoring of salt and fluid balance is essential to prevent severe neurologic and hemodynamic complications.

  13. Central nervous system syndromes in solid organ transplant recipients.

    Science.gov (United States)

    Wright, Alissa J; Fishman, Jay A

    2014-10-01

    Solid organ transplant recipients have a high incidence of central nervous system (CNS) complications, including both focal and diffuse neurologic deficits. In the immunocompromised host, the initial clinical evaluation must focus on both life-threatening CNS infections and vascular or anatomic lesions. The clinical signs and symptoms of CNS processes are modified by the immunosuppression required to prevent graft rejection. In this population, these etiologies often coexist with drug toxicities and metabolic abnormalities that complicate the development of a specific approach to clinical management. This review assesses the multiple risk factors for CNS processes in solid organ transplant recipients and establishes a timeline to assist in the evaluation and management of these complex patients.

  14. Periodic fever syndromes in Eastern and Central European countries: results of a pediatric multinational survey

    Directory of Open Access Journals (Sweden)

    Stefan Mariana

    2010-12-01

    Full Text Available Abstract Objective To analyze the prevalence of diagnosed and suspected autoinflammatory diseases in Eastern and Central European (ECE countries, with a particular interest on the diagnostic facilities in these countries. Methods Two different strategies were used to collect data on patients with periodic fever syndromes from ECE countries- the Eurofever survey and collection of data with the structured questionnaire. Results Data from 35 centers in 14 ECE countries were collected. All together there were 11 patients reported with genetically confirmed familial Mediterranean fever (FMF, 14 with mevalonate-kinase deficiency (MKD, 11 with tumor necrosis factor receptor associated periodic syndrome (TRAPS and 4 with chronic infantile neurological cutaneous and articular syndrome (CINCA. Significantly higher numbers were reported for suspected cases which were not genetically tested. All together there were 49 suspected FMF patients reported, 24 MKD, 16 TRAPS, 7 CINCA and 2 suspected Muckle-Wells syndrome (MWS patients. Conclusions The number of genetically confirmed patients with periodic fever syndromes in ECE countries is very low. In order to identify more patients in the future, it is important to organize educational programs for increasing the knowledge on these diseases and to establish a network for genetic testing of periodic fever syndromes in ECE countries.

  15. MRI morphometric characterisation of the paediatric cervical spine and spinal cord in children with MPS IVA (Morquio-Brailsford syndrome).

    Science.gov (United States)

    Solanki, Guirish A; Lo, William B; Hendriksz, Christian J

    2013-03-01

    Nearly all children with MPS IVA develop skeletal deformities affecting the spine. At the atlanto-axial spine, odontoid hypoplasia occurs. GAG deposition around the dens, leads to peri-odontoid infiltration. Transverse/alar ligament incompetence causes instability. Atlanto-axial instability is associated with cord compression and myelopathy, leading to major morbidity and mortality. Intervention is often required. Does the presence of widened bullet shaped vertebra in platyspondily encroach on the spinal canal and cause spinal stenosis in MPS IVA? So far, there have been no standardised morphometric measurements of the paediatric MPS IVA cervical spine to evaluate whether there is pre-existing spinal stenosis predisposing to compressive myelopathy or whether this is purely an acquired process secondary to instability and compression. This study provides the first radiological quantitative analysis of the cervical spine and spinal cord in a series of affected children. MRI morphometry indicates that the MPS IVA spine is narrower at C1-2 level giving an inverted funnel shape. There is no evidence of a reduction in the Torg ratio (canal-body ratio) in the cervical spine. The spinal canal does not exceed 11 mm at any level, significantly smaller than normal historical cohorts (14 mm). The sagittal diameter and axial surface area of both spinal canal and cord are reduced. C1-2 level cord compression was evident in the canal-cord ratio but the Torg ratio was not predictive of cord compression. In MPS IVA the reduction in the space available for the cord (SAC) is multifactorial rather than due to congenital spinal stenosis.

  16. Spinal Cord Stimulation

    DEFF Research Database (Denmark)

    Meier, Kaare

    2014-01-01

    pain after failed back surgery syndrome (FBSS)(4), pain due to peripheral nerve injury, stump pain(5), peripheral vascular disease(6) and diabetic neuropathy(7,8); whereas phantom pain(9), postherpetic neuralgia(10), chronic visceral pain(11), and pain after partial spinal cord injury(12) remain more...

  17. [Molecular genetics of familial tumour syndromes of the central nervous system].

    Science.gov (United States)

    Murnyák, Balázs; Szepesi, Rita; Hortobágyi, Tibor

    2015-02-01

    Although most of the central nervous system tumours are sporadic, rarely they are associated with familial tumour syndromes. These disorders usually present with an autosomal dominant inheritance and neoplasia develops at younger age than in sporadic cases. Most of these tumours are bilateral, multiplex or multifocal. The causative mutations occur in genes involved in cell cycle regulation, cell growth, differentiation and DNA repair. Studying these hereditary cancer predisposition syndromes associated with nervous system tumours can facilitate the deeper understanding of the molecular background of sporadic tumours and the development of novel therapeutic agents. This review is an update on hereditary tumour syndromes with nervous system involvement with emphasis on molecular genetic characteristics and their clinical implications.

  18. Central Retinal Artery Occlusion in a Patient with Metabolic Syndrome X

    Directory of Open Access Journals (Sweden)

    Sonja Predrag Cekić

    2010-01-01

    Full Text Available Purpose: To report a case of central retinal artery occlusion (CRAO in a patient with metabolic syndrome X. Case Report: A 64 year-old-man presented with abrupt, painless, and severe loss of vision in his left eye. Indirect ophthalmoscopy disclosed signs compatible with CRAO and laboratory investigations revealed erythrocyte sedimentation rate of 74 mm/h, C-reactive protein (CRP level of 21 mg/l, hyperglycemia, hyperuricemia, hypertriglyceridemia and hypercholesterolemia. Fluorescein angiography and immunological studies excluded other systemic disorders. The patient met the full criteria of the National Cholesterol Education Program for metabolic syndrome X. Conclusion: In addition to different vascular complications such as stroke, and cardiovascular disease, metabolic syndrome X may be associated with retinal vascular occlusions.

  19. Ramsay Hunt Syndrome Associated with Central Nervous System Involvement in an Adult

    Directory of Open Access Journals (Sweden)

    Tommy L. H. Chan

    2016-01-01

    Full Text Available Ramsay Hunt syndrome associated with varicella zoster virus reactivation affecting the central nervous system is rare. We describe a 55-year-old diabetic female who presented with gait ataxia, right peripheral facial palsy, and painful vesicular lesions involving her right ear. Later, she developed dysmetria, fluctuating diplopia, and dysarthria. Varicella zoster virus was detected in the cerebrospinal fluid by polymerase chain reaction. She was diagnosed with Ramsay Hunt syndrome associated with spread to the central nervous system. Her facial palsy completely resolved within 48 hours of treatment with intravenous acyclovir 10 mg/kg every 8 hours. However, cerebellar symptoms did not improve until a tapering course of steroid therapy was initiated.

  20. PHOX2B mutations in three Chinese patients with congenital central hypoventilation syndrome

    Institute of Scientific and Technical Information of China (English)

    Siu-Fong June Or; Ming-for Tony Tong; Fai-Man Ivan Lo; Chi-Wai Law; Ting-Yat Miu; Delphine Trochet; Tak-Sum Stephen Lam

    2006-01-01

    @@ Congenital central hypoventilation syndrome(CCHS, OMIM #209880) is a rare autosomal dominant disorder of the autonomic nervous system(ANS) characterized by an abnormal autonomic ventilatory response to progressive hypercarbia and sustained hypoxemia. Patients typically present in the newborn period with hypoventilation or apnea asleep, awake, or both, without any associated cardiac, pulmonary, neuromuscular or brainstem lesions. Rarely, some patients may present at a later age and are diagnosed to have late onset central hypoventilation syndrome (LOCHS).1 Other features of ANS dysfunction such as feeding difficulty due to oesophageal dysmotility, severe constipation in the absence of Hirschsprung disease, poor regulation of basal body temperature, episodes of profuse sweating, pupillary and ocular abnormalities,decreased beat-to-beat variability of heart rate,attenuated response of heart rate to exercise,abnormal fluctuation of blood pressure, decreased perception to pain, and decreased perception to anxiety may be variably present but not essential for diagnosis.

  1. The Rare Painful Phenomena - Chronic Paroxysmal Hemicrania-tic Syndrome as a Clinically Isolated Syndrome of the Central Nervous System.

    Science.gov (United States)

    Ljubisavljevic, Srdjan; Prazic, Ana; Lazarevic, Miodrag; Stojanov, Dragan; Savic, Dejan; Vojinovic, Slobadan

    2017-02-01

    The association of paroxysmal hemicrania with trigeminal neuralgia (TN) has been described and called paroxysmal hemicrania-tic syndrome (PH-tic). We report the case of a patient diagnosed as having chronic PH-tic (CPH-tic) syndrome as a clinically isolated syndrome of the central nervous system (CNS) (CIS).A forty year old woman was admitted to our hospital suffering from right facial pain for the last 2 years. The attacks were paroxysmal, neuralgiform, consisting of throb-like sensations, which developed spontaneously or were triggered by different stimuli in right facial (maxilar and mandibular) areas. Parallel with those, she felt a throbbing orbital and frontal pain with homolateral autonomic symptoms such as conjunctival injection, lacrimation, and the feeling that the ear on the same side was full. This pain lasted most often between 15 and 20 minutes. Beyond hemifacial hypoesthesia in the region of right maxilar and mandibular nerve, the other neurological finding was normal. Magnetic resonance imaging (MRI) study showed a T2-weighted multiple hyperintense paraventricular lesion and hyperintense lesion in the right trigeminal main sensory nucleus and root inlet, all of them being hypointense on T1-weighted image. All of these lesions were hypointense in gadolinium-enhanced T1-weighted images. Neurophysiological studies of trigeminal nerve (somatosensory evoked potentials and blink reflex) correlated with MRI described lesions. The patient's pain bouts were improved immediately after treatment with indomethacin, and were completely relieved with lamotrigine for a longer period. According to the actual McDonald's criteria, clinical state was defined as CIS which was clinically presented by CPH-tic syndrome.Even though it is a clinical rarity and its etiology is usually idiopathic, CPH-tic syndrome can also be symptomatic. When dealing with symptomatic cases, like the one described here, when causal therapy is not possible due to the nature of the primary

  2. Sonography of the umbilical cord.

    Science.gov (United States)

    Dudiak, C M; Salomon, C G; Posniak, H V; Olson, M C; Flisak, M E

    1995-09-01

    Knowledge of the development, normal sonographic appearance, and potential abnormalities of the umbilical cord is important in fetal assessment. The umbilical cord can be visualized throughout most of gestation and is detectable sonographically soon after visualization of the fetal pole. The normal umbilical cord is 50-60 cm long and may coil as many as 40 times, usually to the left. Abnormalities in umbilical cord size, degree of coiling, attachment, and position can have important implications for the outcome of the pregnancy. Structural abnormalities of the umbilical cord such as single umbilical artery, knots, cysts, and tumors may be associated with fetal distress or malformations. Color Doppler ultrasound (US) is useful in the identification and evaluation of structural abnormalities of the cord. By allowing measurement of blood flow velocity in the umbilical artery, duplex Doppler US may provide additional information in the evaluation of intrauterine growth retardation and twin-twin transfusion syndrome.

  3. Two uncommon manifestations of leptospirosis:Sweet’s syndrome and central nervous system vasculitis

    Institute of Scientific and Technical Information of China (English)

    Peter George

    2011-01-01

    To leptospirosis is the commonest spirocheatal infection in the tropical and temperate countries of Indian sub-continent and Africa and the most common zoonosis worldwide. The protean manifestation of this infectious disease is a challenge for practising clinicians across the world. In poor developing countries, at most clinical suspicion it is essential in the diagnosis of this disease. In this report, we are able to document two uncommon manifestations of leptospirosis, namely Sweet’s syndrome and central nervous system vasculitis.

  4. Traumatic cervical cord transection without facet dislocations--a proposal of combined hyperflexion-hyperextension mechanism: a case report.

    Science.gov (United States)

    Cha, Yoo-Hyun; Cho, Tai-Hyoung; Suh, Jung-Keun

    2010-08-01

    A patient is presented with a cervical spinal cord transection which occurred after a motor vehicle accident in which the air bag deployed and the seat belt was not in use. The patient had complete quadriplegia below the C5 level and his imaging study showed cervical cord transection at the level of the C5/6 disc space with C5, C6 vertebral bodies and laminar fractures. He underwent a C5 laminectomy and a C4-7 posterior fusion with lateral mass screw fixation. Previous reports have described central cord syndromes occurring in hyperextension injuries, but in adults, acute spinal cord transections have only developed after fracture-dislocations of the spine. A case involving a post-traumatic spinal cord transection without any evidence of radiologic facet dislocations is reported. Also, we propose a combined hyperflexion-hyperextension mechanism to explain this type of injury.

  5. Viabilidade de células do sistema nervoso central fetal no tratamento da lesão medular em ratos Viability of fetal central nervous system cells in the treatment of spinal cord injury in rats

    Directory of Open Access Journals (Sweden)

    Alexandre Fogaça Cristante

    2010-01-01

    Full Text Available OBJETIVOS: Propor um modelo experimental de transplante de células do sistema nervoso fetal de ratos Wistar para o sítio de lesão medular de ratos adultos que permitisse sua sobrevivência e integração para possibilitar protocolos de pesquisa que identificarão outros fatores de regeneração e recuperação funcional pós trauma raquimedular. MÉTODOS: Vinte ratos adultos foram submetidos a laminectomia, e lesão de 5mm de hemimedula realizada com auxílio de microscópio óptico. Quinze deste ratos tiveram seu sítio de lesão medular transplantado com células do sistema nervoso central de fetos de rato; os ratos foram monitorados por 2 dias e tiveram sua coluna vertebral extraída para análise histológica. RESULTADOS: Evidenciou-se que em 60% dos casos as células transplantadas permaneciam viáveis no sítio da lesão e que a reação inflamatória no grupo transplantado era sempre maior que no grupo controle. CONCLUSÃO: O presente trabalho demonstrou a possibilidade de contar com o modelo de pesquisa para transplante de células fetais que permanecem viáveis 2 dias após seu implante.OBJECTIVE: To propose an experimental model for transplantation of fetal cells from the nervous system of Wistar rats to the site of spinal cord injury in adult rats, to enable their survival and integration for research protocols that identify other factors of regeneration and functional recovery following spinal cord trauma. METHODS: Twenty adult rats were submitted to laminectomy and a 5mm incision was made, using an optical microscope, In fifteen of these rats, the site of the spinal cord lesion was transplanted with cells from the fetal rat central nervous system; the rats were monitored for two days, then the spinal cord was removed for histological analysis. RESULTS: In 60% of cases, the transplanted cells remained viable in the site of the lesion; the inflammatory response in the transplanted group was always greater than in the control group

  6. Bovine Reissner's fiber (RF) and the central canal of the spinal cord: an immunocytochemical study using a set of monoclonal antibodies against the RF-glycoproteins.

    Science.gov (United States)

    Pérez, J; Garrido, O; Cifuentes, M; Alonso, F J; Estivill-Torrús, G; Eller, G; Nualart, F; López-Avalos, M D; Fernández-Llebrez, P; Rodríguez, E M

    1996-10-01

    The subcommissural organ secretes N-linked complex-type glycoproteins into the cerebrospinal fluid. These glycoproteins condense to form Reissner's fiber (RF), which extends along the fourth ventricle and central canal of the spinal cord. A set of three monoclonal antibodies (Mabs 3E6, 3B1, and 2A5) has been obtained using these glycoproteins as immunogens. Competitive and sandwich enzyme-linked immunoassay methods have demonstrated that the three monoclonal antibodies are directed against different epitopes, and that there is no competition among them for their binding to glycoproteins of RF. Mab 3E6 displays immunoblotting properties that are similar to those of a polyclonal antibody against the pool of glycoproteins from RF, but that are different from those of Mabs 3B1 and 2A5. All three antibodies immunostain the bovine subcommissural organ and RF. A population of ependymal cells is stained by the polyclonal antibody, and Mabs 2A5 and 3E6, but not by Mab 3B1. The material present in a population of ependymal cells of the central canal, and the glycoproteins secreted by the subcommissural organ thus probably have partial chemical identity. Some evidence suggests that the immunoreactive ependymal cells are secretory cells. The luminal surface of the central canal is coated by a thin layer of material with immunocytochemical characteristics different from those of the ependymal cells; such a coat may correspond to material released from RF.

  7. Editorial review: an update on central sensitivity syndromes and the issues of nosology and psychobiology.

    Science.gov (United States)

    Yunus, Muhammad B

    2015-01-01

    Central sensitization (CS), simply defined as an amplified response of the central nervous system to peripheral input, is a concept of great importance in clinical medicine. It has helped to explain aspects of the pathophysiology of common diseases, e.g. fibromyalgia syndrome (FMS), irritable bowel syndrome, vulvodynia, headaches, chronic pelvic pain and other overlapping conditions (collectively called central sensitivity syndromes, or CSS). It also applies to pain of complex regional pain syndrome, osteoarthritis (OA), rheumatoid arthritis (RA) and post-operative pain. The pathology-pain gap in CSS is readily explained by CS. Many FMS and other CSS patients have peripheral pathology, e.g. nociceptive areas in the muscles, arthritis, small fiber neuropathy and inflammation. Pro-inflammatory cytokines are elevated in some patients. Identification of CS in patients with structural pathology, e.g. OA and RA, has helped to explain why not all patients benefit from nonsteroidal anti-inflammatory drugs or joint replacement surgery, and require therapy directed at CS. Glial cells are important in pain processing. Remarkable advances have been achieved in neuroimaging, including visualization of grey matter and white matter, not only during provoked pain but also pain at rest. Based on CS mechanisms, targeted individual therapy may now be possible. Appropriate nosology is important particularly for effective patient care. Dichotomy of neurochemical-structural ("functional") and structural ("organic") pathology should be abandoned; many patients have both. Psychobiology is also biology. Patient-blaming terms like somatization, somatizer and catastrophizing should be avoided. For therapy, both pharmacological and non- pharmacological approaches are important, including recognition of subgroups and person/patient-centered care.

  8. Immediate Return to Ambulation and Improved Functional Capacity for Rehabilitation in Complex Regional Pain Syndrome following Early Implantation of a Spinal Cord Stimulation System

    Directory of Open Access Journals (Sweden)

    Brandon Jesse Goff

    2014-01-01

    Full Text Available Complex regional pain syndrome (CRPS is a neuropathic pain condition that is characterized by vasomotor, sensory, sudomotor, and motor symptoms. Spinal cord stimulation (SCS has been successfully utilized for the treatment of pain refractory to conventional therapies. We present a case of a previously highly functioning 54-year-old female who developed a rarely reported case of idiopathic CRPS of the right ankle which spontaneously occurred four months after an uncomplicated anterior cervical disc fusion. This condition resulted in severe pain and functional impairment that was unresponsive to pharmacological management. The patient’s rehabilitation was severely stymied by her excruciating pain. However, with the initiation of spinal cord stimulation, her pain was adequately controlled allowing for progression to full unassisted ambulation, advancing functional capacity, and improving quality of life. This case report supports the concept that rapid progression to neuromodulation, rather than delays that occur due to attempts at serial sympathetic blocks, may better control symptoms leading allowing for a more meaningful recovery.

  9. Central retinal artery occlusion following laser treatment for ocular ischemic aortic arch syndrome

    Directory of Open Access Journals (Sweden)

    Shah, Payal J.

    2015-12-01

    Full Text Available Objective: Ocular ischemic syndrome is a rare blinding condition generally caused by disease of the carotid artery. We describe a 69-year-old female with a 50 pack-year smoking history with aortic arch syndrome causing bilateral ocular ischemic syndrome. Methods: The patient presented with progressive visual loss and temple pain. Slit lamp biomicroscopy revealed bilateral iris neovascularization. This finding prompted a cardiovascular work up. Panretinal photocoagulation with retrobulbar block was performed in the right eye. Results: A temporal artery biopsy was negative. The carotid duplex sound showed only a 1–39% stenosis. MRA revealed a more proximal occlusion of the aortic branch for which she underwent subclavian carotid bypass surgery. At the one month follow up, the right eye suffered profound vision loss secondary to a central retinal artery occlusion. Conclusion: Ocular neovascularization may be one of the clinical manifestations of aortic arch syndrome. This case also illustrates the limitations of relying solely on carotid duplex ultrasound testing. We caution against overly aggressive panretinal photocoagulation utilizing retrobulbar anesthesia.

  10. Cytomegalovirus upregulates expression of CCR5 in central memory cord blood mononuclear cells, which may facilitate in utero HIV type 1 transmission.

    Science.gov (United States)

    Johnson, Erica L; Howard, Chanie L; Thurman, Joy; Pontiff, Kyle; Johnson, Elan S; Chakraborty, Rana

    2015-01-15

    Administration of combination antiretroviral therapy to human immunodeficiency virus type 1 (HIV-1)-infected pregnant women significantly reduces vertical transmission. In contrast, maternal co-opportunistic infection with primary or reactivated cytomegalovirus (CMV) or other pathogens may facilitate in utero transmission of HIV-1 by activation of cord blood mononuclear cells (CBMCs). Here we examine the targets and mechanisms that affect fetal susceptibility to HIV-1 in utero. Using flow cytometry, we demonstrate that the fraction of CD4(+)CD45RO(+) and CD4(+)CCR5(+) CBMCs is minimal, which may account for the low level of in utero HIV-1 transmission. Unstimulated CD4(+) CBMCs that lack CCR5/CD45RO showed reduced levels of HIV-1 infection. However, upon in vitro stimulation with CMV, CBMCs undergo increased proliferation to upregulate the fraction of T central memory cells and expression of CCR5, which enhances susceptibility to HIV-1 infection in vitro. These data suggest that activation induced by CMV in vivo may alter CCR5 expression in CD4(+) T central memory cells to promote in utero transmission of HIV-1.

  11. Central Diabetes Insipidus and Cisplatin-Induced Renal Salt Wasting Syndrome: A Challenging Combination.

    Science.gov (United States)

    Cortina, Gerard; Hansford, Jordan R; Duke, Trevor

    2016-05-01

    We describe a 2-year-old female with a suprasellar primitive neuroectodermal tumor and central diabetes insipidus (DI) who developed polyuria with natriuresis and subsequent hyponatremia 36 hr after cisplatin administration. The marked urinary losses of sodium in combination with a negative sodium balance led to the diagnosis of cisplatin-induced renal salt wasting syndrome (RSWS). The subsequent clinical management is very challenging. Four weeks later she was discharged from ICU without neurological sequela. The combination of cisplatin-induced RSWS with DI can be confusing and needs careful clinical assessment as inaccurate diagnosis and management can result in increased neurological injury.

  12. Potential Central Nervous System Involvement in Sudden Unexpected Infant Deaths and the Sudden Infant Death Syndrome.

    Science.gov (United States)

    Thach, Bradley T

    2015-07-01

    Sudden unexpected infant death (SUID) in infancy which includes Sudden Infant Death Syndrome (SIDS) is the commonest diagnosed cause of death in the United States for infants 1 month to 1 year of age. Central nervous system mechanisms likely contribute to many of these deaths. We discuss some of these including seizure disorders, prolonged breath holding, arousal from sleep and its habituation, laryngeal reflex apnea potentiated by upper airway infection, and failure of brainstem-mediated autoresuscitation. In the conclusions section, we speculate how lives saved through back sleeping might result in later developmental problems in certain infants who otherwise might have died while sleeping prone.

  13. Cord Blood

    Directory of Open Access Journals (Sweden)

    Saeed Abroun

    2014-05-01

    Full Text Available   Stem cells are naïve or master cells. This means they can transform into special 200 cell types as needed by body, and each of these cells has just one function. Stem cells are found in many parts of the human body, although some sources have richer concentrations than others. Some excellent sources of stem cells, such as bone marrow, peripheral blood, cord blood, other tissue stem cells and human embryos, which last one are controversial and their use can be illegal in some countries. Cord blood is a sample of blood taken from a newborn baby's umbilical cord. It is a rich source of stem cells, umbilical cord blood and tissue are collected from material that normally has no use following a child’s birth. Umbilical cord blood and tissue cells are rich sources of stem cells, which have been used in the treatment of over 80 diseases including leukemia, lymphoma and anemia as bone marrow stem cell potency.  The most common disease category has been leukemia. The next largest group is inherited diseases. Patients with lymphoma, myelodysplasia and severe aplastic anemia have also been successfully transplanted with cord blood. Cord blood is obtained by syringing out the placenta through the umbilical cord at the time of childbirth, after the cord has been detached from the newborn. Collecting stem cells from umbilical blood and tissue is ethical, pain-free, safe and simple. When they are needed to treat your child later in life, there will be no rejection or incompatibility issues, as the procedure will be using their own cells. In contrast, stem cells from donors do have these potential problems. By consider about cord blood potency, cord blood banks (familial or public were established. In IRAN, four cord blood banks has activity, Shariati BMT center cord blood bank, Royan familial cord blood banks, Royan public cord blood banks and Iranian Blood Transfusion Organ cord blood banks. Despite 50,000 sample which storage in these banks, but the

  14. Clinical course, management and in-hospital outcomes of acute coronary syndrome in Central Asian women

    Directory of Open Access Journals (Sweden)

    Ravshanbek Kurbanov

    2011-03-01

    Full Text Available The paper describes features history, risk factors and hospital management of acute coronary syndrome (ACS in women of the Central Asia (829 ACS patients are included, and also comparison of hospital outcomes of ACS in groups of men and women.ACS in women in the Central Asia comprises features as more non Q-wave myocardial infarction frequency, smaller adherence to treatment of dyslipidemia, more obesity degrees. Women arrive to hospital average in 4 hours after men; have more percent of the atypical form. In-hospital outcomes in men and women significantly did not differ because of general tendency to late arriving to the hospital and small quantity of revascularization procedures. The reasons of differences between men and women in the region are mostly connected to social sphere (late arriving, non-treated dyslipidemia than to physiological peculiarities.

  15. Central poststroke pain: somatosensory abnormalities and the presence of associated myofascial pain syndrome

    Directory of Open Access Journals (Sweden)

    de Oliveira Rogério Adas

    2012-09-01

    Full Text Available Abstract Background Central post-stroke pain (CPSP is a neuropathic pain syndrome associated with somatosensory abnormalities due to central nervous system lesion following a cerebrovascular insult. Post-stroke pain (PSP refers to a broader range of clinical conditions leading to pain after stroke, but not restricted to CPSP, including other types of pain such as myofascial pain syndrome (MPS, painful shoulder, lumbar and dorsal pain, complex regional pain syndrome, and spasticity-related pain. Despite its recognition as part of the general PSP diagnostic possibilities, the prevalence of MPS has never been characterized in patients with CPSP patients. We performed a cross-sectional standardized clinical and radiological evaluation of patients with definite CPSP in order to assess the presence of other non-neuropathic pain syndromes, and in particular, the role of myofascial pain syndrome in these patients. Methods CPSP patients underwent a standardized sensory and motor neurological evaluation, and were classified according to stroke mechanism, neurological deficits, presence and profile of MPS. The Visual Analogic Scale (VAS, McGill Pain Questionnaire (MPQ, and Beck Depression Scale (BDS were filled out by all participants. Results Forty CPSP patients were included. Thirty-six (90.0% had one single ischemic stroke. Pain presented during the first three months after stroke in 75.0%. Median pain intensity was 10 (5 to 10. There was no difference in pain intensity among the different lesion site groups. Neuropathic pain was continuous-ongoing in 34 (85.0% patients and intermittent in the remainder. Burning was the most common descriptor (70%. Main aggravating factors were contact to cold (62.5%. Thermo-sensory abnormalities were universal. MPS was diagnosed in 27 (67.5% patients and was more common in the supratentorial extra-thalamic group (P Conclusions The presence of MPS is not an exception after stroke and may present in association with CPSP

  16. Central nervous system

    Science.gov (United States)

    The central nervous system is composed of the brain and spinal cord. Your brain and spinal cord serve as the main "processing center" for your entire nervous system. They control all the workings of your body.

  17. Ependyma of the central canal of the rat spinal cord: a light and transmission electron microscopic study.

    OpenAIRE

    Bruni, J.E.; Reddy, K

    1987-01-01

    Ependymal cells of the rat central canal were examined with a view to identifying features that distinguish them regionally and from their counterparts elsewhere in the ventricular system. The results revealed that the lining consisted for the most part of a pseudostratified layer of uniformly organised cuboidal to columnar ependymal cells present in largest numbers in lumbar and sacral segments and in the conus. Two cell variants were identified on the basis of the presence or absence of a r...

  18. Myoclonic status and central fever in Angelman syndrome due to paternal uniparental disomy.

    Science.gov (United States)

    Nicita, Francesco; Garone, Giacomo; Papetti, Laura; Consoli, Federica; Magliozzi, Monia; De Luca, Alessandro; Spalice, Alberto

    2015-01-01

    Myoclonic status in nonprogressive encephalopathy (MSNE) is an early-onset, drug-resistant epileptic syndrome characterized by occurrence of continuous diffuse epileptiform abnormalities, associated with positive and/or negative phenomena and accompanied by transient and recurring motor, cognitive, and behavioral impairment. MSNE has been reported in Angelman syndrome (AS) secondary to 15q11-13 deletions or UBE3A mutations but not to paternal uniparental disomy (UPD). We describe the case of a male patient with AS caused by UPD who developed a myoclonic status (MS) associated with long-lasting fever of central origin, both promptly regressed with introduction of levetiracetam. Only three descriptions of thermal dysregulation in AS exist, and none of the previously reported cases were associated with MS or with UPD. Association of MS and central fever expands the spectrum of epileptic and non-epileptic features in UPD-related AS and provides a further evidence of hypothalamus involvement in the pathogenesis of this neurodevelopmental disorder.

  19. Prospective double-blind preoperative pain clinic screening before microsurgical denervation of the spermatic cord in patients with testicular pain syndrome.

    Science.gov (United States)

    Oomen, Robert J A; Witjens, Annemijke C; van Wijck, Albert J M; Grobbee, Diederik E; Lock, Tycho M T W

    2014-09-01

    Testicular pain syndrome (TPS), defined as an intermittent or constant pain in one or both testicles for at least 3 months, resulting in significant reduction of daily activities, is common. Microsurgical denervation of the spermatic cord (MDSC) has been suggested as an effective treatment option. The study population comprised 180 TPS patients admitted to our outpatient urology clinic between 1999 and 2011. On 3 different occasions, patients were offered a double-blind, placebo-controlled temporary blockade of the spermatic cord. A single blockade consisted of 10 mL 2% lidocaine, 10 mL 0.25% bupivacaine, or 10 mL 0.9% sodium chloride. If the results of these blockades were positive, MDSC was offered. All MDSCs were performed by a single urologist (M.T.W.T.L.) using an inguinal approach. Pain reduction was determined at prospective follow-up. This study evaluated 180 patients. Most patients (61.1%) had undergone a scrotal or inguinal procedure. Patients had complaints during sexual activities (51.7%), sitting (37.5%), and/or cycling (36.7%); 189 randomized blockades were offered to all patients. There was a positive response in 37% and a negative response in 51%. MDSC was performed on 58 testicular units, including 3 patients with a negative outcome of the blockades. At mean follow-up of 42.8 months, 86.2% had a ≥ 50% reduction of pain and 51.7% were completely pain free. MDSC is a valuable treatment option for TPS patients because in this study 86.2% experienced a ≥ 50% reduction of pain. To prevent superfluous diagnostics and treatment, it is mandatory to follow a systematic protocol in the treatment of TPS.

  20. Identification in the human central nervous system, pituitary, and thyroid of a novel calcitonin gene-related peptide, and partial amino acid sequence in the spinal cord.

    Science.gov (United States)

    Petermann, J B; Born, W; Chang, J Y; Fischer, J A

    1987-01-15

    Two human genes encoding precursors for two calcitonin gene-related peptides (CGRP) I (or alpha) and II (or beta) have been identified (Steenbergh, P. H., Höppener, J. W. M., Zandberg, J., Lips, C. J. M., and Jansz, H. S. (1985) FEBS Lett. 183, 403-407). The amino acid sequence of CGRP-I was obtained in medullary thyroid carcinoma extracts (Morris, H. R., Panico, M., Etienne, T., Tippins, J., Girgis, S. I., and MacIntyre, I. (1984) Nature 308, 746-748), but not in normal human tissues. The human CGRP-II peptide remained to be discovered. Here we have determined in the human spinal cord the amino acid composition and the partial amino acid sequence of the DNA-predicted CGRP-I and -II. The data indicate for the first time the existence of a second CGRP different from the known CGRP-I. CGRP-II has been identified in the central nervous system, pituitary, thyroid, and in medullary thyroid carcinoma as a major CGRP form together with CGRP-I.

  1. Growth hormone deficiency and central precocious puberty in Klinefelter syndrome: report of a case and review of KIGS database.

    Science.gov (United States)

    Gonzales-Ellis, Bryn A; Pingul, Mia M; Reddy, Sujana; Boney, Charlotte M; Wajnrajch, Michael P; Quintos, Jose Bernarto

    2013-01-01

    Growth hormone deficiency (GHD) and central precocious puberty (CPP) have each, individually, been described in patients with Klinefelter syndrome. However, the combination of GHD, CPP, and Klinefelter syndrome has never been reported. We described a Klinefelter syndrome patient who developed GHD at age 2 10/12 years and CPP at 8 6/12 years. Despite CPP, GnRH agonist therapy was not initiated because of his excellent predicted adult height. At 11 8/12 years, his height was 164.6 cm, close to his mid-parental target height of 165 cm. We report an additional nine patients with Klinefelter syndrome and GHD from the Pfizer International Growth Study (KIGS) database, none of whom had CPP. We conclude that the combination of GHD and CPP is very rare in Klinefelter syndrome and that CPP is unlikely to compromise final adult height.

  2. A REPORT OF CENTRAL RETINAL ARTERY OCCLUSION (CRAO , IN YOUNG MALES IN ITS INITIAL MANIFESTATION, AS PRIMAR Y ANTIPHOSPHOLIPID SYNDROME

    Directory of Open Access Journals (Sweden)

    Rani

    2013-05-01

    Full Text Available ABSTRACT: AIM: To report a case of Central Retinal Artery Occlusi on (CRAO in young males in its initial manifestation as Primary Antiphospholipid Syndrome. METHODS: 32 year healthy male, with abrupt sudden painless loss of vision in r ight eye since 48 hours, with Grade 2 Relative afferent pupillary defect, visual acuity of hand movements in OD and 6/18 in OS. Fundoscopy disclosed signs compatible of central reti nal artery occlusion confirmed with FFA. Carotid Doppler imaging and echocardiography was done to determine the source. RESULTS: Antiphospholipid antibody cofactor, beta2-glycoprotein 1 antibodies, IgM, was positive with titre of more than 94 un its/ml on two occasions, 1 2 weeks apart, with normal range being less than 20 units/ml for each isotope (IgG, IgM, or IgA .According to the 2006 revised Sapporo criteria Antiphospholipid syndrome was diagnosed. Thor ough examination excluded other system involvement. Immunological studies excluded other systemic disorders. CONCLUSIONS: In literature, prevalence of CRAO is 0.85% for every 100000 and prevalence of Antiphospholipid Syndrome in patients showing a major retinal vascula r obstruction is 5% - 33%. Antiphospholipid syndrome should be ruled out in every young patient who presents with Central retinal artery occlusion. Association must be considered, as Central retinal artery occlusion could be the initial manifestation of ant iphospholipid syndrome with high risk of recurrence.

  3. Central Pontine and Extrapontine Myelinolysis: The Great Masquerader—An Autopsy Case Report

    Directory of Open Access Journals (Sweden)

    Sajish Jacob

    2014-01-01

    Full Text Available Central pontine myelinolysis is a demyelinating disorder characterized by the loss of myelin in the center of the basis pontis usually caused by rapid correction of chronic hyponatremia. The clinical features vary depending on the extent of involvement. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. We herein report a case of myelinolysis involving pons, lateral geniculate bodies, subependymal region, and spinal cord. To the best of our knowledge, this case represents the second case of spinal cord involvement in osmotic demyelination syndrome and the first case of involvement of thoracic region of spinal cord.

  4. Lack of differential pattern in central adiposity and metabolic syndrome in Barrett's esophagus and gastroesophageal reflux disease.

    LENUS (Irish Health Repository)

    Healy, L A

    2012-02-01

    Obesity is an established risk factor for esophageal adenocarcinoma, although the mechanism is unclear. A pathway from reflux to inflammation through metaplasia is the dominant hypothesis, and an added role relating to visceral adiposity and the metabolic syndrome has been mooted in Barrett\\'s esophagus (BE) patients. Whether BE differs from gastroesophageal reflux disease (GERD) in obesity and metabolic syndrome profiles is unclear, and this was the focus of this study. Patients with proven BE or GERD were randomly selected from the unit data registry and invited to attend for metabolic syndrome screening, anthropometry studies including segmental body composition analysis, and laboratory tests including fasting lipids, insulin, and C-reactive protein. Metabolic syndrome was defined using the National Cholesterol Education Program (NCEP) and the International Diabetes Federation (IDF) criteria. One hundred and eighteen BE patients and 113 age- and sex-matched GERD controls were studied. The incidence of obesity (body mass index >30 kg\\/m(2)) was 36% and 38%, respectively, with the pattern of fat deposition predominantly central and an estimated trunk fat mass of 13 and 14 kg, respectively. Using the NCEP criteria, metabolic syndrome was significantly more common in the BE cohort (30% vs 20%, P < 0.05), but there was no significant difference using IDF criteria (42% vs 37%, P= 0.340). Central obesity and the metabolic syndrome are common in both Barrett\\'s and GERD cohorts, but not significantly different, suggesting that central obesity and the metabolic syndrome does not per se impact on the development of BE in a reflux population. In BE, the importance of obesity and the metabolic syndrome in disease progression merits further study.

  5. [A case of central alveolar hypoventilation syndrome associated with cerebral infarction].

    Science.gov (United States)

    Yamada, A; Kamoda, M; Ikezoe, K; Tsukaguchi, M; Katanaka, J; Deguchi, K; Miki, H; Takeuchi, H

    1993-03-01

    Central alveolar hypoventilation syndrome (CAH), or Ondine's curse, is a very rare disease characterized by dysfunction of respiratory center in the brain stem. Here, we report a case of CAH associated with cerebral infarction. A 59-year-old man developed right facial sensory deficit at age 56. Then, the facial sensory deficit spread to the left side and dysarthria and dysphagia also developed. Since age 58, he often developed respiratory failure and consciousness disturbance. Arterial blood gas analysis revealed alveolar hypoventilation and respiratory acidosis. Disorders of peripheral organs such as lung, airway, thorax and neuromuscular diseases were ruled out. Brain MRI showed cerebral infarction in the brain stem. We diagnosed him as CAH associated with brain stem infarction.

  6. Mutation of fibulin-1 causes a novel syndrome involving the central nervous system and connective tissues.

    Science.gov (United States)

    Bohlega, Saeed; Al-Ajlan, Huda; Al-Saif, Amr

    2014-05-01

    Fibulin-1 is an extracellular matrix protein that has an important role in the structure of elastic fibers and basement membranes of various tissues. Using homozygosity mapping and exome sequencing, we discovered a missense mutation, p.(Cys397Phe), in fibulin-1 in three patients from a consanguineous family presented with a novel syndrome of syndactyly, undescended testes, delayed motor milestones, mental retardation and signs of brain atrophy. The mutation discovered segregated with the phenotype and was not found in 374 population-matched alleles. The affected cysteine is highly conserved across vertebrates and its mutation is predicted to abolish a disulfide bond that defines the tertiary structure of fibulin-1. Our findings emphasize the crucial role fibulin-1 has in development of the central nervous system and various connective tissues.

  7. A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome.

    Science.gov (United States)

    Yamashita, Chikara; Shigeto, Hiroshi; Maeda, Norihisa; Torii, Takako; Ohyagi, Yasumasa; Kira, Jun-Ichi

    2015-01-01

    Central pontine myelinolysis (CPM), which was originally considered to be the result of rapid correction of chronic hyponatremia, is not necessarily accompanied by hyponatremia or drastic changes in serum sodium level. Here, we report a case of an anorexic 55-year-old male with a history of pharyngo-laryngo-esophagogastrectomy, initially hospitalized with status epilepticus. Although his consciousness gradually recovered as we were controlling his convulsion, it deteriorated again with new onset of anisocoria, and magnetic resonance imaging (MRI) at this point revealed CPM. Rapid change of serum sodium or osmolarity, which is often associated with CPM, had not been apparent throughout his hospitalization. Instead, a review of the serum biochemistry test results showed that serum phosphate had drastically declined the day before the MRI first detected CPM. In this case, we suspect that hypophosphatemia induced by refeeding syndrome greatly contributed to the occurrence of CPM.

  8. A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome

    Directory of Open Access Journals (Sweden)

    Chikara Yamashita

    2015-10-01

    Full Text Available Central pontine myelinolysis (CPM, which was originally considered to be the result of rapid correction of chronic hyponatremia, is not necessarily accompanied by hyponatremia or drastic changes in serum sodium level. Here, we report a case of an anorexic 55-year-old male with a history of pharyngo-laryngo-esophagogastrectomy, initially hospitalized with status epilepticus. Although his consciousness gradually recovered as we were controlling his convulsion, it deteriorated again with new onset of anisocoria, and magnetic resonance imaging (MRI at this point revealed CPM. Rapid change of serum sodium or osmolarity, which is often associated with CPM, had not been apparent throughout his hospitalization. Instead, a review of the serum biochemistry test results showed that serum phosphate had drastically declined the day before the MRI first detected CPM. In this case, we suspect that hypophosphatemia induced by refeeding syndrome greatly contributed to the occurrence of CPM.

  9. A Case of Turner Syndrome with Concomitant Transient Hypogammaglobulinaemia of Infancy and Central Diabetes Insipidus

    Science.gov (United States)

    Korkmaz, Hüseyin Anıl; Özkan, Behzat; Hazan, Filiz; Büyükinan, Muammer; Çelik, Tanju

    2013-01-01

    Turner syndrome (TS) is a genetic disorder that affects development in females and is characterized by the complete or partial absence of the second sex chromosome, or monosomy X. TS is associated with abnormalities in lymphatic and skeletal development, in growth, and in gonadal function. Cardiac and renal malformations and a number of specific cognitive findings may also be encountered in these patients. An increased risk for hypothyroidism, sensorineural hearing loss, hypertension, and other problems has also been reported. We present the case of a patient with TS accompanied by transient hypogammaglobulinaemia of infancy (THI) and central diabetes insipidus, which we believe is the first reported TS patient with these concomitant disorders. Conflict of interest:None declared. PMID:23419422

  10. Spinal Cauda Equina Stimulation for Alternative Location of Spinal Cord Stimulation in Intractable Phantom Limb Pain Syndrome: A Case Report.

    Science.gov (United States)

    Lee, Pil Moo; So, Yun; Park, Jung Min; Park, Chul Min; Kim, Hae Kyoung; Kim, Jae Hun

    2016-04-01

    Phantom limb pain is a phenomenon in which patients experience pain in a part of the body that no longer exists. In several treatment modalities, spinal cord stimulation (SCS) has been introduced for the management of intractable post-amputation pain. A 46-year-old male patient complained of severe ankle and foot pain, following above-the-knee amputation surgery on the right side amputation surgery three years earlier. Despite undergoing treatment with multiple modalities for pain management involving numerous oral and intravenous medications, nerve blocks, and pulsed radiofrequency (RF) treatment, the effect duration was temporary and the decreases in the patient's pain score were not acceptable. Even the use of SCS did not provide completely satisfactory pain management. However, the trial lead positioning in the cauda equina was able to stimulate the site of the severe pain, and the patient's pain score was dramatically decreased. We report a case of successful pain management with spinal cauda equina stimulation following the failure of SCS in the treatment of intractable phantom limb pain.

  11. Pitfalls in the diagnosis of reversible cerebral vasoconstriction syndrome and primary angiitis of the central nervous system

    NARCIS (Netherlands)

    Koopman, K; Uyttenboogaart, M; Luijckx, G J; De Keyser, J; Vroomen, P C A J

    2007-01-01

    A case of a 51-year-old woman with reversible cerebral vasoconstriction syndrome (RCVS) without an associative cause is reported. Initially the diagnosis primary angiitis of the central nervous system (PACNS) was considered. Both diagnosis are rare and can mimic each other. Distinction between both

  12. Operative Treatment of Haglund Syndrome With Central Achilles Tendon-Splitting Approach.

    Science.gov (United States)

    Ahn, Jae Hoon; Ahn, Chi-Young; Byun, Chu-Hwan; Kim, Yoon-Chung

    2015-01-01

    Haglund syndrome is characterized by chronic posterior heel pain associated with a posterosuperior calcaneal prominence. We assessed the clinical and radiologic outcomes after operative treatment of Haglund syndrome using the central tendon-splitting approach. Fifteen feet in 15 patients were investigated retrospectively after surgery. Of the 15 patients, 14 were males (93.3%) and 1 was female (6.7%). Their mean age was 33.1 ± 8.2 (range 20 to 50) years. The mean follow-up duration was 3.5 ± 1.5 years (range 24 to 90 months). The American Orthopaedic Foot and Ankle Society ankle-hindfoot Scale and Victorian Institute of Sport Assessment-Achilles scores were investigated to assess the clinical outcomes. Patient satisfaction was assessed at the latest follow-up visit. The lateral talo-first metatarsal angle, calcaneal pitch angle, Fowler-Philip angle, and parallel pitch line were measured to assess the foot shape and radiographic outcomes. Clinically, the mean American Orthopaedic Foot and Ankle Society ankle-hindfoot scale score increased from 62.1 ± 7.5 preoperatively to 92.5 ± 3.5 at the latest follow-up visit. The mean Victorian Institute of Sport Assessment-Achilles score increased from 53.2 ± 7.4 to 89.6 ± 3.4. All patients were satisfied with the operative results. Radiographically, all patients had cavus feet with an increased lateral talo-first metatarsal angle (mean +5.9° ± 5.0°) and calcaneal pitch angle (mean 26.0° ± 3.8°). The mean Fowler-Philip angle decreased from 58.9° ± 15.0° to 32.5° ± 7.2° postoperatively, and the positive parallel pitch line had changed to a negative value in all cases. Operative treatment with the central tendon-splitting approach appears to be safe and satisfactory for intractable Haglund syndrome.

  13. Spinal cord involvement in chronic inflammatory demyelinating polyradiculoneuropathy: a clinical and MRI study.

    Science.gov (United States)

    Ioannidis, Panagiotis; Parissis, Dimitris; Karapanayiotides, Theodoros; Maiovis, Pantelis; Karacostas, Dimitris; Grigoriadis, Nikolaos

    2015-06-01

    Concomitant central nervous system (CNS) involvement in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare. Although the spinal nerve roots may present MRI abnormalities in CIDP, hitherto, the spinal cord has been investigated in a single study. We retrospectively investigated clinically and with MRI a cohort of patients with definite CIDP diagnosis (EFNS/PNS criteria) for evidence of brain and spinal cord involvement, who were initially admitted in our department during the last 4 years. Among 12 patients with CIDP (men: 8, mean age: 59.3 years, mean disease duration: 3.8 years), nine patients had their MRI scan during a clinical relapse and three during remission. Brain MRI did not document typical multiple sclerosis lesions in any patient. We did not identify any MRI abnormalities in ten patients without clinical evidence of spinal cord involvement. Conversely, MRI disclosed extensive lesions of the thoracic cord in two patients with an overt spinal cord syndrome, whom we describe. This represents the biggest MRI study of CIDP patients who have been investigated for spinal cord involvement. Our data support earlier observations that a minority of CIDP patients may additionally develop CNS involvement of variable degree.

  14. Neurological involvement in primary Sjogren syndrome: a focus on central nervous system.

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    Manuela Morreale

    Full Text Available OBJECTIVES: Sjögren syndrome is an autoimmune disease involving mainly salivary and lacrimal glands. Beyond widely described PNS involvement, high variable prevalence of CNS manifestations ranging from 2.5 and 60% of all pSS patients has been reported, without specific syndrome definition. The aim of this cohort study was to evaluate the prevalence of CNS signs and symptoms in pSS patients and to identify possible biomarkers of CNS damage. METHODS: 120 patients with pSS diagnosis according to the 2002 American-European Consensus Group criteria were enrolled after exclusion of secondary causes. All patients underwent to a wide neurological, neuropsychological, psychiatric, neuroradiological and ultrasonographic evaluation. RESULTS: Central and peripheral nervous system involvement was observed in 81 patients with a prevalence of 67.5%. The prevalence of CNS involvement was significantly higher than PNS disease (p 0.001. 68 patients (84% shown non-focal CNS symptoms and 64 (79% focal CNS deficits with headache as the most common feature (46.9%, followed by cognitive (44.4% and mood disorders (38.3%. Particularly, we observed a high prevalence of migraine without aura, subcortical frontal executive functions and verbal memory impairment and apathy/alexythimia. MR spectroscopy revealed a reduction of NAA levels or NAA/Cr ratio decrease in subcortical frontal and basal ganglia white matter, while ultrasonography showed an impairment of microvasculature response. At multivariate analysis, headache, cognitive disorders and psychiatric symptoms was significantly associated to serological markers (anti-SSA, MRS and ultrasonographic features. CONCLUSIONS: The higher prevalence of MWO-mimic headache, cognitive dys-executive syndrome and mood disorders observed in this series confirmed previous evidences of a higher diffused CNS compromission rather than focal involvement such as SM-like clinical course or NMO-like syndrome. The association with

  15. Bilateral and Simultaneous Central Retinal Vein Occlusion in a Patient with Obstructive Sleep Apnea Syndrome

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    Andrea Govetto

    2014-05-01

    Full Text Available Purpose: To describe a case of bilateral and simultaneous central retinal vein occlusion (RVO in a young patient diagnosed with obstructive sleep apnea syndrome (OSAS. Case Report: A 38-year-old man with morbid obesity and daytime sleepiness presented with a history of bilateral vision loss. His visual acuity (VA was hand movements, and fundus examination (FE revealed bilateral central RVO. General medical examination revealed untreated hypertension and type II respiratory failure. Laboratory tests for thrombophilia showed increased hematocrit (59% and high levels of fibrinogen and C-reactive protein. Other causes of congenital and acquired hypercoagulability were ruled out. Pathologic polysomnography led to the diagnosis of OSAS. The patient was treated with antihypertensive drugs and continuous positive air pressure. In addition, he received intravitreal ranibizumab. At 10 months after presentation, his VA was no light perception in the right eye and hand movements in the left eye. FE revealed bilateral retinal and optic nerve atrophy, and the occurrence of a nonarteritic anterior ischemic neuropathy in the right eye was considered.

  16. Poro-elastic modeling of Syringomyelia - a systematic study of the effects of pia mater, central canal, median fissure, white and gray matter on pressure wave propagation and fluid movement within the cervical spinal cord.

    Science.gov (United States)

    Støverud, Karen H; Alnæs, Martin; Langtangen, Hans Petter; Haughton, Victor; Mardal, Kent-André

    2016-01-01

    Syringomyelia, fluid-filled cavities within the spinal cord, occurs frequently in association with a Chiari I malformation and produces some of its most severe neurological symptoms. The exact mechanism causing syringomyelia remains unknown. Since syringomyelia occurs frequently in association with obstructed cerebrospinal fluid (CSF) flow, it has been hypothesized that syrinx formation is mechanically driven. In this study we model the spinal cord tissue either as a poro-elastic medium or as a solid linear elastic medium, and simulate the propagation of pressure waves through an anatomically plausible 3D geometry, with boundary conditions based on in vivo CSF pressure measurements. Then various anatomic and tissue properties are modified, resulting in a total of 11 variations of the model that are compared. The results show that an open segment of the central canal and a stiff pia (relative to the cord) both increase the radial pressure gradients and enhance interstitial fluid flow in the central canal. The anterior median fissure, anisotropic permeability of the white matter, and Poisson ratio play minor roles.

  17. The clinical analysis of central neurological dysfunctions in primary Sjogren's syndrome: 4 cases report%原发性干燥综合征中枢神经系统损害4例分析

    Institute of Scientific and Technical Information of China (English)

    姚晓萍; 李智文; 林仕芳

    2012-01-01

    目的 探讨原发性干燥综合征中枢神经系统损害的临床特点,以提高其早期诊断、治疗效果.方法 回顾性分析4例原发性干燥综合征中枢神经系统损害患者的临床特征、诊疗情况,并结合国内外相关文献进行分析.结果 4例患者均为女性,年龄30~52岁.3例患者中枢神经系统损害表现先于干燥综合征诊断,1例患者先诊断干燥综合征后出现中枢神经系统损害.1例患者出现脊髓损害表现,2例患者为脑实质损害表现,1例具有脊髓、脑、视神经损害表现.4例患者MR均表现为点状或小片状长T1长T2信号影.治疗上1例患者单用糖皮质激素,其余3例患者均合用免疫抑制剂,治疗后病情均好转,随访无再新发中枢神经系统损害表现.结论 对不能用其他原因解释的中枢神经系统损害,尤其是中老年女性,应警惕原发性干燥综合征的可能.重视干燥综合征症状的询问,加深对干燥综合征中枢神经系统损害临床表现的认识,进行相关检查,以提高早期诊断、治疗效果,减少患者的致残率.%Objective To explore the clinical features of central neurological dysfunctions in primary Sjogren' s syndrome , and to improve its early diagnosis and treatment. Methods Retrospective review the clinical features, diagnosis and treatment in four patients of central neurological dysfunctions in primary Sjogren s syndrome, and analyze the relative literatures. Results The four patients were female, aged from 30 years old to 52 years old. Three patients of them presented the manifestation of central nervous system damage ahead of the diagnosis of Sjogren' s syndrome. One patient with spinal cord injury , two patients with brain parenchymal damage, and one patient with spinal cord injury, brain parenchyma and optic nerve damage. All of the patients showed punctate or small pieces of long Tl, T2 signal intensity in the MR examination. In terms of treatment, one patient was treated

  18. Syringohydromyelia in the primary children tethered cord syndrome:magnetic resonance imaging features and correlation analysis%儿童脊髓积水空洞症在原发脊髓栓系综合征的 MRI 表现及相关分析

    Institute of Scientific and Technical Information of China (English)

    李荣品

    2016-01-01

    Objective To summary MRI features of children syringohydromyelia in primary tethered cord syndrome and analyze the correlation.Methods 84 syringohydromyelia cases were analyzed retrospectively.The imaging features of syringohydromyelia including the location,the size and the shape,the syrinx index and so on were analyzed,and the correlation of syringohydromyelia with various diseases which caused tethered cord syndrome were analyzed simultaneously.Results The central canal of the spinal cord was filiform in 1 5 cases,fine tubular in 21 cases and cystic dilatation in 48 cases.There were no relation to the length and width with the age.There was statistically significance in syrinx index(>40%)with diastematomyelia(P =0.017),and no relation to other types of tethered cord syndrome.Diastematomyelia and myelocele were risk factors for cystic dilatation of syringohydromyelia.Conclusion Diastematomyelia and myelocele have a higher incidence in cystic dilatation of syringohydromyelia than that in other types of tethered cord syndrome,and diastematomyelia is more common in association with bigger syrinx index of syringohydromyelia.%目的:总结儿童脊髓积水空洞症在原发脊髓栓系综合征的 MRI 表现,并与脊髓栓系综合征进行相关性分析。方法回顾性总结分析84例脊髓积水空洞症的 MRI 表现,包括脊髓积水空洞的位置、大小、形态、空洞指数等,并与引起栓系的各种病变进行统计学分析。结果脊髓积水空洞中央管呈丝状扩张15例,管状扩张21例,囊状扩张48例。脊髓积水空洞的宽度及长度与年龄均不相关。空洞指数>40%与脊髓纵裂有统计学意义(P =0.017),而与脊髓低位栓系其他各类型无相关性。脊髓低位栓系综合征中脊髓纵裂、脊膜膨出是出现脊髓积水空洞症囊状扩张的危险因素。结论脊髓纵裂和脊膜膨出更容易合并脊髓积水空洞症囊状扩张的发生,而且脊髓纵裂更容易出现大

  19. Bilateral Central Retinal Vein Occlusions Combined with Artery Occlusions in A Patient with Acquired Immune Deficiency Syndrome

    Institute of Scientific and Technical Information of China (English)

    Feng Wen; Xuemei Chen; Haitai Li; Ruiduan Liao; Dezheng Wu

    2002-01-01

    Purpose: This is the first report of a bilateral nonischemic central retinal vein occlusionscombined with artery occlusions in a patient with acquired immune deficiency syndrome(AIDS). Methods: Case report. Results: A 22-year-old Chinese(male) with a positive human immunodeficiency virus(HIV) infection developed bilateral nonischemic central retinal vein occlusions combinedwith artery occlusions and severe vision loss. The manifestations of the fundus andfluorescein angiography were similar in both eyes.Conclusion: This case report provides the evidences that central retinal vein and arteryocclusions are probably part of the spectrum of AIDS vascular diseases.

  20. Imaging Findings of Central Nervous System Vasculitis Associated with Goodpasture's Syndrome: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jee Young; Ahn, Kook Jin; Jung, Jung Im; Jung, So Lyung; Kim, Bum Soo; Hahn, Seong Tae [College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

    2007-12-15

    We report a rare case of CNS vasculitis associated with Goodpasture's syndrome in a 34-year-old man, who presented with a seizure and sudden onset of right sided weakness. He also had recurrent hemoptysis of one month's duration. Goodpasture's syndrome is histologically diagnosed by intense linear deposits of IgG along the glomerular basement membrane in both renal and lung tissues. oodpasture's syndrome is a rare disease, characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage and circulating antiglomerular basement membrane antibody (anti-GBM antibody). Central nervous system (CNS) manifestations in Goodpasture's syndrome are extremely rare, with only a few cases having been reported in the literature (8 10). Therefore, we present our imaging findings of CNS vasculitis associated with Goodpasture's syndrome, together with a review of the relevant literature. In summary, CNS vasculitis associated with Goodpasture's syndrome is extremely rare. Awareness of the imaging findings, as well as the clinical significance of CNS vasculitis associated with Goodpasture's syndrome, can be helpful in making the correct diagnosis and subsequent management of this rare condition.

  1. Evaluation of the Endothelial Cell Density and the Central Corneal Thickness in Pseudoexfoliation Syndrome and Pseudoexfoliation Glaucoma

    Directory of Open Access Journals (Sweden)

    Bożydar T. Tomaszewski

    2014-01-01

    Full Text Available Purpose. Evaluation of central corneal thickness (CCT and endothelial cell density (ECD in patients with senile cataract and coexisting pseudoexfoliation (PEX syndrome with glaucoma (PEXG and without glaucoma using specular microscopy. Participants and Methods. The study included 122 patients (217 eyes. In this group of patients we identified 133 eyes with PEX syndrome (65 with glaucoma, 68 without glaucoma and 84 eyes without PEX syndrome. ECD and CCT were measured in each eye by specular microscopy. Results. ECD in eyes with PEX syndrome without glaucoma (2297 ± 359 cell/mm2 and in eyes with PEXG (2241 ± 363 cell/mm2 was lower than in the control group (2503 ± 262 cell/mm2 (P<0.001. CCT in eyes with PEXG (508.2 ± 32.6 μm was thinner than in eyes with PEX syndrome without glaucoma (529.7 ± 30.3 μm and control group (527.7 ± 29.4 μm (P<0.001. Conclusions. This research shows that in eyes with PEX syndrome, both with and without glaucoma, ECD was statistically significantly lower than in the control group. In patients with PEXG, CCT was statistically significantly thinner than in the PEX syndrome and control group.

  2. Circulating vascular progenitor cells and central arterial stiffness in polycystic ovary syndrome.

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    Cecile Dessapt-Baradez

    Full Text Available OBJECTIVE: Subjects with Polycystic ovarian syndrome (PCOS are at increased risk of Type 2 diabetes mellitus (T2DM. The mechanism of this enhanced risk is unclear. Circulating vascular progenitor cells (VPC are immature bone marrow derived cells capable of differentiating into mature endothelial cells. VPC number/function and central arterial stiffness predict cardio-metabolic disease in at-risk populations. DESIGN: We studied VPC and arterial stiffness measures in non-obese PCOS subjects as compared to age and body mass index (BMI matched healthy controls in a cross-sectional study. METHODS: Fourteen subjects with PCOS and 12 controls of similar age, BMI (all <30 kg/m(2 and metabolic profile were studied. VPC number and in vitro function were studied by flow cytometry and tube formation assays respectively. Augmentation index (AIx, a measure of central arterial stiffness, and central (aortic blood pressures (BP were measured by applanation tonometry. RESULTS: Subjects with PCOS had a reduced number, mean±SEM, of circulating CD34(+133(+ VPCs (317.5±51.0 vs. 558.3±101.2, p = 0.03 and impaired in vitro tube formation (completed tube area 1.0±0.06 vs. 1.2±0.05×10(6 µm(2 p = 0.02. PCOS subjects had significantly higher AIx (18.4±1.9% vs. 4.9±2.0% and this difference remained significant even after adjustments for age, BMI and smoking (p = 0.003 in multivariate analyses. Central systolic and pulse pressure were higher in PCOS subjects but these differences were not statistically significant after adjustment for age. Brachial systolic and pulse pressures were similar. VPC number/function and arterial stiffness or BP measures were not correlated. CONCLUSIONS: Non-obese PCOS is characterized by a reduced VPC number, impaired VPC function and increased central arterial stiffness. These changes in novel vascular risk markers may explain the enhanced risk of T2DM and CVD in PCOS.

  3. Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: an overview of Down syndrome, autism, Fragile X and Rett syndrome.

    Science.gov (United States)

    Valenti, Daniela; de Bari, Lidia; De Filippis, Bianca; Henrion-Caude, Alexandra; Vacca, Rosa Anna

    2014-10-01

    Clinical manifestations typical of mitochondrial diseases are often present in various genetic syndromes associated with intellectual disability, a condition leading to deficit in cognitive functions and adaptive behaviors. Until now, the causative mechanism leading to intellectual disability is unknown and the progression of the condition is poorly understood. We first report latest advances on genetic and environmental regulation of mitochondrial function and its role in brain development. Starting from the structure, function and regulation of the oxidative phosphorylation apparatus, we review how mitochondrial biogenesis and dynamics play a central role in neurogenesis and neuroplasticity. We then discuss how dysfunctional mitochondria and alterations in reactive oxygen species homeostasis are potentially involved in the pathogenesis of various neurodevelopmental syndromes with a special focus on Down, Rett, Fragile X syndromes and autism spectrum disorders. Finally, we review and suggest novel therapeutic approaches aimed at improving intellectual disability by activating mitochondrial function and reducing oxidative stress to amiliorate the quality of life in the subjects affected.

  4. Congenital central hypoventilation syndrome associated with Hirschsprung's Disease: case report and literature review

    Science.gov (United States)

    Sandoval, Renata Lazari; Zaconeta, Carlos Moreno; Margotto, Paulo Roberto; Cardoso, Maria Teresinha de Oliveira; França, Evely Mirella Santos; Medina, Cristina Touguinha Neves; Canó, Talyta Matos; de Faria, Aline Saliba

    2016-01-01

    Abstract Objective: To report the case of a newborn with recurrent episodes of apnea, diagnosed with Congenital Central hypoventilation syndrome (CCHS) associated with Hirschsprung's disease (HD), configuring Haddad syndrome. Case description: Third child born at full-term to a non-consanguineous couple through normal delivery without complications, with appropriate weight and length for gestational age. Soon after birth he started to show bradypnea, bradycardia and cyanosis, being submitted to tracheal intubation and started empiric antibiotic therapy for suspected early neonatal sepsis. During hospitalization in the NICU, he showed difficulty to undergo extubation due to episodes of desaturation during sleep and wakefulness. He had recurrent episodes of hypoglycemia, hyperglycemia, metabolic acidosis, abdominal distension, leukocytosis, increase in C-reactive protein levels, with negative blood cultures and suspected inborn error of metabolism. At 2 months of age he was diagnosed with long-segment Hirschsprung's disease and was submitted to segment resection and colostomy through Hartmann's procedure. A genetic research was performed by polymerase chain reaction for CCHS screening, which showed the mutated allele of PHOX2B gene, confirming the diagnosis. Comments: This is a rare genetic, autosomal dominant disease, caused by mutation in PHOX2B gene, located in chromosome band 4p12, which results in autonomic nervous system dysfunction. CCHS can also occur with Hirschsprung's disease and tumors derived from the neural crest. There is a correlation between phenotype and genotype, as well as high intrafamilial phenotypic variability. In the neonatal period it can simulate cases of sepsis and inborn errors of metabolism. PMID:26838603

  5. Systemic 5-fluorouracil treatment causes a syndrome of delayed myelin destruction in the central nervous system

    Directory of Open Access Journals (Sweden)

    Han Ruolan

    2008-04-01

    Full Text Available Abstract Background Cancer treatment with a variety of chemotherapeutic agents often is associated with delayed adverse neurological consequences. Despite their clinical importance, almost nothing is known about the basis for such effects. It is not even known whether the occurrence of delayed adverse effects requires exposure to multiple chemotherapeutic agents, the presence of both chemotherapeutic agents and the body's own response to cancer, prolonged damage to the blood-brain barrier, inflammation or other such changes. Nor are there any animal models that could enable the study of this important problem. Results We found that clinically relevant concentrations of 5-fluorouracil (5-FU; a widely used chemotherapeutic agent were toxic for both central nervous system (CNS progenitor cells and non-dividing oligodendrocytes in vitro and in vivo. Short-term systemic administration of 5-FU caused both acute CNS damage and a syndrome of progressively worsening delayed damage to myelinated tracts of the CNS associated with altered transcriptional regulation in oligodendrocytes and extensive myelin pathology. Functional analysis also provided the first demonstration of delayed effects of chemotherapy on the latency of impulse conduction in the auditory system, offering the possibility of non-invasive analysis of myelin damage associated with cancer treatment. Conclusions Our studies demonstrate that systemic treatment with a single chemotherapeutic agent, 5-FU, is sufficient to cause a syndrome of delayed CNS damage and provide the first animal model of delayed damage to white-matter tracts of individuals treated with systemic chemotherapy. Unlike that caused by local irradiation, the degeneration caused by 5-FU treatment did not correlate with either chronic inflammation or extensive vascular damage and appears to represent a new class of delayed degenerative damage in the CNS.

  6. Impaired Neural Structure and Function Contributing to Autonomic Symptoms in Congenital Central Hypoventilation Syndrome

    Directory of Open Access Journals (Sweden)

    Ronald M Harper

    2015-10-01

    Full Text Available Congenital central hypoventilation syndrome (CCHS patients show major autonomic alterations in addition to their better-known breathing deficiencies. The processes underlying CCHS, mutations in the PHOX2B gene, target autonomic neuronal development, with frame shift extent contributing to symptom severity. Many autonomic characteristics, such as impaired pupillary constriction and poor temperature regulation, reflect parasympathetic alterations, and can include disturbed alimentary processes, with malabsorption and intestinal motility dyscontrol. The sympathetic nervous system changes can exert life-threatening outcomes, with dysregulation of sympathetic outflow leading to high blood pressure, time-altered and dampened heart rate and breathing responses to challenges, cardiac arrhythmia, profuse sweating, and poor fluid regulation. The central mechanisms contributing to failed autonomic processes are readily apparent from structural and functional magnetic resonance imaging studies, which reveal substantial cortical thinning, tissue injury, and disrupted functional responses in hypothalamic, hippocampal, posterior thalamic, and basal ganglia sites and their descending projections, as well as insular, cingulate, and medial frontal cortices, which influence subcortical autonomic structures. Midbrain structures are also compromised, including the raphe system and its projections to cerebellar and medullary sites, the locus coeruleus, and medullary reflex integrating sites, including the dorsal and ventrolateral medullary nuclei. The damage to rostral autonomic sites overlaps metabolic, affective and cognitive regulatory regions, leading to hormonal disruption, anxiety, depression, behavioral control, and sudden death concerns. The injuries suggest that interventions for mitigating hypoxic exposure and nutrient loss may provide cellular protection, in the same fashion as interventions in other conditions with similar malabsorption, fluid turnover

  7. Clinical Observation of Acupunture Treatment for Central Pain after Spinal Cord Injury%针刺治疗脊髓损伤后中枢性疼痛的临床分析

    Institute of Scientific and Technical Information of China (English)

    陈少娜; 张丽霞

    2015-01-01

    目的:观察探讨针刺治疗脊髓损伤后中枢性疼痛临床疗效。方法:将46例脊髓损伤后中枢性疼痛患者分为治疗组和对照组各23例,两组患者均予常规的康复治疗及药物治疗,治疗组同时进行针刺治疗,1个月后采用McGill疼痛问卷评定疗效。结果:治疗后McGill疼痛各项评分结果显示,患者疼痛感觉和强度等指标评分均下降,而且治疗组对患者疼痛的改善明显优于对照组(P <0.05)。结论:针刺治疗脊髓损伤后中枢性疼痛有良好的疗效。%Objective: To observe the effects of acupunture treatment for central pain after spinal cord injury. Methods: 46 patients with central pain after spinal cord injury were randomly divided into the treatment group (n=23) and the control group (n=23). The patients in the treatment group were treated with acupunture treatment, while the patients in the control group were treated with routine treatment. The simplified Mcgill Pain Questionnaire was used to evaluate the effect 1 month after the treatment. Results: At the end of the treatment course,all the values of pain parameter were significantly decreased between the two groups (P<0.05). Conclusion: The curative effect of acupuncture treatment has potentially valuable for central pain after spinal cord injury.

  8. 电针夹脊穴治疗脊髓损伤后中枢性疼痛疗效观察%Observations on the Efficacy of Electroacupuncture at Huatuojiaji Points in Treating Central Pain After Spinal Cord Injury

    Institute of Scientific and Technical Information of China (English)

    樊留博; 马利中; 王韵

    2011-01-01

    Objective To compare the clinical efficacy of electroacupuncture at Huatuojiaji points in treating central pain after spinal cord injury. Methods Thirty-four patients with central pain after spinal cord injury were treated with electroacupuncture at Huatuojiaji points. Pre- and post-treatment evaluations were made using the Simplified Mcgill Pain Questionnaire, the Visual Analogous Scale (VAS) and the Activities of Daily Living (ADL). Results At the end of 2 treatment courses, all 6 pain parameter values decreased (P > 0.0 5) and the activities of daily living (ADL) improved markedly (P > 0.01 ). Conclusion Electroacupuncture at Huatuojiaji points has a marked therapeutic effect on central pain after spinal cord injury.%目的 观察电针夹脊穴治疗脊髓损伤后中枢性疼痛的临床疗效.方法 将34例脊髓损伤后中枢性疼痛患者采取电针夹脊穴进行治疗,治疗前、治疗后采用简式McGill疼痛问卷、疼痛视觉模拟评分法(VAS)及日常生活活动能力(ADL)进行评定.结果 2个疗程结束后,患者疼痛的6项参数值均有降低(P<0.05),日常生活活动(ADL)能力明显提高(P<0.01).结论 电针夹脊穴治疗脊髓损伤后中枢性疼痛效果明显.

  9. Comprehensive rehabilitation measure treatment on spinal cord injury central pain of clinical observation%综合康复措施治疗脊髓损伤中枢性疼痛的临床观察

    Institute of Scientific and Technical Information of China (English)

    肖登; 夏新蜀

    2011-01-01

    目的:探讨综合康复治疗措施对脊髓损伤后中枢性疼痛的治疗效果.方法:对31例脊髓损伤后中枢性疼痛患者采用阿米替林、按摩、心理治疗及经皮神经电刺激(TENS)进行治疗.采用McGill疼痛问卷(McGill pain questionnaire,MPQ)、疼痛视觉模拟评分法(Visual Analogous Score,VAS)于治疗前和治疗1疗程后对疼痛和日常生活活动(activities of daily living,ADL)能力进评定.结果:治疗后患者疼痛的各项评分均有明显降低(Pcentral pain of patients with spinal cord injury. Methods: 31 cases of spinal cord injury with central pain were treated with amitriptyline, psychotherapy and transcutaneous electric nerve stimulation ( TENS ), and the effects were assessed by McGill Pain Questionnaire (MPQ), Visual Analogous Score (VAS) and activities of daily living (ADL). Results:The scores of pain of all patients significantly decreased and the scores of ADL highly increased after treatment ( P < 0.01 ). Conclusion: Multidisciplinary therapy is effective on central pain in patients with spinal cord injury.

  10. Infantile Hemophagocytic Lymphohistiocytosis in a Case of Chediak-Higashi Syndrome Caused by a Mutation in the LYST/CHS1 Gene Presenting With Delayed Umbilical Cord Detachment and Diarrhea

    DEFF Research Database (Denmark)

    Nielsen, Christian; Agergaard, Charlotte N; Jakobsen, Marianne A;

    2015-01-01

    perforin content in CD8 T cells seems to correlate to the immune activation state of the patient with 82% and 8% perforin-containing CD8 T cells at active and nonactive hemophagocytic lymphohistiocytosis (HLH) disease, respectively. HLH was confirmed by hemophagocytosis in bone marrow and absent natural...... in this severe phenotype of Chediak-Higashi syndrome was probably induced by rotaviral infection. Interestingly, the intracellular perforin content in CD8 T cells seems to correlate to the immune activation state of the patient. Late separation of the umbilical cord in concordance with clinical symptoms should...

  11. Central nervous system involvement in primary Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Xiao-yu DONG

    2015-04-01

    Full Text Available Clinical and imaging data of 4 cases of primary Sjögren's syndrome (pSS combined with central nervous system (CNS lesions were retrospectively analyzed. Clinical symptoms of 4 patients were as follows: 2 cases had fever; 2 cases had blurred vision; 2 cases had lower extremity weakness; one case had single lower limb weakness; one case had unilateral limb weakness; one case had hemisensory reduction; one case had bilateral abducens paralysis; 3 cases had peripheral neuropathy. Analysis of cerebrospinal fluid (CSF revealed increased CSF pressure (210-270 mm H2O in 3 cases, increased number of leukocyte [(50-380 × 10 6/L] in 3 cases, and increased protein (1.30-2.56 g/L in 3 cases. Brain MRI showed multiple lesions in white matters. After high-dose steroid therapy, 3 cases had good prognosis, and one had recurrent lower extremity weakness after 6 months. pSS combined with CNS lesions is rare. Lumbar puncture and brain MRI is important for diagnosis, and high-dose steroid therapy may have good therapeutic effect. DOI: 10.3969/j.issn.1672-6731.2015.04.014

  12. Similar deficits of central histaminergic system in patients with Down syndrome and Alzheimer disease.

    Science.gov (United States)

    Schneider, C; Risser, D; Kirchner, L; Kitzmüller, E; Cairns, N; Prast, H; Singewald, N; Lubec, G

    1997-02-01

    In order to study whether Alzheimer-like neuropathological changes involve the central histaminergic system we measured the concentration of histamine, its precursor histidine as well as the activity of histidine decarboxylase (HDC) and histamine-N-methyl-transferase (HMT) in frontal cortex of aging Down syndrome (DS) patients, Alzheimer patients and control individuals. The study populations were also investigated for choline acetyltransferase (ChAT) activity, since reduced ChAT activity is an established biochemical hallmark in DS and Alzheimer disease (AD). HDC and ChAT activity were reduced in brains of both DS and Alzheimer patients versus control patients. Additionally, we observed a significant decrease of histamine levels in the DS group. Histamine levels in AD brains tended to be decreased. Histidine concentrations and HMT activities were comparable between the three groups. Thus, our results for the first time show histaminergic deficits in brains of patients with DS resembling the neurochemical pattern in AD. Neuropathological changes may be responsible for similar neurochemical alterations of the histaminergic system in both dementing disorders.

  13. Exposure levels of anti-LINGO-1 Li81 antibody in the central nervous system and dose-efficacy relationships in rat spinal cord remyelination models after systemic administration.

    Science.gov (United States)

    Pepinsky, R Blake; Shao, Zhaohui; Ji, Benxiu; Wang, Qin; Meng, Gym; Walus, Lee; Lee, Xinhua; Hu, Yinghui; Graff, Christilyn; Garber, Ellen; Meier, Werner; Mi, Sha

    2011-11-01

    LINGO-1 (leucine-rich repeat and Ig domain containing NOGO receptor interacting protein-1) is a negative regulator of myelination and repair of damaged axons in the central nervous system (CNS). Blocking LINGO-1 function leads to robust remyelination. The anti-LINGO-1 Li81 antibody is currently being evaluated in clinical trials for multiple sclerosis (MS) and is the first MS therapy that directly targets myelin repair. LINGO-1 is selectively expressed in brain and spinal cord but not in peripheral tissues. Perhaps the greatest concern for Li81 therapy is the limited access of the drug to the CNS. Here, we measured Li81 concentrations in brain, spinal cord, and cerebral spinal fluid in rats after systemic administration and correlated them with dose-efficacy responses in rat lysolecithin and experimental autoimmune encephalomyelitis spinal cord models of remyelination. Remyelination was dose-dependent, and levels of Li81 in spinal cord that promoted myelination correlated well with affinity measurements for the binding of Li81 to LINGO-1. Observed Li81 concentrations in the CNS of 0.1 to 0.4% of blood levels are consistent with values reported for other antibodies. To understand the features of the antibody that affect CNS penetration, we also evaluated the pharmacokinetics of Li81 Fab2, Fab, and poly(ethylene glycol)-modified Fab. The reagents all showed similar CNS exposure despite large differences in their sizes, serum half-lives, and volumes of distribution, and area under the curve (AUC) measurements in the CNS directly correlated with AUC measurements in serum. These studies demonstrate that exposure levels achieved by passive diffusion of the Li81 monoclonal antibody into the CNS are sufficient and lead to robust remyelination.

  14. Dorsal border periaqueductal gray neurons project to the area directly adjacent to the central canal ependyma of the C4-T8 spinal cord in the cat

    NARCIS (Netherlands)

    Mouton, LJ; Kerstens, L; VanderWant, J; Holstege, G

    1996-01-01

    In a previous study horseradish peroxidase (HRP) injections in the upper thoracic and cervical spinal cord revealed some faintly labeled small neurons at the dorsal border of the periaqueductal gray (PAG). The present light microscopic and electronmicroscopic tracing study describes the precise loca

  15. Umbilical cord blood stem cells treatment for central pain: one case report%异体脐带血干细胞治疗中枢性疼痛一例

    Institute of Scientific and Technical Information of China (English)

    岳宝玲; 范亚林; 李香社; 郑丽; 侯会荣; 高丽昕

    2012-01-01

    Objective To study the effective of stem cell transplantation in a central pain patient. Methods There was a central pain case who had suffered central pain which resulted in brain ischemia-hypoxia. The patient was given 6 packets of umbilical cord blood stem cells transplantation ( 2 intravenous injection and 4 lumber puncture ). She was received stem cell treatment every 5 days. We observed the condition of central pain for 2 months. Result There was some improvement of pain and life quality from the 2rd stem cells transplantation. But patient felt pain after the 2rd month. Conclusion Umbilical cord blood stem cell transplantation was a new treatment method for central pain.%目的 探讨1例中枢性疼痛患者接受脐带血干细胞移植后症状转归.方法 随访我院1例脑缺血缺氧后中枢性疼痛患者给予2次静脉、4次腰穿鞘内注射途径移植干细胞,每次移植间隔5天,治疗后随访患者疼痛变化情况2个月.结果 从第2次脐带血干细胞移植开始,患者的疼痛情况及生活质量均有一定改善,移植结束后2个月患者疼痛又开始反复.结论 脐带血干细胞移植可能为中枢性疼痛的治疗提供新的治疗途径.

  16. Spinal and pelvic corrections in a patient with spondylocostal dysplasia syndrome and hemimyelomeningocele

    Directory of Open Access Journals (Sweden)

    Ali Al Kaissi

    2014-01-01

    Full Text Available Congenital malformation complex of the spine and the spinal cord can be a syndromic entity rather than a symptom complex. The spinal cord lesion is usually bilaterally symmetrical, but, there are occasional cases with one or more hemivertebrae, often associated with a central bony spur splitting the cord (diastematomyelia, in which one leg is virtually normal while the other is severely paralysed. Hemimyelomeningocele over the lumbar area may be associated with extensive spine malsegmentation compatible with the diagnosis of spondylocostal dysplasia syndrome. In this report, we present a 3-year-old girl who underwent neurological evaluation and spinal imaging studies for extensive spine malsegmentation compatible with spondylocostal dysostosis syndrome associated with hemimyelomeningocele. She had a series of corrective orthopaedic interventions to reconstruct her pelvic girdle and spine deformities, with a satisfactory outcome.

  17. Distribution of Wfs1 protein in the central nervous system of the mouse and its relation to clinical symptoms of the Wolfram syndrome

    DEFF Research Database (Denmark)

    Luuk, H.; Koks, S.; Plaas, M.;

    2008-01-01

    Mutations in the coding region of the WFS1 gene cause Wolfram syndrome, a rare multisystem neurodegenerative disorder of autosomal recessive inheritance. Patients with Wolfram syndrome display considerable clinical pleiomorphism, and symptoms such as neurological complications and psychiatric dis...... and psychiatric symptoms found in Wolfram syndrome. Enrichment of Wfs1 protein in the central extended amygdala suggests a role in the modulation of anxiety and fear Udgivelsesdato: 2008/8/20...

  18. Treatment of neuroblastoma in congenital central hypoventilation syndrome with a PHOX2B polyalanine repeat expansion mutation: New twist on a neurocristopathy syndrome.

    Science.gov (United States)

    Armstrong, Amy E; Weese-Mayer, Debra E; Mian, Amir; Maris, John M; Batra, Vandana; Gosiengfiao, Yasmin; Reichek, Jennifer; Madonna, Mary Beth; Bush, Jonathan W; Shore, Richard M; Walterhouse, David O

    2015-11-01

    Neuroblastoma in patients with congenital central hypoventilation syndrome (CCHS) as part of a neurocristopathy syndrome is a rare finding and has only been associated with paired-like homeobox 2b (PHOX2B) non-polyalanine-repeat-expansion mutations. To the best of our knowledge, we report the first case of a child with CCHS and Hirschsprung disease who had a PHOX2B polyalanine-repeat-expansion mutation (PARM) (genotype 20/33) and developed high-risk neuroblastoma. We further describe his treatment including chemotherapy and therapeutic I(131) -metaiodobenzylguanidine. This case highlights the need to consider neuroblastoma in patients with CCHS and the longest PHOX2B PARMs and to individualize treatment based on co-morbidities.

  19. Dor neuropática central após lesão medular traumática: capacidade funcional e aspectos sociais Dolor neuropático central después de lesión medular traumática: capacidad funcional y aspectos sociales Central neuropathic pain after traumatic spinal cord injury: functional capacity and social aspects

    Directory of Open Access Journals (Sweden)

    Janaina Vall

    2005-12-01

    Full Text Available Estudo de caso comparativo com o objetivo de avaliar a capacidade funcional e os aspectos sociais de dois pacientes, ambos com lesão medular traumática, sem e com dor neuropática central associada, respectivamente. Para avaliar a capacidade funcional, foi utilizado como instrumento o Functional Independence Measure ou Escala de Independência Funcional. E para avaliar os aspectos sociais foi construído o ecomapa de cada paciente, preconizado pelo modelo Calgary de avaliação de famílias. Ambos foram aplicados no domicílio do paciente. Os resultados mostraram que o paciente com dor neuropática central secundária à lesão medular possui baixa capacidade funcional e precária rede social de apoio, quando comparado com o paciente com as mesmas condições, porém sem dor associada.Estudio de caso comparativo con el objetivo de evaluar la capacidad funcional y los aspectos sociales de dos paciente, ambos con lesión medular traumática, sin y con el dolor neuropático central, respectivamente. Para evaluar la capacidad funcional, se usó como instrumento la Escala de Independencia Funcional. Y para evaluar los aspectos sociales, el ecomapa de cada paciente fue construido, preconizado por el modelo Calgary de evaluación de familias. Los dos furon aplicados en la casa del paciente. Los resultados mostraron que el paciente con dolor neuropatico central secundario a la lesión medular posee capacidad funcional baja y precaria red social de apoyo, cuando comparado con el paciente con las mismas condiciones, pero sin el dolor asociado.Comparative study of case with the aim of evaluating the functional capacity and social aspects of two patients, both with traumatic spinal cord injury, without and with central neuropathic pain associated, respectively. To evaluate the functional capacity it was used as instrument Functional Independence Measure. And to evaluate the social aspects the ecomap of each patient one it was built, extolled by the model

  20. The cerebral cost of breathing: an FMRI case-study in congenital central hypoventilation syndrome.

    Directory of Open Access Journals (Sweden)

    Mike Sharman

    Full Text Available Certain motor activities--like walking or breathing--present the interesting property of proceeding either automatically or under voluntary control. In the case of breathing, brainstem structures located in the medulla are in charge of the automatic mode, whereas cortico-subcortical brain networks--including various frontal lobe areas--subtend the voluntary mode. We speculated that the involvement of cortical activity during voluntary breathing could impact both on the "resting state" pattern of cortical-subcortical connectivity, and on the recruitment of executive functions mediated by the frontal lobe. In order to test this prediction we explored a patient suffering from central congenital hypoventilation syndrome (CCHS, a very rare developmental condition secondary to brainstem dysfunction. Typically, CCHS patients demonstrate efficient cortically-controlled breathing while awake, but require mechanically-assisted ventilation during sleep to overcome the inability of brainstem structures to mediate automatic breathing. We used simultaneous EEG-fMRI recordings to compare patterns of brain activity between these two types of ventilation during wakefulness. As compared with spontaneous breathing (SB, mechanical ventilation (MV restored the default mode network (DMN associated with self-consciousness, mind-wandering, creativity and introspection in healthy subjects. SB on the other hand resulted in a specific increase of functional connectivity between brainstem and frontal lobe. Behaviorally, the patient was more efficient in cognitive tasks requiring executive control during MV than during SB, in agreement with her subjective reports in everyday life. Taken together our results provide insight into the cognitive and neural costs of spontaneous breathing in one CCHS patient, and suggest that MV during waking periods may free up frontal lobe resources, and make them available for cognitive recruitment. More generally, this study reveals how the

  1. Multifocal spinal hemangioblastoma in von Hippel-Lindau syndrome: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ok Hwa [Dept. of Radiology, Inje University College of Medicine, Haeundae Paik Hospital, Busan (Korea, Republic of)

    2015-03-15

    Hemangioblastoma is a benign vascular neoplasm of the central nervous system that occurs frequently in the cerebellum and other areas of the central nervous system including spinal cord and brainstem. Spinal hemangioblastoma can present as a sporadic isolated lesion or as a component of von Hippel-Lindau syndrome. The author presents a case of 32-year-old man with von Hippel-Lindau syndrome and spinal hemangioblastomas represented by multiple small spinal lesions, with an emphasis on the magnetic resonance imaging findings and clinical characteristics of von Hippel-Lindau syndrome-associated spinal hemangioblastomas.

  2. Reversible cerebral vasoconstriction syndrome (RCVS) in antiphospholipid antibody syndrome (APLA): the role of centrally acting vasodilators. Case series and review of literature.

    Science.gov (United States)

    Gupta, Sarthak; Zivadinov, Robert; Ramasamy, Deepa; Ambrus, Julian L

    2014-12-01

    Reversible cerebral vasoconstriction syndrome (RCVS) is Raynaud's phenomenon of the brain. Changes in neurological function are dependent upon which areas of the brain are deprived of normal blood flow. Antiphospholipid antibody syndrome (APLA) is a common cause of Raynaud's phenomenon that can occur anywhere in the body, including the brain. Management of CNS vasospasm generally involves the use of centrally acting calcium channel blockers, which have been shown to relieve the associated headaches and transient neurological symptoms associated with it. Three patients with APLA and RCVS from our clinics are illustrated. It is demonstrated that the use of centrally acting calcium channel-blocking drugs, such as nimodipine, which prevent and reverse CNS vasospasm, led to clinical improvement in our patients over the course of 5-9 years. All of them had MRIs done at the initiation of therapy and 5-9 years after being on therapy. MRI measures of T2 lesion volumes (LVs) and number were obtained. All three patients had a good response in controlling clinical symptoms related to CNS vasospasm, Raynaud's phenomenon, visual disturbances, confusion, headaches, and hearing loss. There was also a resolution in the MRI findings of these patients. This case series of three patients shows a clinical improvement and decrease in T2 LV and number in patients with APLA and Raynaud's syndrome on centrally acting calcium channel blockers. RCVS is much more common than that currently appreciated. APLA is the common cause of RCVS. Further studies are needed to determine the optimal methods to diagnose RCVS and optimal therapies to treat it.

  3. Unilateral central retinal artery occlusion as the sole presenting sign of Susac syndrome in a young man: case report

    Directory of Open Access Journals (Sweden)

    Samira Luiza dos Apóstolos-Pereira

    2013-06-01

    Full Text Available We report the case of a 24-year-old man presenting with sudden visual loss in the left eye from a central retinal artery occlusion. An extensive clinical investigation revealed no etiology. Three weeks later, however, the patient developed hearing loss followed by encephalopathy and multiple branch retinal artery occlusions in the right eye. Fluorescein angiography confirmed retinal vascular occlusions with no sign of vasculitis. The neurological examination revealed a diffuse encephalopathy while the MRI scan disclosed several small areas of infarcts in the brain. Bilateral sensorineural hearing loss was confirmed on audiometry. The patient was diagnosed with Susac syndrome and treated with methylprednisolone and cyclophosphamide, resulting in slight improvement and stabilization. This case shows that Susac syndrome may be diagnosed late due to the absence at onset of one or more of the symptoms of the classic triad (encephalopathy, multiple branch retinal artery occlusions and hearing loss. This case also serves to emphasize that Susac syndrome should be considered in the differential diagnosis of central retinal artery occlusion, even in apparently healthy young men.

  4. Spinal Cord Contusion

    Institute of Scientific and Technical Information of China (English)

    Gong Ju; Jian Wang; Yazhou Wang; Xianghui Zhao

    2014-01-01

    Spinal cord injury is a major cause of disability with devastating neurological outcomes and lim-ited therapeutic opportunities, even though there are thousands of publications on spinal cord injury annually. There are two major types of spinal cord injury, transaction of the spinal cord and spinal cord contusion. Both can theoretically be treated, but there is no well documented treatment in human being. As for spinal cord contusion, we have developed an operation with fabulous result.

  5. Dispersal syndromes related to edge distance in cerrado sensu stricto fragments of central-western Brazil

    Directory of Open Access Journals (Sweden)

    André Vitor Fleuri Jardim

    2009-10-01

    Full Text Available The main selective forces affecting the fruiting strategies are related to the environment in which plants occur. As a savanna, microclimatic conditions should not vary in relation to distance from edge in cerrado sensu stricto fragments. Thus, we postulated that the importance of different dispersal syndromes would not vary towards the fragment core. Our aim was to test in four cerrado sensu stricto fragments in central Brazil whether the absolute density of anemo-, auto-, and zoochorous individuals varied in relation to edge distance. According to results, the absolute density of anemo-and autochorous individuals did not vary, whereas those of zoochorous individuals increased with edge distance, pointing out that there were other factors rather than abiotic conditions shifting zoochorous species to the interior of cerrado sensu stricto fragments.As principais forças seletivas que afetam as estratégias de frutificação estão relacionadas ao ambiente em que as plantas ocorrem. Como fragmentos de cerrado sensu stricto são savânicos, as condições microclimáticas não devem variar em relação à distância da borda. Assim, postulamos que a importância das diferentes síndromes de dispersão não varia da borda ao interior de um fragmento de vegetação. O objetivo deste trabalho foi testar, em quatro fragmentos de cerrado sensu stricto (centro-oeste do Brasil, se a densidade absoluta de indivíduos anemo, auto e zoocóricos variava em função da distância da borda. Segundo nossos resultados, a densidade absoluta de indivíduos anemo e autocóricos não variou significativamente borda em direção ao interior dos fragmentos, enquanto que a dos indivíduos zoocóricos aumentou, indicando que existem outros fatores, que não as condições abióticas, deslocando as espécies zoocóricas para o interior dos fragmentos de cerrado sensu stricto.

  6. Klinefelter's syndrome in azoospermic infertile males of Vidarbha region, Central India

    Directory of Open Access Journals (Sweden)

    Gajanan L. Maske

    2016-04-01

    Results: Three subjects had a chromosomal count of 47 in all the metaphase studied. The additional chromosome was closely matching with the X chromosome. Hence the karyotype showed numerical aberration with an extra and lsquo;X' chromosome i. e. 47, XXY suggestive of Klinefelter's syndrome. This was confirmed by G-banding. All of the three subjects had bilateral testicular atrophy and one had typical features of Klinefelter's syndrome except gynecomastia. Conclusions: On cytogenetic analysis of 30 azoospermic infertile subjects, chromosomal abnormality of 47, XXY (Klinefelter's syndrome was found in 3 subjects. The total percentage of Klinefelter's syndrome in present study comes to 10%. [Int J Res Med Sci 2016; 4(4.000: 1045-1050

  7. Animal models of Central Diabetes Insipidus: Human relevance of acquired beyond hereditary syndromes and the role of oxytocin.

    Science.gov (United States)

    Bernal, Antonio; Mahía, Javier; Puerto, Amadeo

    2016-07-01

    The aim of this study was to review different animal models of Central Diabetes Insipidus, a neurobiological syndrome characterized by the excretion of copious amounts of diluted urine (polyuria), a consequent water intake (polydipsia), and a rise in the serum sodium concentration (hypernatremia). In rodents, Central Diabetes Insipidus can be caused by genetic disorders (Brattleboro rats) but also by various traumatic/surgical interventions, including neurohypophysectomy, pituitary stalk compression, hypophysectomy, and median eminence lesions. Regardless of its etiology, Central Diabetes Insipidus affects the neuroendocrine system that secretes arginine vasopressin, a neurohormone responsible for antidiuretic functions that acts trough the renal system. However, most Central Diabetes Insipidus models also show disorders in other neurobiological systems, specifically in the secretion of oxytocin, a neurohormone involved in body sodium excretion. Although the hydromineral behaviors shown by the different Central Diabetes Insipidus models have usually been considered as very similar, the present review highlights relevant differences with respect to these behaviors as a function of the individual neurobiological systems affected. Increased understanding of the relationship between the neuroendocrine systems involved and the associated hydromineral behaviors may allow appropriate action to be taken to correct these behavioral neuroendocrine deficits.

  8. Imaging diagnosis--spinal cord histiocytic sarcoma in a dog.

    Science.gov (United States)

    Taylor, Amanda; Eichelberger, Bunita; Hodo, Carolyn; Cooper, Jocelyn; Porter, Brian

    2015-01-01

    A 12-year-old mixed breed dog was presented for evaluation of progressive paraparesis and ataxia. Magnetic resonance (MR) imaging was performed and identified multifocal intradural spinal cord mass lesions. The lesions were hyperintense in T2-weighted sequences, isointense to mildly hyperintense in T1-weighted sequences with strong contrast enhancement of the intradural lesions and spinal cord meninges. Spinal cord neoplasia was suspected. A diagnosis of intramedullary spinal cord histiocytic sarcoma, confined to the central nervous system, was confirmed histopathologically. Spinal cord histiocytic sarcoma is a rare neoplasm, but should be included in the differential diagnosis for dogs with clinical signs of myelopathy.

  9. Frontal lobe dementia syndrome as a first manifestation of primary angiitis of the central nervous system (PACNS).

    Science.gov (United States)

    Bönstrup, Marlene; Ott, Katja; Glatzel, Markus; Magnus, Tim

    2016-02-01

    This case presents a clinical course of a frontal lobe dysexecutive syndrome with dementia caused by a primary angiitis of the central nervous system (PACNS) of exclusively very small vessels. An isolated frontal lobe dementia syndrome as a primary manifestation of PACNS highlights the diverse clinical manifestations of the disease. The patient presented with a progressive cognitive decline with loss of memory, disinhibited behavior, inappropriate affect and frontal release signs. The diagnostic workup essentially revealed a lymphocytic pleocytosis in the cerebrospinal fluid and a generalized cortical atrophy without any vascular abnormalities. To grasp a diagnosis for this enigmatic clinical picture of a frontal lobe syndrome with signs of inflammation we targeted a tissue-based diagnosis. A brain biopsy gave the decisive hint towards a microvasculitis. Although the histopathologic picture showed peculiarities, a destruction of the vascular bed of very small vessels by lymphocytic infiltration was evident. Our case illustrates an uncommon clinical picture of a PACNS and points to shortcomings of the current histopathologic criteria if only very small vessels are involved.

  10. An experimental model for the transplantation of fetal central nervous system cells to the injured spinal cord in rats Modelo experimental de transplante de células do sistema nervoso central fetal para lesão de medula espinal em ratos

    Directory of Open Access Journals (Sweden)

    Tarcísio Eloy Pessoa de Barros Filho

    2002-01-01

    Full Text Available INTRODUCTION: Traumatic spinal cord injury is one of the most disabling conditions occurring in man and thus stimulates a strong interest in its histopathological, biochemical, and functional changes, primarily as we search for preventive and therapeutic methods. PURPOSE: To develop an experimental model for transplantation of cells from the fetal rat central nervous system to the site of an injured spinal cord of an adult rat in which the transplanted cells survive and become integrated. This experimental model will facilitate investigations of factors that promote regeneration and functional recovery after spinal cord trauma. MATERIAL AND METHODS: Fifteen adult Wistar rats underwent laminectomy, and an spinal cord lesion was made with microdissection. Fetal spinal cord tissue was then transplanted to the site of the injury. The rats were monitored over a 48-hour period, and then their vertebral column was completely removed for histological analysis. RESULTS: In 60% of transplanted rats, the fetal tissue at the injured site remained viable in the site of the lesion.INTRODUÇÃO: A lesão traumática da medula espinal consiste numa das mais incapacitantes lesões que o ser humano pode sofrer e tem despertado grande interesse no conhecimento das alterações histopatológicas, bioquímicas, funcionais e principalmente na busca de métodos de prevenção e tratamento. OBJETIVO: Propor um modelo experimental de transplante de células do sistema nervoso fetal de ratos para o sítio de lesão medular de ratos adultos que permitisse sua sobrevivência e integração para possibilitar protocolos de pesquisa para identificar outros fatores de regeneração e recuperação funcional pós trauma raquimedular. MATERIAL E MÉTODOS: Utilizaram-se 15 ratos adultos que foram submetidos a laminectomia e lesão de 5mm de hemimelula realizada com auxílio de microscópio óptico. Os ratos tiveram seu sítio de lesão medular transplantado com células do

  11. Defining incidence, risk factors, and impact on survival of central line-associated blood stream infections following hematopoietic cell transplantation in acute myeloid leukemia and myelodysplastic syndrome.

    Science.gov (United States)

    Lukenbill, Joshua; Rybicki, Lisa; Sekeres, Mikkael A; Zaman, Muhammad Omer; Copelan, Alexander; Haddad, Housam; Fraser, Thomas; DiGiorgio, Megan J; Hanna, Rabi; Duong, Hien; Hill, Brian; Kalaycio, Matt; Sobecks, Ronald; Bolwell, Brian; Copelan, Edward

    2013-05-01

    Central line-associated blood stream infections (CLABSI) commonly complicate the care of patients with acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS) after allogeneic stem cell transplantation (HCT). We developed a modified CLABSI (MCLABSI) definition that attempts to exclude pathogens usually acquired because of disruption of mucosal barriers during the vulnerable neutropenic period following HCT that are generally included under the original definition (OCLABSI). We conducted a retrospective study of all AML and MDS patients undergoing HCT between August 2009 and December 2011 at the Cleveland Clinic (N = 73), identifying both OCLABSI and MCLABSI incidence. The median age at transplantation was 52 years (range, 16 to 70); 34 had a high (≥3) HCT comorbidity index (HCT-CI); 34 received bone marrow (BM), 24 received peripheral stem cells (PSC), and 15 received umbilical cord blood cells (UCB). Among these 73 patients, 23 (31.5%) developed OCLABSI, of whom 16 (69.6%) died, and 8 (11%) developed MCLABSI, of whom 7 (87.5%) died. OCLABSI was diagnosed a median of 9 days from HCT: 5 days (range, 2 to 12) for UCB and 78 days (range, 7 to 211) for BM/PSC (P < .001). MCLABSI occurred a median of 12 days from HCT, with similar earlier UCB and later BM/PSC diagnosis (P = .030). Risk factors for OCLABSI in univariate analysis included CBC (P < .001), human leukocyte antigen (HLA)-mismatch (P = .005), low CD34(+) count (P = .007), low total nucleated cell dose (P = .016), and non-Caucasian race (P = .017). Risk factors for OCLABSI in multivariable analysis were UCB (P < .001) and high HCT-CI (P = .002). There was a significant increase in mortality for both OCLABSI (hazard ratio, 7.14; CI, 3.31 to 15.37; P < .001) and MCLABSI (hazard ratio, 6.44; CI, 2.28 to 18.18; P < .001). CLABSI is common and associated with high mortality in AML and MDS patients undergoing HCT, especially in UCB recipients and those with high HCT-CI. We propose

  12. Primary ciliary dyskinesia: Kartagener syndrome with central giant cell granuloma. A case report.

    Science.gov (United States)

    Türkoğlu, Kivanç; Orhan, Kaan; Demir, Pinar; Karabulut, Bariş; Can-Karabulut, Deniz C

    2010-10-01

    This paper describes a clinical case of both giant cell granuloma and Kartagener syndrome in a 15-year-old male patient, with emphasis on the radiographic aspects of this extremely unusual pathology. To our knowledge, the presence of these 2 rare clinical conditions in the same patient has not been previously reported.

  13. Spinal-cord syndrome due to non-compressive Paget's disease of bone: a spinal-artery steal phenomenon reversible with calcitonin.

    Science.gov (United States)

    Herzberg, L; Bayliss, E

    1980-07-05

    A 76-year-old man had progressive low back pain, leg weakness, and sensory loss. Radiology showed changes consistent with wide-spread Paget's disease, but no cord compression or involvement of nerve roots was detected by myelography or computerised axial tomography. His symptoms were relieved within 12 days of starting 100 MRC units of subcutaneous salmon calcitonin and recurred when calcitonin was discontinued for 5 days. The improvement continued on calcitonin treatment for 1 year, with falls in serum alkaline phosphatase and urinary hydroxyproline excretion. It is suggested that calcitonin treatment, in reducing the abnormally high metabolic activity of the diseased bone, and hence its vascular perfusion, allows more blood to reach the spinal cord.

  14. Vascular dysfunctions following spinal cord injury.

    Science.gov (United States)

    Popa, Constantin; Popa, Florian; Grigorean, Valentin Titus; Onose, Gelu; Sandu, Aurelia Mihaela; Popescu, Mihai; Burnei, Gheorghe; Strambu, Victor; Sinescu, Crina

    2010-01-01

    The aim of this article is to analyze the vascular dysfunctions occurring after spinal cord injury (SCI). Vascular dysfunctions are common complications of SCI. Cardiovascular disturbances are the leading causes of morbidity and mortality in both acute and chronic stages of SCI. Neuroanatomy and physiology of autonomic nervous system, sympathetic and parasympathetic, is reviewed. SCI implies disruption of descendent pathways from central centers to spinal sympathetic neurons, originating in intermediolateral nuclei of T1-L2 cord segments. Loss of supraspinal control over sympathetic nervous system results in reduced overall sympathetic activity below the level of injury and unopposed parasympathetic outflow through intact vagal nerve. SCI associates significant vascular dysfunction. Spinal shock occurs during the acute phase following SCI and it is a transitory suspension of function and reflexes below the level of the injury. Neurogenic shock, part of spinal shock, consists of severe arterial hypotension and bradycardia. Autonomic dysreflexia appears during the chronic phase, after spinal shock resolution, and it is a life-threatening syndrome of massive imbalanced reflex sympathetic discharge occurring in patients with SCI above the splanchnic sympathetic outflow (T5-T6). Arterial hypotension with orthostatic hypotension occurs in both acute and chronic phases. The etiology is multifactorial. We described a few factors influencing the orthostatic hypotension occurrence in SCI: sympathetic nervous system dysfunction, low plasma catecholamine levels, rennin-angiotensin-aldosterone activity, peripheral alpha-adrenoceptor hyperresponsiveness, impaired function of baroreceptors, hyponatremia and low plasmatic volume, cardiovascular deconditioning, morphologic changes in sympathetic neurons, plasticity within spinal circuits, and motor deficit leading to loss of skeletal muscle pumping activity. Additional associated cardiovascular concerns in SCI, such as deep vein

  15. Central obesity as a precursor to the metabolic syndrome in the AusDiab study and Mauritius.

    Science.gov (United States)

    Cameron, Adrian J; Boyko, Edward J; Sicree, Richard A; Zimmet, Paul Z; Söderberg, Stefan; Alberti, K George M M; Tuomilehto, Jaakko; Chitson, Pierrot; Shaw, Jonathan E

    2008-12-01

    Evidence from epidemiologic studies that central obesity precedes future metabolic change and does not occur concurrently with the appearance of the blood pressure, glucose, and lipid abnormalities that characterize the metabolic syndrome (MetS) has been lacking. Longitudinal surveys were conducted in Mauritius in 1987, 1992, and 1998, and in Australia in 2000 and 2005 (AusDiab). This analysis included men and women (aged > or = 25 years) in three cohorts: AusDiab 2000-2005 (n = 5,039), Mauritius 1987-1992 (n = 2,849), and Mauritius 1987-1998 (n = 1,999). MetS components included waist circumference, systolic blood pressure, fasting and 2-h postload plasma glucose, high-density lipoprotein (HDL) cholesterol, triglycerides, and homeostasis model assessment of insulin sensitivity (HOMA-S) (representing insulin sensitivity). Linear regression was used to determine which baseline components predicted deterioration in other MetS components over 5 years in AusDiab and 5 and 11 years in Mauritius, adjusted for age, sex, and ethnic group. Baseline waist circumference predicted deterioration (P Mauritius 1987-1992, and four of six in Mauritius 1987-1998. In contrast, an increase in waist circumference between baseline and follow-up was only predicted by insulin sensitivity (HOMA-S) at baseline, and only in one of the three cohorts. These results suggest that central obesity plays a central role in the development of the MetS and appears to precede the appearance of the other MetS components.

  16. Spinal cord injury reveals multilineage differentiation of ependymal cells.

    Science.gov (United States)

    Meletis, Konstantinos; Barnabé-Heider, Fanie; Carlén, Marie; Evergren, Emma; Tomilin, Nikolay; Shupliakov, Oleg; Frisén, Jonas

    2008-07-22

    Spinal cord injury often results in permanent functional impairment. Neural stem cells present in the adult spinal cord can be expanded in vitro and improve recovery when transplanted to the injured spinal cord, demonstrating the presence of cells that can promote regeneration but that normally fail to do so efficiently. Using genetic fate mapping, we show that close to all in vitro neural stem cell potential in the adult spinal cord resides within the population of ependymal cells lining the central canal. These cells are recruited by spinal cord injury and produce not only scar-forming glial cells, but also, to a lesser degree, oligodendrocytes. Modulating the fate of ependymal progeny after spinal cord injury may offer an alternative to cell transplantation for cell replacement therapies in spinal cord injury.

  17. Spinal cord injury reveals multilineage differentiation of ependymal cells.

    Directory of Open Access Journals (Sweden)

    Konstantinos Meletis

    2008-07-01

    Full Text Available Spinal cord injury often results in permanent functional impairment. Neural stem cells present in the adult spinal cord can be expanded in vitro and improve recovery when transplanted to the injured spinal cord, demonstrating the presence of cells that can promote regeneration but that normally fail to do so efficiently. Using genetic fate mapping, we show that close to all in vitro neural stem cell potential in the adult spinal cord resides within the population of ependymal cells lining the central canal. These cells are recruited by spinal cord injury and produce not only scar-forming glial cells, but also, to a lesser degree, oligodendrocytes. Modulating the fate of ependymal progeny after spinal cord injury may offer an alternative to cell transplantation for cell replacement therapies in spinal cord injury.

  18. [Toxic demyelination of the central nervous system. I. Effect of an intercalating gliotoxic drug on the spinal cord in Wistar rats].

    Science.gov (United States)

    Graça, D L

    1989-09-01

    Small volumes of ethidium bromide were injected into the dorsal columns of the spinal cord of Wistar rats. It was induced a demyelinating process, variable in nature and speed of repair according to the dose used. The induced lesions were classified into three groups (type I or fast lesions; type II or slow lesions; type III or intermediate lesions) according to the histological appearance and extension of remyelination. In some lesions or in areas within the same lesion, myelin and glial cell debris were rapidly processed by macrophages and the naked axons soon remyelinated by Schwann cells. In other lesions of similar duration, or areas within the lesions, myelin underwent transformation into mazes of membranes which persisted around the axons for long periods of time. In the lesions which contained such myelin-derived membranes, macrophages were scant and remyelination by Schwann cells was slow and arduous. It may be concluded that the slow resolution of some lesions resulted from the time elapsed between intoxication and disappearance of myelin-related cells. That time difference determined that the cellular responses to demyelination took place in an area devoid of glial cells and therefore without a support for the cellular migration needed for the removal of myelin debris and eventual remyelination. This investigation indicates that the development and outcome of demyelination may be altered by the cellular events that occur during oligodendrocyte degeneration.

  19. Central Sensitization Syndrome and the Initial Evaluation of a Patient with Fibromyalgia: A Review

    Directory of Open Access Journals (Sweden)

    Kevin C. Fleming

    2015-04-01

    Full Text Available In both primary care and consultative practices, patients presenting with fibromyalgia (FM often have other medically unexplained somatic symptoms and are ultimately diagnosed as having central sensitization (CS. Central sensitization encompasses many disorders where the central nervous system amplifies sensory input across many organ systems and results in myriad symptoms. A pragmatic approach to evaluate FM and related symptoms, including a focused review of medical records, interviewing techniques, and observations, is offered here, giving valuable tools for identifying and addressing the most relevant symptoms. At the time of the clinical evaluation, early consideration of CS may improve the efficiency of the visit, reduce excessive testing, and help in discerning between typical and atypical cases so as to avoid an inaccurate diagnosis. Discussion of pain and neurophysiology and sensitization often proves helpful.

  20. Atypical presentation of CLIPPERS syndrome: a new entity in the differential diagnosis of central nervous system rheumatologic diseases.

    Science.gov (United States)

    Gul, Maryam; Chaudhry, Ammar A; Chaudhry, Abbas A; Sheikh, Mubashir A; Carsons, Steven

    2015-04-01

    Numerous autoimmune diseases can affect the central nervous system (CNS), and variable clinical presentations confound the differential diagnosis. The challenging task of properly characterizing various CNS autoimmune diseases enables patients to be rapidly triaged and appropriately treated. In this review article, we aim to explore different CNS manifestations of rheumatologic diseases with emphasis on the utility of imaging and cerebrospinal fluid findings. We review the classic physical examination findings, characteristic imaging features, cerebrospinal fluid results, and serum biomarkers. In addition, we also present a unique case of newly described autoimmune entity CLIPPERS syndrome. Our case is unique in that this is the first case which demonstrates involvement of the supratentorial perivascular spaces in addition to the classic infratentorial involvement as initially described by Pittock et al (Brain. 2010;133:2626-2634).

  1. Association of CHRDL1 mutations and variants with X-linked megalocornea, Neuhauser syndrome and central corneal thickness.

    Directory of Open Access Journals (Sweden)

    Alice E Davidson

    Full Text Available We describe novel CHRDL1 mutations in ten families with X-linked megalocornea (MGC1. Our mutation-positive cohort enabled us to establish ultrasonography as a reliable clinical diagnostic tool to distinguish between MGC1 and primary congenital glaucoma (PCG. Megalocornea is also a feature of Neuhäuser or megalocornea-mental retardation (MMR syndrome, a rare condition of unknown etiology. In a male patient diagnosed with MMR, we performed targeted and whole exome sequencing (WES and identified a novel missense mutation in CHRDL1 that accounts for his MGC1 phenotype but not his non-ocular features. This finding suggests that MMR syndrome, in some cases, may be di- or multigenic. MGC1 patients have reduced central corneal thickness (CCT; however no X-linked loci have been associated with CCT, possibly because the majority of genome-wide association studies (GWAS overlook the X-chromosome. We therefore explored whether variants on the X-chromosome are associated with CCT. We found rs149956316, in intron 6 of CHRDL1, to be the most significantly associated single nucleotide polymorphism (SNP (p = 6.81×10(-6 on the X-chromosome. However, this association was not replicated in a smaller subset of whole genome sequenced samples. This study highlights the importance of including X-chromosome SNP data in GWAS to identify potential loci associated with quantitative traits or disease risk.

  2. Preschool weight and body mass index in relation to central obesity and metabolic syndrome in adulthood

    DEFF Research Database (Denmark)

    Graversen, Lise; Sørensen, Thorkild; Petersen, Liselotte

    2014-01-01

    Artiklen ser på vægt og BMI i alderen 0-5 år, og de to faktorers betydning for overvægt og metabolisk syndrom i voksenalderen. Studiet viser, at der er en lineær sammenhæng mellem vægt (0-1 år) og BMI (2-5 år) og senere BMI og taljeomfang. Studiet viser også, at børn, der ligger blandt de øverste 5...... % (sammenlignet med børn blandt de nederste 50 % i vægt og BMI) har en øget risiko, der stiger op gennem førskolealderen. Således har børn, der ligger blandt de øverste 5 % i BMI i 5-årsalderen en 6,2 gange større risiko for svær overvægt og en 2,5 gange større risiko for metabolisk syndrom som voksne. Det...... betyder, at en tredjedel af disse børn var svært overvægtige, og at en fjerdedel havde metabolisk syndrom som voksne. Undersøgelsen bygger på data for 2120 børn fra den populationsbaserede Northern Finland Birth Cohort (født i 1966), som er fulgt tæt med højde- og vægtmålinger i barnealderen og har...

  3. Metabolic syndrome in the rural population of Wardha, Central India: An exploratory factor analysis

    Directory of Open Access Journals (Sweden)

    Pradeep R Deshmukh

    2013-01-01

    Full Text Available Background and Objectives: Metabolic syndrome - a plausible precondition for type II diabetes and cardiovascular diseases is also on rise. To understand the mechanistic complexity of metabolic syndrome it is imperative to study the specific contribution of the determinants of metabolic syndrome. Such study can help to identify the most significant factor which may be of use in early detection as well as prevention efforts. Such information is scarcely available from India and especially from rural India. Hence, the present study was undertaken to explore for such factor which might be considered crucial for development of such pathogenesis particularly in rural population of Wardha. Methods: A cross-sectional study comprising of 300 subjects was carried out in rural area of Primary Health Center, attached to medical college with approximate 31,000 populations. The anthropometric parameters such as height, weight, waist circumference were measured. Overnight fasting samples were collected for lipid profile (total cholesterol, triglyceride, high density lipoproteins, low density lipoproteins, very low density lipoproteins and fasting blood glucose levels. The National Cholesterol Education Programme Adult Treatment Panel, ATP-III guidelines were used to categorize the study subjects. As many of the variables are highly intercorrelated, exploratory factor analysis was carried out to reduce the data to a smaller number of independent factors that accounts for the most of the variances in the data. Principal component analysis was used as a method of extraction. Results: For both sexes, three factors were extracted accounting for about 71% variance in the measured variables. An adiposity factor which accounted for highest explained variance (28%, was the initial factor extracted. It was loaded positively by waist circumference, triglyceride, and very low density lipoprotein and negatively loaded by high density lipoprotein. Second factor extracted

  4. Nevirapine-induced Stevens-Johnson syndrome from a tertiary care hospital in Central India

    Directory of Open Access Journals (Sweden)

    Vandana Badar

    2014-04-01

    Full Text Available A 42 year old man, a known case of AIDS receiving antiretroviral therapy – stavudine, lamivudine, and nevirapine since 4 weeks, was admitted with erythematous rash, with blisters, and abnormal liver function test. A diagnosis of Steven-Johnson syndrome was made excluding other opportunistic infection and differential diagnosis. Nevirapine was discontinued until the patient recovered. After recovery patient was started with stavudine, lamivudine, and efavirenz treatment without re-challenge with nevirapine. [Int J Basic Clin Pharmacol 2014; 3(2.000: 408-409

  5. Hypocretin Deficiency Associated with Narcolepsy Type 1 and Central Hypoventilation Syndrome in Neurosarcoidosis of the Hypothalamus.

    Science.gov (United States)

    Mayo, Mary Catherine; Deng, Jane C; Albores, Jeffrey; Zeidler, Michelle; Harper, Ronald M; Avidan, Alon Y

    2015-09-15

    We report a case of a 53-year-old man presenting with depressed alertness and severe excessive sleepiness in the setting of neurosarcoidosis. Neuroimaging demonstrated hypothalamic destruction due to sarcoidosis with a CSF hypocretin level of 0 pg/mL. The patient also experienced respiratory depression that presumably resulted from hypocretin-mediated hypothalamic dysfunction as a result of extensive diencephalic injury. This is a novel case, demonstrating both hypocretin deficiency syndrome, as well as respiratory dysfunction from destruction of hypocretin neurons and extensive destruction of key diencephalic structures secondary to the underlying neurosarcoidosis.

  6. Spinal Cord Dysfunction (SCD)

    Data.gov (United States)

    Department of Veterans Affairs — The Spinal Cord Dysfunction (SCD) module supports the maintenance of local and national registries for the tracking of patients with spinal cord injury and disease...

  7. Adult spinal cord ependymal layer: A promising pool of quiescent stem cells to treat spinal cord injury

    OpenAIRE

    Stavros eMalas; Elena ePanayiotou

    2013-01-01

    Spinal cord injury is a major health burden and currently there is no effective medical intervention. Research performed over the last decade revealed that cells surrounding the central canal of the adult spinal cord and forming the ependymal layer acquire stem cell properties either in vitro or in response to injury. Following spinal cord injury activated ependymal cells generate progeny cells which migrate to the injury site but fail to produce the appropriate type of cells in sufficient nu...

  8. Spinal Cord Injuries

    Science.gov (United States)

    ... forth between your body and your brain. A spinal cord injury disrupts the signals. Spinal cord injuries usually begin with a blow that fractures or ... down on the nerve parts that carry signals. Spinal cord injuries can be complete or incomplete. With a complete ...

  9. APOPTOSIS AFTER SPINAL CORD INJURY IN RATS

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Objective To confirm the role played by apoptosis in spinal cord injury. Methods 36 rats models of spinal cord injury were made by Allen method. Histological examinations using HE staining and in situ end-labeling were used to observe apoptosis in spinal cord tissues from 1h to 21d after injury. Results HE staining sections showed hemorrhage and necrosis, neuronal degeneration and gliai cell proliferation. In situ end-labeling sections showed the appearance of apoptosis in both gray and white matter as well as in both central and surrounding region. The number of apoptotic cells increased from 12h after injury, increased to the peak at 4d and declined to normal at 21d. Conclu sion The results suggest that apoptosis, especially glial apoptosis, plays a role in the pathogenesis of spinal cord in jury.

  10. Pathology of tissue loss (white syndrome) in Acropora sp. corals from the Central Pacific

    Science.gov (United States)

    Work, Thierry M.; Aeby, Greta S.

    2011-01-01

    We performed histological examination of 69 samples of Acropora sp. manifesting different types of tissue loss (Acropora White Syndrome-AWS) from Hawaii, Johnston Atoll and American Samoa between 2002 and 2006. Gross lesions of tissue loss were observed and classified as diffuse acute, diffuse subacute, and focal to multifocal acute to subacute. Corals with acute tissue loss manifested microscopic evidence of necrosis sometimes associated with ciliates, helminths, fungi, algae, sponges, or cyanobacteria whereas those with subacute tissue loss manifested mainly wound repair. Gross lesions of AWS have multiple different changes at the microscopic level some of which involve various microorganisms and metazoa. Elucidating this disease will require, among other things, monitoring lesions over time to determine the pathogenesis of AWS and the potential role of tissue-associated microorganisms in the genesis of tissue loss. Attempts to experimentally induce AWS should include microscopic examination of tissues to ensure that potentially causative microorganisms associated with gross lesion are not overlooked.

  11. Central nervous system mechanisms contributing to the cachexia-anorexia syndrome.

    Science.gov (United States)

    Plata-Salamán, C R

    2000-10-01

    The cachexia-anorexia syndrome occurs in chronic pathophysiologic processes including cancer, infection with human immunodeficiency virus, bacterial and parasitic diseases, inflammatory bowel disease, liver disease, obstructive pulmonary disease, cardiovascular disease, and rheumatoid arthritis. Cachexia makes an organism susceptible to secondary pathologies and can result in death. Cachexia-anorexia may result from pain, depression or anxiety, hypogeusia and hyposmia, taste and food aversions, chronic nausea, vomiting, early satiety, malfunction of the gastrointestinal system (delayed digestion, malabsorption, gastric stasis and associated delayed emptying, and/or atrophic changes of the mucosa), metabolic shifts, cytokine action, production of substances by tumor cells, and/or iatrogenic causes such as chemotherapy and radiotherapy. The cachexia-anorexia syndrome also involves metabolic and immune changes (mediated by either the pathophysiologic process, i.e., tumor, or host-derived chemical factors, e.g., peptides, neurotransmitters, cytokines, and lipid-mobilizing factors) and is associated with hypertriacylglycerolemia, lipolysis, and acceleration of protein turnover. These changes result in the loss of fat mass and body protein. Increased resting energy expenditure in weight-losing cachectic patients can occur despite the reduced dietary intake, indicating a systemic dysregulation of host metabolism. During cachexia, the organism is maintained in a constant negative energy balance. This can rarely be explained by the actual energy and substrate demands by tumors in patients with cancer. Overall, the cachectic profile is significantly different than that observed during starvation. Cachexia may result not only from anorexia and a decreased caloric intake but also from malabsorption and losses from the body (ulcers, hemorrhage, effusions). In any case, the major deficit of a cachectic organism is a negative energy balance. Cytokines are proposed to participate

  12. Characteristics of functional recovery of spinal cord after operation for central spinal cord injury: 11 cases analysis%中央型脊髓损伤患者术后脊髓功能恢复特征:11例分析

    Institute of Scientific and Technical Information of China (English)

    李翔; 刘锋; 吴乃庆; 王道新

    2005-01-01

    背景:颈段脊髓损伤的治疗有多种方式,但效果均不十分理想.目的:探讨手术等综合治疗对颈椎中央型脊髓损伤患者术后功能恢复的影响.设计:前后对照设计.单位:一所大学医院的骨科病房.对象:1999-01/2003-01江苏省人民医院骨科共收治中央型脊髓损伤患者11例,均为男性,年龄36~65岁,交通事故9例,跌伤1例,头部砸伤1例,病程2 h~14 d,伴颈椎间盘突出9例,颈椎管狭窄1例,C6椎体骨折1例.MRI片单处损伤9例,两处损伤2例.方法:伤后4~20 d手术治疗,9例行前路颈椎间盘摘除植骨加钢板内固定,2例行颈椎后路减压植骨加侧块钢板内固定.主要观察指标:①患者手术前后Frankel分级.②手术后脊髓功能恢复结果.结果:11例患者术前Frankel分级为0级3例、Ⅰ级3例、Ⅱ级5例.术后为0级1例、Ⅲ级4例、Ⅳ级6例.平均肌力恢复2~5级,复查内固定牢靠、在位,植骨愈合良好.结论:颈椎中央型脊髓损伤一旦确诊提倡以手术为主的综合性治疗措施,手术尽可能去除致病因素,其他辅助措施则以促进脊髓功能恢复为目的.%BACKGROUND: There are many treatment approaches of the cervical spinal cord injury but the results were all unpleased.OBJECTIVE: To discuss the effect of the operation and other colligate treatments on the functional recovery of the patients who suffered from the cervical central spinal cord injury.DESIGN: A preoperative and postoperative control study.SETTING: Orthopaedic department in an affiliated hospital of a university.PARTICIPANTS: Eleven male patients of the central spinal cord injury,whose ages ranged from 36 to 65, were chosen from the Orthopedic Department of People' s Hospital of Jiangsu Province from January 1999 to January 2003. Nine cases suffered from traffic accidents. One case suffered from falling injury. One case suffered from head smash injury. The courses of diseases were from 2 hours to 14 days. Nine cases were

  13. 脊柱裂致脊髓栓系综合征患者性功能现状评估%Evaluation of sexual function in men with tethred cord syndrome associated with spina bifida

    Institute of Scientific and Technical Information of China (English)

    马彦; 韩晓敏; 汪隆旺; 李兵; 潘峰

    2012-01-01

    Objective:To assess sexual function in men with tethered cord syndrome (TCS) caused by Spinal Bifida. Methods: A cross-sectional study was performed in 16 adult men with TCS who had have stabilized sexual partners in recent 10 years. The International Index of Erectile Function (IIEF) and Erection Hardness Score (EHS) questionnaire was mailed to each man. Results:Of the 16 patients with TCS, 12 completed the IIEF questionnaire and EHS finally. Of the 12 men who replied, 10 (83%) had erectile dysfunction (ED). 70% of the patients were mild to moderate and moderate ED. Only 1 patients (8%) with TCS developed grade 4 on the EHS, and 58% of the patients developed grade 3. Conclusions:The incidence rate of ED in TCS patients was high. IIEF is equally effective to assess sexual function in men with TCS.%目的:研究脊柱裂(spina bifida,SB)致脊髓栓系综合征(tethered cord syndrome,TCS)患者勃起功能状况.方法:近10年来收治TCS患者中,成年男性且有稳定性伴侣者16例.采用邮件问卷方法,对这些患者行国际勃起功能指数(IIEF)问卷及阴茎硬度分级问卷评估.结果:16例TCS患者中,4例未完成问卷.余12例患者中,10例(83%)诊断为勃起功能障碍(erectile dysfunction,ED),其中轻-中度和中度ED占总例数的70%.仅有1例(8%)TCS患者阴茎硬度达到4级,阴茎达到3级硬度的占58%.结论:TCS患者有较高的ED发生.IIEF自评量表能很好地反映TCS患者ED的发病情况.

  14. Bevacizumab for the treatment of surgically unresectable cervical cord hemangioblastoma: a case report

    Directory of Open Access Journals (Sweden)

    Omar Ayman I

    2012-08-01

    Full Text Available Abstract Introduction Hemangioblastomas are highly vascular tumors that can arise within the central nervous system as well as other organ systems within the body. They can arise sporadically or as part of von Hippel-Lindau syndrome. Those arising in critical locations within the central nervous system can be difficult to resect surgically and therefore pose a significant challenge and result in morbidity and even mortality. Hemangioblastomas express high levels of vascular endothelial growth factor that drives angiogenesis and tumor progression. We hypothesized that bevacizumab through its inhibitory effect on vascular endothelial growth factor will result in hemangioblastoma tumor regression as well as a meaningful clinical response. Case presentation We present the case of a 51-year-old Caucasian man with surgically unresectable cervical cord hemangioblastoma presenting with progressive weakness leading to quadriparesis. He was treated with bevacizumab and his follow up magnetic resonance imaging scans showed marked tumor regression. After only six cycles of intravenous bevacizumab (10mg/kg every two weeks, he started ambulating after being wheelchair bound. He is currently still receiving treatment almost two years after initiation of bevacizumab. Conclusions We have shown for the first time that bevacizumab can result in significant tumor regression and a sustained clinical improvement in a patient with an otherwise unresectable spinal cord hemangioblastoma. This novel approach can be immensely useful for patients with difficult to resect hemangioblastomas or those with multiple lesions such as in von Hippel-Lindau syndrome.

  15. Does repair of spinal cord injury follow the evolutionary theory?

    Institute of Scientific and Technical Information of China (English)

    Zhicheng Zhang; Fang Li; Tiansheng Sun

    2012-01-01

    Lower vertebrates, such as fish and amphibians, and higher vertebrates in embryonic development can acquire complete regeneration of complex body structures, including the spinal cord, an important part of the central nervous system. However, with species evolution and development, this regenerative capacity gradually weakens and even disappears, but the cellular and molecular mechanisms remain poorly understood. We explored the differences in mechanisms of spinal cord regeneration capability between lower and higher vertebrates, investigated differences in their cellular and molecular mechanisms and between the spinal cord structures of lower vertebrates and mammals, such as rat and monkey, to search for theoretical evidence and therapeutic targets for nerve regeneration in human spinal cord.

  16. Ictal central apnea and bradycardia in temporal lobe epilepsy complicated by obstructive sleep apnea syndrome

    Directory of Open Access Journals (Sweden)

    Yoko Nishimura

    2015-01-01

    Full Text Available We describe the case of a 12-year-old boy who developed temporal lobe epilepsy (TLE with daily complex partial seizures (CPS and monthly generalized seizures. Moreover, he frequently snored while asleep since early childhood. Polysomnography (PSG revealed severe obstructive sleep apnea with apnea–hypopnea index (AHI of 37.8/h. Video-PSG with simultaneous electroencephalography (EEG recording captured two ictal apneic episodes during sleep, without any motor manifestations. The onset of rhythmic theta activity in the midtemporal area on EEG was preceded by the onset of apnea by several seconds and disappeared soon after cessation of central apnea. One episode was accompanied by ictal bradycardia of <48 beats/min which persisted for 50 s beyond the end of epileptic activity. After treatment with carbamazepine and tonsillectomy/adenoidectomy, the seizures were well controlled and AHI decreased to 2.5/h. Paroxysmal discharges also disappeared during this time. Uncontrolled TLE complicated by sleep apnea should be evaluated for the presence of ictal central apnea/bradycardia.

  17. Spasticity: the misunderstood part of the upper motor neuron syndrome.

    Science.gov (United States)

    Ivanhoe, Cindy B; Reistetter, Timothy A

    2004-10-01

    Spasticity is a sensorimotor phenomenon related to the integration of the nervous system motor responses to sensory input. Although most commonly considered a velocity-dependent increase to tonic stretch, it is related to hypersensitivity of the reflex arc and changes that occur within the central nervous system, most notably, the spinal cord. Injury to the central nervous system results in loss of descending inhibition, allowing for the clinical manifestation of abnormal impulses. Muscle activity becomes overactive. This is mediated at several areas of the stretch-reflex pathway. Although spasticity is part of the upper motor neuron syndrome, it is frequently tied to the other presentations of the said syndrome. Contracture, hypertonia, weakness, and movement disorders can all coexist as a result of the upper motor neuron syndrome. Although basic science descriptions of spasticity are being elucidated, clinically, confusion exists.

  18. Role of central histaminergic system in lorazepam withdrawal syndrome in rats.

    Science.gov (United States)

    Nath, C; Gupta, M B

    2001-04-01

    Effects of histaminergic agonists and antagonists were investigated on withdrawal signs in lorazepam-dependent rats. Physical dependence was developed by giving lorazepam admixed with the food in the following dose schedule (in mg/kg given daily x days): 10 x 4, 20 x 4, 40 x 4, 80 x 4, and 120 x 7. The parameters observed during the periods of administration of lorazepam and after its withdrawal were spontaneous locomotor activity (SLA), reaction time to pain, foot shock aggression (FSA), and audiogenic seizures. During the withdrawal period, the rats were divided into groups of 10 each. Control-withdrawal group did not receive any drug. The drugs (in mg/kg administered intramuscularly)--L-histidine (50), histamine-N-methyl (2), promethazine (10), pheniramine (10), astemizole (10), and thioperamide (1)--were given separately in other groups daily during the withdrawal period. The withdrawal signs in control group were hyperkinesia, hyperaggression, and audiogenic seizures. L-Histidine, precursor of histamine, and thioperamide, antagonist of H3 receptor, potentiated hyperkinesia, hyperaggression, and audiogenic seizures. Histamine-N-methyl, agonist of H3 receptor, and H1 receptor antagonists, promethazine and pheniramine, blocked all the withdrawal signs. Astemizole, a peripheral antagonist of H1 receptor, could not affect any withdrawal sign. It may be concluded that histamine H1 receptors are facilitatory and H3 receptors are inhibitory for benzodiazepine (BZD) withdrawal syndrome.

  19. A rare case of concomitant sicca keratopathy and ipsilateral central facial palsy in Wallenberg’s dorsolateral medullary syndrome

    Science.gov (United States)

    De Bruyn, Deborah; Van Aken, Elisabeth; Herman, Kristien

    2017-01-01

    Objective: To describe a patient with a right-sided supranuclear facial palsy and concomitant sicca keratopathy of the right eye following right-sided dorsolateral medullary infarction. Methods: Our patient underwent a complete ophthalmologic and neurologic examination including biomicroscopy, fundus examination, cranial nerve examination, Shirmer I test, and magnetic resonance imaging of the brain. Results: A 61-year-old woman presented in emergency with a central facial nerve palsy on the right side and truncal ataxia. Neurologic assessment revealed a concurrent dysphagia, dysarthria, hypoesthesia of the right face, and weakness of the right upper limb. Magnetic resonance imaging of the brain showed an old left-sided cerebellar infarction, but a recent ischemic infarction at the level of the right dorsolateral medulla oblongata was the cause of our patient’s current problems. One month after diagnosis of the right-sided dorsolateral medullary syndrome, there were complaints of ocular irritation and a diminished visual acuity in the right eye. Biomicroscopy showed a sicca keratopathy with nearly complete absence of tear secretion on the Shirmer I test, but with normal eye closure and preserved corneal reflexes and sensitivity. Conclusion: A dorsolateral medullary syndrome can have a variable expression in symptomatology. Our case is special because of the combination of an ipsilateral supranuclear facial palsy with normal upper facial muscle function together with an ipsilateral sicca keratopathy as a result of a nearly absent tear secretion. We hypothesized that the mechanism underlying the patient’s sicca keratopathy ipsilateral to the supranuclear facial palsy involved the superior salivatory nucleus, which is situated in the caudal pons inferiorly of the motor facial nucleus and is most probably affected by a superior extension of the infarcted area in the right medulla oblongata. PMID:28293537

  20. Ependymal variations in the caudal spinal cord.

    Science.gov (United States)

    Stoltenburg-Didinger, G; Bienentreu, R

    1981-01-01

    The lumbosacral spinal cord including the cauda equina was examined in 100 unselected autopsied cases of infants dying at or near term. A striking finding in 10% of these cases was the occurrence of bilateral collections of ependymal cells lying in rows on the surface of the lumbosacral cord. In another 25% the central canal was forked or duplicate. These foci were not associated with evidence of defective neural tube closure or spina bifida; the central canal was of normal length. There was no relationship between the ependymal structures and systemic clinical or pathological findings. The pathogenesis and significance of these lesions is unclear. Our observations suggest that they occur regularly and are not associated with neurological disturbances. The relationship of the ependymal cell collections to ependymomas of the caudal spinal cord is open to speculation.

  1. Central Anticholinergic Syndrome due to Hypoxia-Induced Bradycardia in a Child with Difficult Intubation Undergoing Complete Dental Restoration: A Case Report.

    Science.gov (United States)

    Gharavifard, Mohamad; Razavi, Majid; Ghandehari Motlagh, Mehdi; Ziyaeifard, Mohsen

    2014-09-01

    Central anticholinergic syndrome (CAS) following general anesthesia (GA) is a well known syndrome in children and adults. Many cases of CAS have been previously reported in the literature. However, there are only two reports of post resuscitation CAS after administration of small doses of atropine. Hereby, we report a case of CAS in a child undergoing complete dental restoration under GA after receiving a small dose of atropine to reverse hypoxia induced bradycardia. Intraoperative events such as hypoxia or cardiac arrest may play a role as triggers for CAS. However, we cannot establish a causal relationship between the occurrence of CAS and such critical events.

  2. Central Anticholinergic Syndrome due to Hypoxia-Induced Bradycardia in a Child with Difficult Intubation Undergoing Complete Dental Restoration: A Case Report.

    Directory of Open Access Journals (Sweden)

    Mohamad Gharavifard

    2014-10-01

    Full Text Available Central anticholinergic syndrome (CAS following general anesthesia (GA is a well known syndrome in children and adults. Many cases of CAS have been previously reported in the literature. However, there are only two reports of post resuscitation CAS after administration of small doses of atropine. Hereby, we report a case of CAS in a child undergoing complete dental restoration under GA after receiving a small dose of atropine to reverse hypoxia induced bradycardia. Intraoperative events such as hypoxia or cardiac arrest may play a role as triggers for CAS. However, we cannot establish a causal relationship between the occurrence of CAS and such critical events.

  3. Implanted central venous catheter-related acute superior vena cava syndrome: management by metallic stent and endovascular repositioning of the catheter tip

    Energy Technology Data Exchange (ETDEWEB)

    Qanadli, S.D.; Mesurolle, B.; Sissakian, J.F.; Chagnon, S.; Lacombe, P. [Service de Radiologie, Hopital Ambroise Pare, 92 - Boulogne (France)

    2000-08-01

    We describe a case of a 49-year-old woman with stage-IIIB lung adenocarcinoma who experienced an acute superior vena cava syndrome related to an implanted central venous catheter without associated venous thrombosis. The catheter was surgically implanted for chemotherapy. Superior vena cava syndrome appeared after the procedure and was due to insertion of the catheter through a subclinical stenosis of the superior vena cava. Complete resolution of the patient's symptoms was obtained using stent placement and endovascular repositioning of the catheter tip. (orig.)

  4. Neuropathological changes of central nervous system in Guillain-Barré syndrome.%吉兰-巴雷综合征中枢神经系统病理改变

    Institute of Scientific and Technical Information of China (English)

    林世和; 赵节绪; 江新梅; 宋晓南

    2001-01-01

    Objective  To investigate the neuropathological changes of central nervous system in Guillain-Barré syndrom. Methods Brain, spinal cord and sciatic nerve were obtained from 22 cases of Guillain-Barré syndrome. Eight cases were examined by general autopsy, 14 cases were examined by limited autopsy. HE, KB, Bielschowsky, Weil and Sudan Ⅲ staining were carried out, the sections were observed by light microscopy. Results 1.Cerebral superficial veins congested, widening of the cortical sulci, narrowed gyri and mild cerebellar tonsillar hernia were present. 2. Majority of cerbral neurons presented an ischemic changes. Slightly loss of hippocampal pyramidal neurons were found. There was chromatolysis of motor neurons of brain stem. Lymphocytic infiltration around the small vessels occurred in the pons and medullary oblongata in 8 cases. Focal demyelination was noted in pons and frontal white matter in 2 cases. Loss of Purkinje cells and appearance of glial nodules were observed in molecular layer of cerebellum. 3. Swellin, central chromatolysis and eccentric nuclei of anterior horn cells appeared in 16 cases, which were pronounced in cervical and lumbal segment of spinal cord. Vaculated neuroplasma and lymphacytic infiltrition could be seen. 4. Segmental demyelination and lymphocytic infiltration were the main neuropathological changes observed in 20 cases. There were two other cases in which the axon were severely involved, which showed swelling and breakdown of axons and as well as axonal bulbs. Conclusions 1. Lymphocytic infiltration in brain stem and spinal cord were in continuousness of pathological changes of peripheral nerves. 2. Finding of glial nodules suggested that there was possibility of infection of neurotropic virus. 3. Occurence of focal demyelination in cerebrum and brain stem indicated that Guillain-Barré syndrome may have combined involvement of central and peripheral nervous system.%目的观察

  5. Acute Cerebrovascular Radiation Syndrome: Radiation Neurotoxicity , mechanisms of CNS radiation injury, advanced countermeasures for Radiation Protection of Central Nervous System.

    Science.gov (United States)

    Popov, Dmitri; Jones, Jeffrey; Maliev, Slava

    Key words: Cerebrovascular Acute Radiation Syndrome (Cv ARS), Radiation Neurotoxins (RNT), Neurotransmitters, Radiation Countermeasures, Antiradiation Vaccine (ArV), Antiradiation Blocking Antibodies, Antiradiation Antidote. Psychoneuroimmunology, Neurotoxicity. ABSTRACT: To review the role of Radiation Neurotoxins in triggering, developing of radiation induced central nervous system injury. Radiation Neurotoxins - rapidly acting blood toxic lethal agent, which activated after irradiation and concentrated, circulated in interstitial fluid, lymph, blood with interactions with cell membranes, receptors and cell compartments. Radiation Neurotoxins - biological molecules with high enzymatic activity and/or specific lipids and activated or modified after irradiation. The Radiation Neurotoxins induce increased permeability of blood vessels, disruption of the blood-brain barrier, blood-cerebrospinal fluid (CSF) barrier and developing severe disorder of blood macro- and micro-circulation. Principles of Radiation Psychoneuro-immunology and Psychoneuro-allergology were applied for determination of pathological processes developed after irradiation or selective administration of Radiation Neurotoxins to radiation naïve mammals. Effects of radiation and exposure to radiation can develop severe irreversible abnormalities of Central Nervous System, brain structures and functions. Antiradiation Vaccine - most effective, advanced methods of protection, prevention, mitigation and treatment and was used for of Acute Radiation Syndromes and elaboration of new technology for immune-prophylaxis and immune-protection against ϒ, Heavy Ion, Neutron irradiation. Results of experiments suggested that blocking, antitoxic, antiradiation antibodies can significantly reduce toxicity of Radiation Toxins. New advanced technology include active immune-prophylaxis with Antiradiation Vaccine and Antiradiation therapy that included specific blocking antibodies to Radiation Neurotoxins

  6. BURNOUT SYNDROME, JOB SATISFACTION LEVELS AND RELATED FACTORS IN CENTRAL TRABZON PROVINCE PRIMARY HEALTH CENTER WORKERS

    Directory of Open Access Journals (Sweden)

    Asuman YAVUZYILMAZ

    2007-02-01

    Full Text Available Burnout manifests itself in individuals working in professions involving face-to-face contact with the public in depersonalization towards others, feelings of emotional exhaustion, and reduced feelings of personal achievement and adequacy. The objective in this study was to determine burnout and job satisfaction levels and related factors in primary health center personnel in the central part of the Turkish province of Trabzon. A total of 227 people working in central Trabzon province primary health centers participated in this cross-sectional study, a level of 90.4%. The Maslach Burnout Inventory was used to determine burnout level and the Job Satisfaction Inventory for job satisfaction. Burnout levels in health personnel were high among women (15.06±5.57, married individuals (14.80±5.65 and those dissatisfied with their working conditions (16.80±5.81; physicians (5.00±2.79, those without children (5.19±2.54, those whose spouses were not working (4.69±2.70 and smokers (4.71±3.29 had a high level of depersonalization; and married individuals were determined to have a low personal achievement level (10.24±4.14 (p=0.020, p=0.028, p=0.011, p=0.038, p=0.028, p=0.012 and p=0.010, respectively. In conclusion, gender, marital status, age, satisfaction with working conditions and income level were determined to be related to burnout and job satisfaction. [TAF Prev Med Bull 2007; 6(1.000: 41-50

  7. BURNOUT SYNDROME, JOB SATISFACTION LEVELS AND RELATED FACTORS IN CENTRAL TRABZON PROVINCE PRIMARY HEALTH CENTER WORKERS

    Directory of Open Access Journals (Sweden)

    Murat TOPBAS

    2007-02-01

    Full Text Available Burnout manifests itself in individuals working in professions involving face-to-face contact with the public in depersonalization towards others, feelings of emotional exhaustion, and reduced feelings of personal achievement and adequacy. The objective in this study was to determine burnout and job satisfaction levels and related factors in primary health center personnel in the central part of the Turkish province of Trabzon. A total of 227 people working in central Trabzon province primary health centers participated in this cross-sectional study, a level of 90.4%. The Maslach Burnout Inventory was used to determine burnout level and the Job Satisfaction Inventory for job satisfaction. Burnout levels in health personnel were high among women (15.06±5.57, married individuals (14.80±5.65 and those dissatisfied with their working conditions (16.80±5.81; physicians (5.00±2.79, those without children (5.19±2.54, those whose spouses were not working (4.69±2.70 and smokers (4.71±3.29 had a high level of depersonalization; and married individuals were determined to have a low personal achievement level (10.24±4.14 (p=0.020, p=0.028, p=0.011, p=0.038, p=0.028, p=0.012 and p=0.010, respectively. In conclusion, gender, marital status, age, satisfaction with working conditions and income level were determined to be related to burnout and job satisfaction. [TAF Prev Med Bull. 2007; 6(1: 41-50

  8. Central orchestration of peripheral nutrient partitioning and substrate utilization: implications for the metabolic syndrome.

    Science.gov (United States)

    Denis, R G P; Joly-Amado, A; Cansell, C; Castel, J; Martinez, S; Delbes, A S; Luquet, S

    2014-06-01

    Energy homoeostasis is maintained through a complex interplay of nutrient intake and energy expenditure. The central nervous system is an essential component of this regulation, as it integrates circulating signals of hunger and satiety to develop adaptive responses at the behavioural and metabolic levels, while the hypothalamus is regarded as a particularly crucial structure in the brain in terms of energy homoeostasis. The arcuate nucleus (ARC) of the hypothalamus contains at least two intermingled neuronal populations: the neurons that produce neuropeptide Y (NPY); and the Agouti-related protein (AgRP) produced by AgRP/NPY neurons situated below the third ventricle in close proximity to proopiomelanocortin (POMC)-producing neurons. POMC neurons exert their catabolic and anorectic actions by releasing α-melanocyte-stimulating hormone (α-MSH), while AgRP neurons oppose this action by exerting tonic GABAergic inhibition of POMC neurons and releasing the melanocortin receptor inverse agonist AgRP. The release of neurotransmitters and neuropeptides by second-order AgRP neurons appears to take place on a multiple time scale, thereby allowing neuromodulation of preganglionic neuronal activity and subsequent control of nutrient partitioning - in other words, the coordinated regulation of conversion, storage and utilization of carbohydrates vs. lipids. This suggests that the function of AgRP neurons extends beyond the strict regulation of feeding to the regulation of efferent organ activity, such that AgRP neurons may now be viewed as an important bridge between central detection of nutrient availability and peripheral nutrient partitioning, thus providing a mechanistic link between obesity and obesity-related disorders.

  9. microRNA expression profiles of mononuclear cells from Down syndrome fetal cord blood%唐氏综合征胎儿脐带血单个核细胞microRNA差异表达研究

    Institute of Scientific and Technical Information of China (English)

    李武县; 徐勇; 李紫微; 乔王敏; 曾君; 戴勇; 涂植光

    2012-01-01

    Objective To detect the differentially expressed miRNAs in mononuclear cells from Down syndrome (DS) fetal cord blood. Methods Solexa high-throughput sequencing technology was conducted to evaluate the miRNA expression in 6 pairs of fetal cord blood mononuclear cells samples from DS fetuses (DS group) and normal fetuses (control group) . Stem-loop qRT-PCR was performed to verify the results of Solexa sequencing. Results A total of 167 miRNAs were found significantly dysregulated expression in the DS fetal cord blood mononuclear cells compared to the controls. And there were 8 up-regulated miRNAs and 159 down-regulated miRNAs in DS group. Moreover, 38 miRNAs and 139 miRNAs were specifically expressed in the DS group and control group, respectively. For the 5 miRNAs encoded by 21 chromosome, with the exception of miR-802, which was up-regulated in DS group, the other 4 Hsa21-derived miRNAs (i. e. , hsa-miR-99a, let-7c, miR-125b, and miR-155) displayed reduced expression. Conclusion These results suggested that there is a significant difference between the expression patterns of miRNAs in the DS group and control group, which may be related to the immune deficiency of DS fetus, and the decrease of T/B lymphocytes in DS fetal circulation. The significantly dysregulated and specifically expressed miRNAs are potential prenatal diagnosis biomarkers for DS fetus.%目的 检测唐氏综合征( Down syndrome,DS)胎儿和正常胎儿脐带血单个核细胞全基因组microRNA( miRNA)的差异表达.方法 运用Solexa高通量测序技术对6例DS胎儿(DS组)和6例正常胎儿(对照组)脐带血单个核细胞miRNA表达水平进行检测,比较分析2组miRNA表达的差异,并用Stem-loop qRT-PCR对Solexa测序结果进行验证.结果 2组样本显著性差异表达miRNA有167种,8种miRNA在DS组样本中表达上调,159种miRNA表达下调;还有38种和139种miRNA分别特异性表达于DS组和对照组样本中;21号染色体上5个miRNA,除miR-802

  10. Effect of Granulocyte Colony-Stimulating Factor-Combined Conditioning in Cord Blood Transplantation for Myelodysplastic Syndrome and Secondary Acute Myeloid Leukemia: A Retrospective Study in Japan.

    Science.gov (United States)

    Konuma, Takaaki; Takahashi, Satoshi; Uchida, Naoyuki; Kuwatsuka, Yachiyo; Yamasaki, Satoshi; Aoki, Jun; Onishi, Yasushi; Aotsuka, Nobuyuki; Ohashi, Kazuteru; Mori, Takehiko; Masuko, Masayoshi; Nakamae, Hirohisa; Miyamura, Kouichi; Kato, Koji; Atsuta, Yoshiko; Kato, Seiko; Asano, Shigetaka; Takami, Akiyoshi; Miyazaki, Yasushi

    2015-09-01

    Granulocyte colony-stimulating factor (G-CSF) increases the susceptibility of dormant malignant or nonmalignant hematopoietic cells to cytarabine arabinoside (Ara-C) through the induction of cell cycle entry. Therefore, G-CSF-combined conditioning before allogeneic stem cell transplantation might positively contribute to decreased incidences of relapse and graft failure without having to increase the dose of cytotoxic drugs. We conducted a retrospective nationwide study of 336 adult patients with myelodysplastic syndrome (MDS) and secondary acute myeloid leukemia (sAML) after single-unit cord blood transplantation (CBT) who underwent 4 different kinds of conditioning regimens: total body irradiation (TBI) ≥ 8 Gy + Ara-C/G-CSF + cyclophosphamide (CY) (n = 65), TBI ≥ 8 Gy + Ara-C + CY (n = 119), TBI ≥ 8 Gy + other (n = 104), or TBI < 8 Gy or non-TBI (n = 48). The TBI ≥ 8 Gy + Ara-C/G-CSF + CY regimen showed significantly higher incidence of neutrophil engraftment (hazard ratio, 1.52; 95% confidence interval [CI], 1.10 to 2.08; P = .009) and lower overall mortality (hazard ratio, .46; 95% CI, .26 to .82; P = .008) rates compared with those without a G-CSF regimen. This retrospective study shows that the G-CSF-combined conditioning regimen provides better engraftment and survival results in CBT for adults with MDS and sAML.

  11. Impact of Initial Central Venous Pressure on Outcomes of Conservative versus Liberal Fluid Management in Acute Respiratory Distress Syndrome

    Science.gov (United States)

    Semler, Matthew W.; Wheeler, Arthur P.; Thompson, B. Taylor; Bernard, Gordon R.; Wiedemann, Herbert P.; Rice, Todd W.

    2016-01-01

    Objective In acute respiratory distress syndrome (ARDS), conservative fluid management increases ventilator-free days without affecting mortality. Response to fluid management may differ based on patients’ initial central venous pressure (CVP). We hypothesized initial CVP would modify the effect of fluid management on outcomes. Design Retrospective analysis of the Fluid and Catheter Treatment Trial, a multicenter randomized trial comparing conservative to liberal fluid management in ARDS. We examined the relationship between initial CVP, fluid strategy, and 60-day mortality in univariate and multivariable analysis. Setting Twenty acute care hospitals. Patients Nine hundred and thirty-four ventilated ARDS patients with a CVP available at enrollment, 609 without baseline shock (for whom fluid balance was managed by study protocol). Interventions None. Measurements and Main Results Among patients without baseline shock, those with initial CVP > 8 mmHg experienced similar mortality with conservative and liberal fluid management (18% versus 18%, p=0.928), whereas those with CVP ≤8 mmHg experienced lower mortality with a conservative strategy (17% versus 36%, p=0.005). Multivariable analysis demonstrated an interaction between initial CVP and the effect of fluid strategy on mortality (p=0.031). At higher initial CVPs, the difference in treatment between arms was predominantly furosemide administration, which was not associated with mortality (p=0.122). At lower initial CVPs, the difference between arms was predominantly fluid administration, with additional fluid associated with increased mortality (p=0.013). Conclusions Conservative fluid management decreases mortality for ARDS patients with a low initial central venous pressure. In this population, the administration of intravenous fluids appears to increase mortality. PMID:26741580

  12. Cerebral salt wasting syndrome following atlantoaxial fracture dislocation in Down syndrome.

    Science.gov (United States)

    Abdel-Latif, Mohamed El-Amin; Chan, Patrick W-K; Goh, Adrian Yu-Teik; Lum, Lucy Chai-See

    2009-01-01

    We describe cerebral salt wasting syndrome (CSWS) in a 5-year-old female child with Down syndrome who had acute myelopathy secondary to chronic atlantoaxial subluxation and fracture dislocation of the odontoid process. The patient developed hyponatraemia associated with excessive urine output and elevated urine sodium concentration following her injury. An administered volume-for-volume replacement of urine loss with 0.9% sodium chloride resulted in an excellent outcome. This patient illustrates the importance of ascertaining CSWS in children with spinal cord disorders, in addition to the syndrome of inappropriate antidiuretic hormone (SIADH) secretion and diabetes insipidus (DI) commonly encountered following a central nervous system (CNS) injury, as the specific treatment approaches is clearly associated with an excellent outcome.

  13. A preliminary report on the use of laser-Doppler flowmetry during tethered spinal cord release.

    Science.gov (United States)

    Schneider, S J; Rosenthal, A D; Greenberg, B M; Danto, J

    1993-02-01

    Neurological deterioration in the tethered cord syndrome has been postulated to result from a compromise of blood flow in the distal spinal cord. In order to evaluate vascular perfusion in human subjects, a new technique of laser-Doppler flowmetry was used to monitor continuously the microcirculation of the distal spinal cord during surgery for tethered cord release in 10 children. For further comparison, five children undergoing selective dorsal rhizotomy were also monitored. In the tethered cord syndrome group, spinal cord blood flow before untethering was a mean of 12.6 ml/min per 100 g of tissue and increased in all cases after release to a mean of 29.4 ml/min per 100 g of tissue. All patients improved neurologically. The selective dorsal rhizotomy group had a preoperative mean spinal cord blood flow of 30.8 ml/min per 100 g of tissue, which was not altered by the operative procedure. Significant improvement occurs in distal spinal cord blood flow after tethered cord release, which may be representative of an important mechanism in the pathophysiology of the tethered cord syndrome.

  14. Central obesity and atherogenic dyslipidemia in metabolic syndrome are associated with increased risk for colorectal adenoma in a Chinese population

    Directory of Open Access Journals (Sweden)

    Lin Tsann

    2010-05-01

    Full Text Available Abstract Background Metabolic syndrome (MetS is composed of cardiovascular risk factors including insulin resistance, obesity, dyslipidemia, and hypertension. Most of the components of MetS have been linked to the development of neoplasm. The purpose of this study was to evaluate the relationship between individual components of MetS and colorectal adenoma. Methods The study subjects were recruited from a pool of 4872 individuals who underwent a health check-up examination during the period January 2006 to May 2008. Each participant fulfilled a structured questionnaire. MetS was defined based on the America Heart Association and National Heart Lung Blood Institute criteria. Subjects with history of colon cancer, colon polyps, colitis, or prior colonic surgery were excluded. Results A total of 4122 subjects were included for final analysis (2367 men and 1755 women; mean age, 49.6 ± 11.7 years. Of them, MetS was diagnosed in 708 men (29.9% and in 367 women (20.9%. Among the patients with MetS, 34.6% had adenoma, 31.7% had hyperplastic polyps and 23.3% were polyp-free (p Conclusions Of the components of MetS analyzed in this study, central obesity and dyslipidemia are independent risk factors for colorectal adenoma. With regard to the prevention of colorectal neoplasm, life-style modification such as weight reduction is worthwhile.

  15. Common genetic variants near the Brittle Cornea Syndrome locus ZNF469 influence the blinding disease risk factor central corneal thickness.

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    Yi Lu

    2010-05-01

    Full Text Available Central corneal thickness (CCT, one of the most highly heritable human traits (h(2 typically>0.9, is important for the diagnosis of glaucoma and a potential risk factor for glaucoma susceptibility. We conducted genome-wide association studies in five cohorts from Australia and the United Kingdom (total N = 5058. Three cohorts were based on individually genotyped twin collections, with the remaining two cohorts genotyped on pooled samples from singletons with extreme trait values. The pooled sample findings were validated by individual genotyping the pooled samples together with additional samples also within extreme quantiles. We describe methods for efficient combined analysis of the results from these different study designs. We have identified and replicated quantitative trait loci on chromosomes 13 and 16 for association with CCT. The locus on chromosome 13 (nearest gene FOXO1 had an overall meta-analysis p-value for all the individually genotyped samples of 4.6x10(-10. The locus on chromosome 16 was associated with CCT with p = 8.95x10(-11. The nearest gene to the associated chromosome 16 SNPs was ZNF469, a locus recently implicated in Brittle Cornea Syndrome (BCS, a very rare disorder characterized by abnormal thin corneas. Our findings suggest that in addition to rare variants in ZNF469 underlying CCT variation in BCS patients, more common variants near this gene may contribute to CCT variation in the general population.

  16. 原发干燥综合征中枢神经系统损害的MRI表现%MRI Features of Central Nervous System Complication in Primary Sjogren Syndrome

    Institute of Scientific and Technical Information of China (English)

    谢芳芳; 王小宜; 左晓霞; 邢妩

    2011-01-01

    目的 探讨原发干燥综合征中枢神经系统损害(CNS-PSS)的MRI表现,提高对CNS-PSS的MRI表现的认识.资料与方法 搜集经临床证实的14例CNS-PSS患者的临床和影像学资料进行回顾分析.结果 14例患者中,5例脑内病变,4例脊髓病变,3例脑内合并脊髓病变,1例脑膜炎,1例脑积水.脑内病变多发生于白质,较少累及皮质,仅1例小脑病灶累及灰质,但病灶主体仍位于白质.脑内7例为多发病灶,1例为单发病灶.脊髓6例为单发病灶,1例为多发病灶.脑内及脊髓内病灶T1W1呈稍低信号,T2WI呈较高信号.除1例脑膜炎患者外有6例患者行增强检查,仅1例脊髓内病变边缘可见强化.结论 CNS-PSS可累及脑及脊髓,脑内病变主要累及白质,MRI表现多为稍长T1、较长T2信号,增强扫描大多无明显强化;CNS-PSS的MRI表现缺乏特异性,需结合临床及实验室检查进行诊断.%Objective To evaluate the MRI features of the central nervous system complication in primary Sjogren syndrome(CNS-PSS). Materials and Methods MRI images of 14 patients with primary Sjogren syndrome associated with central nervous system complication were analyzed retrospectively. Results In 14 cases,5 cases were brain lesions,4 cases were spinal cord lesions,3 cases were cerebral and spinal lesions,complicated with meningitis in i patient, hydrocephalus in 1 patient. The lesions mostly located in white matter,less involved gray matter,and only one lesion involved the gray matter. The lesions showed high signal intensity on T2 WI and low signal intensity on T1 WI. Six patients performed enhanced MRI examination with Gd DTPA injected, and only one lesion showed enhancement on the edge. Conclusion CNS-PSS can affects brain and spinal cord,mainly involving white matter. The MRI features include high signal intensity on T2WI and low signal intensity on T1 WI. The lesions wont show obvious enhancement after injected Gd-DTPA. CNSPSS lack of specificity MRI features

  17. Early-onset multisystem degeneration with central motor, autonomic and optic nerve disturbances: unusual Riley-Day syndrome or new clinical entity?

    Science.gov (United States)

    Schnitzler, A; Witte, O W; Kunesch, E; Freund, H J; Benecke, R

    1998-02-05

    We report a 21-year-old woman presenting with a slowly progressive tetraparesis, optic nerve atrophy on both sides, and autonomic disturbances since early childhood. The patient has been carefully followed up for 5 years with clinical and ancillary investigations. The results and the time course strongly suggest an underlying degenerative syndrome affecting parts of three major systems: autonomic, motor and visual. Some symptoms resemble familial dysautonomia (FD, Riley-Day syndrome), however, hallmarks of FD, such as absence of fungiform papillae of the tongue, abnormal reaction on intradermal histamine injection, absent tendon reflexes, are missing, and central motor disturbances have not been described in FD. We consider this syndrome a slowly progressive multisystemic degeneration with two unusual hitherto unreported features: the combination of affected systems (autonomic and motor systems, optic nerves), and the early onset.

  18. Serum thyroid-stimulating hormone and anti-thyroglobulin antibody are independently associated with lesions in spinal cord in central nervous system demyelinating diseases.

    Directory of Open Access Journals (Sweden)

    Youming Long

    Full Text Available Transverse myelitis (TM is associated with neuromyelitis optica (NMO and multiple sclerosis (MS. Early recognition of useful parameters may be helpful to distinguish their difference. This retrospective study analyzed thyroid parameters from 243 serum samples (relapse = 128; remission = 115 of 178 patients with demyelinating diseases (NMO, n = 25; TM, n = 48; MS, n = 105. The relationship between thyroid and clinical parameters was analyzed. Patients with NMO and TM had a higher frequency of abnormal thyroid-stimulating hormone (TSH, anti-thyroglobulin antibodies (TG-Ab, and antithyroid peroxidase antibody (TPO-Ab than MS patients (p<0.05. The level of TSH and TG-Ab returned to normal levels after administration of high-dose intravenous methylprednisolone (p<0.05. In 96 patients (NMO, n = 19; TM, n = 25; MS, n = 52 without treatment, serum levels of TSH, TG-Ab and TPO-Ab were significantly different between patients with and without myelitis (p<0.01. Patients positive for aquaporin-4 (AQP4 antibodies showed higher abnormalities of TSH (p = 0.001, TG-Ab (p = 0.004 and TPO-Ab (p<0.0001 levels than AQP4 antibodies negative patients. Logistic regression analyses revealed independent relationships between TSH (odds ratio [OR]  = 33.994; p<0.0001, TG-Ab (OR = 7.703; p = 0.017 and myelitis occurrence in 96 patients at the active stage. In 52 MS patients experiencing their first attack, MS patients with myelitis were associated with TSH abnormalities (OR = 42.778; p<0.0001. This study showed increased abnormalities of thyroid parameters in patients with NMO and TM than in MS patients. MS patients with myelitis also had greater TSH abnormality than in MS patients without myelitis. Abnormal TSH and TG-Ab were independently associated with myelitis occurrence in central nervous system demyelinating disorders.

  19. The role of respiratory failure caused by congenital central nervous system abnormalities and the effect of β-casomorphins in sudden infant death syndrome pathogenesis

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    Barbara Sumińska-Ziemann

    2015-08-01

    Full Text Available The aim of the paper is to discuss the role of respiratory failure caused by endogenous (both structural and functional abnormalities in the central nervous system and exogenous food-derived opioid-like peptides in the pathogenesis of sudden infant death syndrome (SIDS. By stimulating μ-opioid receptors, opioid-like peptides may suppress the tonic activity of the respiratory centre in the brain stem.

  20. Double-level Incomplete Spinal Cord Injuries: A case report

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    Saeed Bin Ayaz

    2014-04-01

    Full Text Available Brown-Séquard Syndrome is a type of Incomplete Spinal Cord Injury characterized by a relatively greater ipsilateral loss of proprioception and motor function, with contralateral loss of pain and temperature sensations. The residual deficits in balance produced by such injury may render a person liable to fall that may result in vertebral fracture and another injury to the spinal cord. We present here a case who initially had Brown-Séquard Syndrome due to penetrating knife injury to the neck and later on developed Cauda Equina Syndrome (another Incomplete Spinal Cord Injury due to fractured LV1 following a fall. The fracture was fixed through Pedicle Screws and the patient underwent effective rehabilitation to gain maximum achievable independence in functional activities. [Cukurova Med J 2014; 39(2.000: 392-398

  1. Spinal Cord Diseases

    Science.gov (United States)

    ... damages the vertebrae or other parts of the spine, this can also injure the spinal cord. Other spinal cord problems include Tumors Infections such as meningitis and polio Inflammatory diseases Autoimmune diseases Degenerative diseases such as amyotrophic lateral sclerosis and spinal ...

  2. Split Cord Malformations

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    Yurdal Gezercan

    2015-06-01

    Full Text Available Split cord malformations are rare form of occult spinal dysraphism in children. Split cord malformations are characterized by septum that cleaves the spinal canal in sagittal plane within the single or duplicated thecal sac. Although their precise incidence is unknown, split cord malformations are exceedingly rare and represent %3.8-5 of all congenital spinal anomalies. Characteristic neurological, urological, orthopedic clinical manifestations are variable and asymptomatic course is possible. Earlier diagnosis and surgical intervention for split cord malformations is associated with better long-term fuctional outcome. For this reason, diagnostic imaging is indicated for children with associated cutaneous and orthopedic signs. Additional congenital anomalies usually to accompany the split cord malformations. Earlier diagnosis, meticuolus surgical therapy and interdisciplinary careful evaluation and follow-up should be made for good prognosis. [Cukurova Med J 2015; 40(2.000: 199-207

  3. Primary multifocal gliosarcoma of the spinal cord

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    Ramesh M. Kumar

    2016-03-01

    Full Text Available Gliosarcoma (GS is a rare and exceedingly malignant neoplasm of the central nervous system. It displays clinical features similar to glioblastoma, yet is histologically unique as it harbors both gliomatous and sarcomatous cellular components. Involvement of the neuroaxis is predominantly limited to the cerebral parenchyma and meninges. Primary GS of the spinal cord is rarely encountered. We report a case of a 54 year old male who presented with 2 months of progressive, bilateral lower extremity sensory deficits. Magnetic resonance imaging of the neuro-axis revealed multiple intradural lesions involving the cervical and thoracic spinal cord without evidence of intracranial involvement. Surgical resection of a dural based, extramedullary cervical lesion and two exophytic, intramedullary thoracic lesions revealed gliosarcoma, WHO grade IV. The patient died approximately 11 months after presentation. This report confirms that GS is not limited to supratentorial involvement and can primarily affect the spinal cord.

  4. Modeling spinal cord biomechanics

    Science.gov (United States)

    Luna, Carlos; Shah, Sameer; Cohen, Avis; Aranda-Espinoza, Helim

    2012-02-01

    Regeneration after spinal cord injury is a serious health issue and there is no treatment for ailing patients. To understand regeneration of the spinal cord we used a system where regeneration occurs naturally, such as the lamprey. In this work, we analyzed the stress response of the spinal cord to tensile loading and obtained the mechanical properties of the cord both in vitro and in vivo. Physiological measurements showed that the spinal cord is pre-stressed to a strain of 10%, and during sinusoidal swimming, there is a local strain of 5% concentrated evenly at the mid-body and caudal sections. We found that the mechanical properties are homogeneous along the body and independent of the meninges. The mechanical behavior of the spinal cord can be characterized by a non-linear viscoelastic model, described by a modulus of 20 KPa for strains up to 15% and a modulus of 0.5 MPa for strains above 15%, in agreement with experimental data. However, this model does not offer a full understanding of the behavior of the spinal cord fibers. Using polymer physics we developed a model that relates the stress response as a function of the number of fibers.

  5. Association of misoprostol, moebius syndrome and congenital central alveolar hypoventilation: case report Associação de misoprostol, síndrome de Moebius e hipoventilação central congênita: relato de caso

    Directory of Open Access Journals (Sweden)

    MAGDA LAHORGUE NUNES

    1999-03-01

    Full Text Available We report a case showing the association of Moebius syndrome, the use of misoprostol during pregnancy and the development of central congenital alveolar hypoventilation. Pathophysiological aspects of these three diseases are discussed and also the unfavorable prognosis of this association.Descrevemos o caso de um paciente com Síndrome de Moebius associada ao uso de misoprostol durante a gestação. A criança necessitou de suporte ventilatório desde o primeiro dia de vida e evoluiu com quadro de hipoventilação alveolar central congênita, persistindo dependente de ventilação mecânica. São discutidos aspectos fisiopatológicos que poderiam justificar a comorbidade destes três eventos, assim como o prognóstico reservado desta associação.

  6. Spinal Cord Injury

    Science.gov (United States)

    ... indicated by a total lack of sensory and motor function below the level of injury. People who survive a spinal cord injury will most likely have medical complications such as chronic pain and bladder and bowel ...

  7. Spinal cord abscess

    Science.gov (United States)

    ... drugs The infection often begins in the bone ( osteomyelitis ). The bone infection may cause an epidural abscess ... Boils Cerebral spinal fluid (CSF) collection Epidural abscess Osteomyelitis Pulmonary tuberculosis Sepsis Spinal cord trauma Swelling Review ...

  8. Vocal cord palsy: An uncommon presenting feature of myasthenia gravis

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    Sethi Prahlad

    2011-01-01

    Full Text Available Vocal cord palsy can have myriad causes. Unilateral vocal cord palsy is common and frequently asymptomatic. Trauma, head, neck and mediastinal tumors as well as cerebrovascular accidents have been implicated in causing unilateral vocal cord palsy. Viral neuronitis accounts for most idiopathic cases. Bilateral vocal cord palsy, on the other hand, is much less common and is a potentially life-threatening condition. Myasthenia gravis, an autoimmune disorder caused by antibodies targeting the post-synaptic acetylcholine receptor, has been infrequently implicated in its causation. We report here a case of bilateral vocal cord palsy developing in a 68-year-old man with no prior history of myasthenia gravis 2 months after he was operated on for diverticulitis of the large intestine. Delay in considering the diagnosis led to endotracheal intubation and prolonged mechanical ventilation with attendant complications. Our case adds to the existing literature implicating myasthenia gravis as an infrequent cause of bilateral vocal cord palsy. Our case is unusual as, in our patient, acute-onset respiratory distress and stridor due to bilateral vocal cord palsy was the first manifestation of a myasthenic syndrome.

  9. Flail arm-like syndrome associated with HIV-1 infection

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    Nalini A

    2009-01-01

    Full Text Available During the last 20 years at least 23 cases of motor neuron disease have been reported in HIV-1 seropositive patients. In this report we describe the clinical picture of a young man with HIV-1 clade C infection and flail arm-like syndrome, who we were able to follow-up for a long period. We investigated and prospectively monitored a 34-year-old man with features of flail arm syndrome, who developed the weakness and wasting 1 year after being diagnosed with HIV-1 infection after a routine blood test. He presented in 2003 with progressive, symmetrical wasting and weakness of the proximal muscles of the upper limb of 2 years′ duration. He had severe wasting and weakness of the shoulder and arm muscles. There were no pyramidal signs. He has been on HAART for the last 4 years and the weakness or wasting has not worsened. At the last follow-up in July 2007, the patient had the same neurological deficit and no other symptoms or signs of HIV-1 infection. MRI of the spinal cord in 2007 showed characteristic T2 hyperintense signals in the central part of the spinal cord, corresponding to the central gray matter. Thus, our patient had HIV-1 clade C infection associated with a ′flail arm-like syndrome.′ The causal relationship between HIV-1 infection and amyotrophic lateral sclerosis (ALS-like syndrome is still uncertain. The syndrome usually manifests as a lower motor neuron syndrome, as was seen in our young patient. It is known that treatment with antiretroviral therapy (ART stabilizes/improves the condition. In our patient the weakness and atrophy remained stable over a period of 3.5 years after commencing HAART regimen.

  10. Spinal cord compression in {beta}-thalassemia: follow-up after radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Fonseca, Silvana Fahel da; Figueiredo, Maria Stella; Cancado, Rodolfo Delfini; Nakadakare, Fernando; Segreto, Roberto; Kerbauy, Jose [Universidade Federal de Sao Paulo (UNIFESP), SP (Brazil). Escola Paulista de Medicina

    1998-12-01

    Spinal cord compression due to extramedullary hematopoiesis is a well-described bu rare syndrome encountered in several hematologic disorders, including {beta}-thalassemia. We report a case of a patient with intermediate {beta}-thalassemia and crural paraparesis due to spinal cord compression by a paravertebral extramedullary mass. She was successfully treated with low-dose radiotherapy and transfusions. After splenectomy, she was regularly followed up for over four years without transfusion or recurrence of spinal cord compression. Extramedullary hematopoiesis should be investigated in patients with hematologic disorders and spinal cord symptoms. The rapid recognition and treatment with radiotherapy can dramatically alleviate symptoms. (author)

  11. Spinal cord compression in b-thalassemia: follow-up after radiotherapy

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    Silvana Fahel da Fonseca

    Full Text Available CONTEXT: Spinal cord compression due to extramedullary hematopoiesis is a well-described but rare syndrome encountered in several clinical hematologic disorders, including b-thalassemia. CASE REPORT: We report the case of a patient with intermediate b-thalassemia and crural paraparesis due to spinal cord compression by a paravertebral extramedullary mass. She was successfully treated with low-dose radiotherapy and transfusions. After splenectomy, she was regularly followed up for over four years without transfusion or recurrence of spinal cord compression. DISCUSSION: Extramedullary hematopoiesis should be investigated in patients with hematologic disorders and spinal cord symptoms. The rapid recognition and treatment with radiotherapy can dramatically alleviate symptoms.

  12. Transcutaneous electrical spinal-cord stimulation in humans

    OpenAIRE

    Gerasimenko, Yury; Gorodnichev, Ruslan; Moshonkina, Tatiana; Sayenko, Dimitry; Gad, Parag; Edgerton, V. Reggie

    2015-01-01

    Locomotor behavior is controlled by specific neural circuits called central pattern generators primarily located at the lumbosacral spinal cord. These locomotor-related neuronal circuits have a high level of automaticity; that is, they can produce a “stepping” movement pattern also seen on electromyography (EMG) in the absence of supraspinal and/or peripheral afferent inputs. These circuits can be modulated by epidural spinal-cord stimulation and/or pharmacological intervention. Such interven...

  13. Imbalance between thyroid hormones and the dopaminergic system might be central to the pathophysiology of restless legs syndrome: a hypothesis

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    Jose Carlos Pereira Jr.

    2010-01-01

    Full Text Available Data collected from medical literature indicate that dopaminergic agonists alleviate Restless Legs Syndrome symptoms while dopaminergic agonists antagonists aggravate them. Dopaminergic agonists is a physiological regulator of thyroid-stimulating hormone. Dopaminergic agonists infusion diminishes the levels of thyroid hormones, which have the ability to provoke restlessness, hyperkinetic states, tremors, and insomnia. Conditions associated with higher levels of thyroid hormones, such as pregnancy or hyperthyroidism, have a higher prevalence of Restless Legs Syndrome symptoms. Low iron levels can cause secondary Restless Legs Syndrome or aggravate symptoms of primary disease as well as diminish enzymatic activities that are involved in dopaminergic agonists production and the degradation of thyroid hormones. Moreover, as a result of low iron levels, dopaminergic agonists diminishes and thyroid hormones increase. Iron therapy improves Restless Legs Syndrome symptoms in iron deprived patients. Medical hypothesis. To discuss the theory that thyroid hormones, when not counterbalanced by dopaminergic agonists, may precipitate the signs and symptoms underpinning Restless Legs Syndrome. The main cause of Restless Legs Syndrome might be an imbalance between the dopaminergic agonists system and thyroid hormones.

  14. Segmental hypersensitivity and spinothalamic function in spinal cord injury pain

    DEFF Research Database (Denmark)

    Finnerup, Nanna Brix; Sørensen, Leif Hougaard; Biering-Sørensen, Fin;

    2007-01-01

    The mechanisms underlying central pain following spinal cord injury (SCI) are unsettled. The purpose of the present study was to examine differences in spinothalamic tract function below injury level and evoked pain in incomplete SCI patients with neuropathic pain below injury level (central pain...

  15. Adult spinal cord ependymal layer: a promising pool of quiescent stem cells to treat spinal cord injury

    OpenAIRE

    Panayiotou, Elena; Malas, Stavros

    2013-01-01

    Spinal cord injury (SCI) is a major health burden and currently there is no effective medical intervention. Research performed over the last decade revealed that cells surrounding the central canal of the adult spinal cord and forming the ependymal layer acquire stem cell properties either in vitro or in response to injury. Following SCI activated ependymal cells generate progeny cells which migrate to the injury site but fail to produce the appropriate type of cells in sufficient number to l...

  16. Gardner Syndrome

    Science.gov (United States)

    ... or central nervous system tumor less than 1% Stomach cancer 0.5% Bile duct cancer small, but increased Adrenal gland cancer small, but increased What are the screening options for Gardner syndrome? The screening options for ...

  17. Evidence of involvement of central neural mechanisms in generating fibromyalgia pain.

    Science.gov (United States)

    Staud, Roland

    2002-08-01

    Fibromyalgia syndrome (FMS) is characterized by widespread pain, fatigue, sleep abnormalities, and distress. Because FMS lacks consistent evidence of tissue abnormalities, recent investigations have focused on central nervous system mechanisms of pain. Abnormal temporal summation of second pain (wind-up) and central sensitization have been described recently in patients with FMS. Wind-up and central sensitization, which rely on central pain mechanisms, occur after prolonged C-nociceptor input and depend on activation of nociceptor-specific neurons and wide dynamic range neurons in the dorsal horn of the spinal cord. Other abnormal central pain mechanisms recently detected in patients with FMS include diffuse noxious inhibitory controls. These pain inhibitory mechanisms rely on spinal cord and supraspinal systems involving pain facilitatory and pain inhibitory pathways. Brain-imaging techniques that can detect neuronal activation after nociceptive stimuli have provided additional evidence for abnormal central pain mechanisms in FMS. Brain images have corroborated the augmented reported pain experience of patients with fibromyalgia during experimental pain stimuli. In addition, thalamic activity, which contributes significantly to pain processing, was decreased in fibromyalgia. However, central pain mechanisms of fibromyalgia may not depend exclusively on neuronal activation. Neuroglial activation has been found to play an important role in the induction and maintenance of chronic pain. These findings may have important implications for future research and the treatment of fibromyalgia pain.

  18. Spinal cord injury as a trigger to develop periodic leg movements during sleep: an evolutionary perspective

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    Susana Cristina Lerosa Telles

    2012-11-01

    Full Text Available The primary trigger to periodic limb movement (PLM during sleep is still unknown. Its association with the restless legs syndrome (RLS is established in humans and was reported in spinal cord injury (SCI patients classified by the American Spinal Injury Association (ASIA as A. Its pathogenesis has not been completely unraveled, though recent advances might enhance our knowledge about those malfunctions. PLM association with central pattern generator (CPG is one of the possible pathologic mechanisms involved. This article reviewed the advances in PLM and RLS genetics, the evolution of CPG functioning, and the neurotransmitters involved in CPG, PLM and RLS. We have proposed that SCI might be a trigger to develop PLM.

  19. Phrenic nerve afferents elicited cord dorsum potential in the cat cervical spinal cord

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    Davenport Paul W

    2005-05-01

    Full Text Available Abstract Background The diaphragm has sensory innervation from mechanoreceptors with myelinated axons entering the spinal cord via the phrenic nerve that project to the thalamus and somatosensory cortex. It was hypothesized that phrenic nerve afferent (PnA projection to the central nervous system is via the spinal dorsal column pathway. Results A single N1 peak of the CDP was found in the C4 and C7 spinal segments. Three peaks (N1, N2, and N3 were found in the C5 and C6 segments. No CDP was recorded at C8 dorsal spinal cord surface in cats. Conclusion These results demonstrate PnA activation of neurons in the cervical spinal cord. Three populations of myelinated PnA (Group I, Group II, and Group III enter the cat's cervical spinal segments that supply the phrenic nerve

  20. A Systematic Review of Peripheral and Central Nervous System Involvement of Rheumatoid Arthritis, Systemic Lupus Erythematosus, Primary Sjögren’s Syndrome, and Associated Immunological Profiles

    Directory of Open Access Journals (Sweden)

    Anastasia Bougea

    2015-01-01

    Full Text Available Both central (CNS and peripheral nervous system (PNS complications are frequent and varied in connective tissue diseases. A systematic review was conducted between 1989 and 2014 in the databases Medline, Scopus, and Cochrane Library using the search terms, peripheral and central nervous complications and immunological profiles, to identify studies in specific connective tissue disorders such as rheumatoid arthritis, systemic lupus erythematosus, and primary Sjögren’s syndrome. A total of 675 references were identified, of which 118 were selected for detailed analysis and 22 were included in the final review with a total of 2338 participants. Our search focused only on studies upon connective tissue disorders such as rheumatoid arthritis, systemic lupus erythematosus, and primary Sjögren’s syndrome associated with seroimmunological data. The reported prevalence of CNS involvement ranges from 9 to 92% across the reported studies. However, the association between CNS and PNS manifestations and seroimmunological profiles remains controversial. Τo date, no laboratory test has been shown as pathognomonic neither for CNS nor for PNS involvement.

  1. A 15-year-old boy with central nervous system vasculopathy presenting with dysarthria-clumsy hand syndrome.

    Science.gov (United States)

    Golomb, Meredith R; Weiss, Shelly K; Ibrahim, Shahnaz H; deVeber, Gabrielle A

    2002-03-01

    Dysarthria-clumsy hand stroke syndrome has been described frequently in adults but not in children. We report a 15-year-old right-handed boy with sudden onset of dysarthria, dysphagia, right facial weakness, and mild right-hand clumsiness. Computed tomographic scan and magnetic resonance imaging demonstrated infarction in the genu and posterior limb of the left internal capsule. Magnetic resonance angiography and conventional angiography demonstrated stenosis of the supraclinoid portion of the left internal carotid artery and the origin of the left ophthalmic artery. Lacunar infarction in an older adult is not the only mechanism leading to dysarthria-clumsy hand syndrome.

  2. Russell–Silver syndrome associated with low conus medullaris

    Science.gov (United States)

    Gabor, Larisa; Canaz, Huseyin; Canaz, Gokhan; Kara, Nursu; Alatas, Ibrahim; Bozkus, Hakan

    2016-01-01

    Russell–Silver syndrome is a rare heterogeneous disorder mainly characterized by intrauterine and postnatal growth retardation, craniofacial disproportion, clinodactyly, variation in urogenital development, and skeletal asymmetry. It is rare to come across tethered cord-associated Russell–Silver syndrome. We report a rare case of Russell–Silver syndrome associated with low conus medullaris in a 2-year-old patient with demonstrative phenotype. Magnetic resonance imaging indicated a low conus medullaris at the inferior border of the L3 vertebral body. Urodynamic study revealed detrusor-sphincter dyssynergia and detrusor overactivity. A decision to follow-up the patient was made because of the suspicion of tethered cord syndrome. Even though tethered cord syndrome is not a common finding in Russell–Silver syndrome, it is important to consider tethered cord syndrome to avoid scoliosis and other long-term complications. PMID:28217167

  3. Radiation nephritis causing nephrotic syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Jennette, J.C.; Ordonez, N.G.

    1983-12-01

    Clinical symptoms of acute radiation nephritis with nephrotic syndrome developed in a fifty-six-year-old woman after abdominal radiation therapy for an astrocytoma of the spinal cord. The diagnosis of radiation nephritis was confirmed by renal biopsy. To our knowledge, this is the first documented case of radiation nephritis associated with nephrotic syndrome.

  4. Serotonergic modulation of post-synaptic inhibition and locomotor alternating pattern in the spinal cord

    OpenAIRE

    Laurent eVinay; Florian eGackière

    2014-01-01

    The central pattern generators (CPGs) for locomotion, located in the lumbar spinal cord, are functional at birth in the rat. Their maturation occurs during the last few days preceding birth, a period during which the first projections from the brainstem start to reach the lumbar enlargement of the spinal cord. Locomotor burst activity in the mature intact spinal cord alternates between flexor and extensor motoneurons through reciprocal inhibition and between left and right sides through commi...

  5. Effect of Microgene pSVPoMcat Implantation to Modify Genetically Schwann Cells on Central Conduction Function after Spinal Cord Injury%微基因修饰雪旺氏细胞移植对脊髓损伤中枢传导功能的作用

    Institute of Scientific and Technical Information of China (English)

    陈礼刚; 高立达; 周虎田; 曾凡俊; 毛伯镛; 李讯; 卢敏

    2001-01-01

    Objective To approach the effect of microgene pSVPoMcat to modify genetically Schwann cells (SC) on central conduction functionafter spinal cord injury (SCI) . Method Experimental animals were divided into three groups: the group of microgene pSVPoMcat implanted to genetically modify SC (group A), SC implanted group (group B), and the control group (group C). The cortical somatasensory evoked potentials (CESP) and combined behavioral score (CBS) were continuous monitored from operation second week to twelfth week Result The result showed that the peak latency and peak amplitudes of group A, B have a recovery tendency and it was constant with CBS. Conclusion We concluded that the microgene pSVPoMcat to modify genetically Sc may play a promotion role in recovery of central conduction function after SCI.

  6. Loss-of-function mutations in IGSF1 cause an X-linked syndrome of central hypothyroidism and testicular enlargement

    NARCIS (Netherlands)

    Sun, Y.; Bak, B.; Schoenmakers, N.; Trotsenburg, A.S. van; Oostdijk, W.; Voshol, P.; Cambridge, E.; White, J.K.; Tissier, P. le; Gharavy, S.N.; Martinez-Barbera, J.P.; Stokvis-Brantsma, W.H.; Vulsma, T.; Kempers, M.J.; Persani, L.; Campi, I.; Bonomi, M.; Beck-Peccoz, P.; Zhu, H.; Davis, T.M.; Hokken-Koelega, A.C.; Del Blanco, D.G.; Rangasami, J.J.; Ruivenkamp, C.A.; Laros, J.F.; Kriek, M.; Kant, S.G.; Bosch, C.A.; Biermasz, N.R.; Appelman-Dijkstra, N.M.; Corssmit, E.P.; Hovens, G.C.; Pereira, A.M.; Dunnen, J.T. den; Wade, M.G.; Breuning, M.H.; Hennekam, R.C.; Chatterjee, K.; Dattani, M.T.; Wit, J.M.; Bernard, D.J.

    2012-01-01

    Congenital central hypothyroidism occurs either in isolation or in conjunction with other pituitary hormone deficits. Using exome and candidate gene sequencing, we identified 8 distinct mutations and 2 deletions in IGSF1 in males from 11 unrelated families with central hypothyroidism, testicular enl

  7. External fixator to correct stiff foot deformity in tethered cord syndrome%外固定器治疗脊髓栓系综合征僵硬性足部畸形

    Institute of Scientific and Technical Information of China (English)

    胡新永; 陈建文; 王义生

    2012-01-01

    [Objective] To study the clinical outcomes of stiff foot deformity corrected by external fixator in the patients with tethered cord syndrome, and to explore the new method of surgical correction and functional reconstruction of spinal neuro-genic foot deformity. [Methods] From October 1988 to June 2006, 61 cases (97 feet) with stiff foot deformity were treated with operation. Accoding to the characteristics of every foot deformity, unique external fixator was disigned. Among these patients , foot deformity in the type of upper motor neurons injury were mainly treated with functional selective tibial neurotomy and extra - articular arthrodesis of the subtalar joint or subtalar fusion and external fixator was installed. Foot deformity in the type of lower motor neurons injury were mainly treated with soft tissue release, transformation of tendon and osteotomy and installed external fixator was installed. [ Results ] Eighty - six feet in 54 patients were followed up for at least 3 years and were generally analyzed. Thirty - nine feet were talipes equinovarus, 21 feet were talipes equinus, 16 feet were talipes equinovalgus, 6 feet were talipes of paralysis achillis tendon, 4 feet were talipes cavus. Nineteen feet were in the type of upper motor neurons injury, 67 feet were in the type of lower motor neurons injury. The fixation time was 8-12 weeks (mean, 11 weeks) . Clinical outcomes were evaluated according to Laaveg - Ponseti foot score. There were excellent results in 38 cosses, good in 29, fair in 13, and poor in 6. The rate of excellent and good results were 77.9%. Relapse cases after first operation were in 3 feet and the recurrence rate was 3. 5%. Pin track infection occurred in 18 feet, which were cured by removing secretion of inflammatory or replacing the location of pin. Plantar ulcer occurred in 3 feet , which were cured by debridement and avoiding weight bearing walking in short term. [ Conclusion] Using external fixator to correct the stiff foot deformity

  8. [Spinal cord infarction].

    Science.gov (United States)

    Naumann, N; Shariat, K; Ulmer, S; Stippich, C; Ahlhelm, F J

    2012-05-01

    Infarction of the spinal cord can cause a variety of symptoms and neurological deficits because of the complex vascular supply of the myelon. The most common leading symptom is distal paresis ranging from paraparesis to tetraplegia caused by arterial ischemia or infarction of the myelon. Venous infarction, however, cannot always be distinguished from arterial infarction based on the symptoms alone.Modern imaging techniques, such as computed tomography angiography (CTA) and magnetic resonance angiography (MRA) assist in preoperative planning of aortic operations to reliably identify not only the most important vascular structure supplying the spinal cord, the artery of Adamkiewicz, but also other pathologies such as tumors or infectious disorders. In contrast to CT, MRI can reliably depict infarction of the spinal cord.

  9. Localization of Brain Natriuretic Peptide Immunoreactivity in Rat Spinal Cord

    Directory of Open Access Journals (Sweden)

    Essam M Abdelalim

    2016-12-01

    Full Text Available Brain natriuretic peptide (BNP exerts its functions through natriuretic peptide receptors. Recently, BNP has been shown to be involved in a wide range of functions. Previous studies reported BNP expression in the sensory afferent fibers in the dorsal horn of the spinal cord. However, BNP expression and function in the neurons of the central nervous system are still controversial. Therefore, in this study, we investigated BNP expression in the rat spinal cord in detail using RT-PCR and immunohistochemistry. RT-PCR analysis showed that BNP mRNA was present in the spinal cord and DRG. BNP immunoreactivity was observed in different structures of the spinal cord, including the neuronal cell bodies and neuronal processes. BNP immunoreactivity was observed in the dorsal horn of the spinal cord and in the neurons of the intermediate column and ventral horn. Double-immunolabeling showed a high level of BNP expression in the afferent fibers (laminae I-II labeled with calcitonin gene-related peptide (CGRP, suggesting BNP involvement in sensory function. In addition, BNP was co-localized with CGRP and choline acetyltransferase in the motor neurons of the ventral horn. Together, these results indicate that BNP is expressed in sensory and motor systems of the spinal cord, suggesting its involvement in several biological actions on sensory and motor neurons via its binding to NPR-A and/or NPR-B in the DRG and spinal cord.

  10. Spinal cord swelling and candidiasis

    Energy Technology Data Exchange (ETDEWEB)

    Ho, K.; Gronseth, G.; Aldrich, M.; Williams, A.

    1982-11-01

    Fusiform swelling of the spinal cord was noted myelographically in a patient with Hodgkin's disease. Autopsy revealed that the swelling was caused by Candida infection of the spinal cord. It is suggested that fungal infection be included in the differential diagnosis of spinal cord swelling in the immunosuppressed cancer patient.

  11. Clinical diagnosis analysis in 21 cases of spinal cord disease

    Institute of Scientific and Technical Information of China (English)

    ZHANG Hong

    2000-01-01

    21 cases of spinal cord disease were clinically analyzed of which 14 cases were male. 7 female, aged from 30 to 69, weraged 50.9. This group contained 2 cases of consciusness dysfunction. 2l of sensational dysfunction. 19 of morion dysrunction, 11 of aotonomic nerve dysrunction, 2 of, sexual dysfunction. 2 of Brown-Sequrd syndrome. llhad been chrmcally sympromatic for more than two months. the other were of acute for sub acute onset. The segments of the diseases were found mainly at cervical and thoracic ones. to which more attention should be pazd clincally. The incidences of intramedullary lesions were a bit more than that of extramedullary ones. Of the 11 extramendullary cases, 8 arised srorn verteoral body (72.7%), of which 6 cases arisen form the intervertebral dies, or 75%. On which emphasis should be laid clinically Foci in brain and spinal cord were found synchronically in 5 cases (23.8%). They were Wernick cerebral disease, metastetic cerebral tumor, cerebral infarction, polioencephalomyelitis. So possibility of brain disease should also be considerod when diagnosing spinai cord disease, especially in stenosis of cervical canal. Oppressed spinal cord was accodiated with cerebellopontine angle tumor. Disease in brain was negiected because of concermng spinal cord disease and cerehellopontine angie giant meningiona was discovered 5 yeas laaer. There are several methods to diagnose spinal cord disease, including X-rays photography, CSF test, CT, and MRI, etc. X-rays photography should be used for involved vertebral body in lateral and P-A position at first when to suspect spinal cord disease. It should be avoided that neglecting X-rays photography and using CT or MRIfirst. It should be noted when taking segments examination by CT or MRI, thut the actual vertebral body is usually located 7'- 14 segments below the spinal cord involved. Otherwise, misdiagnosis would be resulted. 4 c ases of oppressive spinal cord disease of this group were treated with operating

  12. Spinal cord stimulation with hybrid lead relieves pain in low back and legs

    NARCIS (Netherlands)

    Vos, de Cecile C.; Dijkstra, Cindy; Lenders, Mathieu W.P.M.; Holsheimer, Jan; Levy, R.M.

    2012-01-01

    Objective: The failed back surgery syndrome (FBSS) is the most common chronic pain syndrome. Whereas it is relatively easy to achieve pain relief in the lower limbs of FBSS patients with spinal cord stimulation (SCS), it is difficult to manage low back pain with SCS. The performance of a paddle-shap

  13. Trombose de veia central da retina bilateral associada à síndrome de hiperviscosidade sanguínea: relato de caso Bilateral central retinal vein occlusion associated with blood hyperviscosity syndrome: case report

    Directory of Open Access Journals (Sweden)

    John Helal Jr

    2005-02-01

    Full Text Available Relato de caso de um paciente masculino de 16 anos de idade com queixa inicial de baixa da acuidade visual e que no exame oftalmológico foi encontrado edema de papila bilateral, que evoluiu para trombose da veia central da retina em ambos os olhos. Na investigação laboratorial, foi feito diagnóstico de um mieloma múltiplo tipo IgA que cursava com síndrome de hiperviscosidade sanguínea, o que explicava o quadro oftalmológico. Após tratamento específico, o paciente apresentou melhora tanto da acuidade visual quanto do aspecto fundoscópico. O achado de oclusão de veia central da retina bilateral pode levar ao diagnóstico de importantes doenças sistêmicas. os achados fundoscópicos podem servir de parâmetro na avaliação do tratamento.The authors report the case of a 16-year-old male patient who presented with blurred vision and bilateral optic disc edema, then developing bilateral central retinal vein occlusion. On laboratory work-up, he was found to have multiple myeloma IgA along with hyperviscosity syndrome, which led to the ophthalmological features. After proper treatment, the patient recovered visual acuity and normalized his eye fundus changes. Bilateral central retinal vein occlusion finding may yield the diagnosis of major systemic diseases. Fundoscopic features may serve as parameters on treatment evaluation.

  14. Idiopathic dural herniation of the thoracic spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Hausmann, O.N. [National Hospital for Neurology and Neurosurgery, London (United Kingdom). Lysholm Radiological Dept.; Moseley, I.F. [National Hospital for Neurology and Neurosurgery, London (United Kingdom). Lysholm Radiological Dept.

    1996-08-01

    Symptomatic anterior or anterolateral dural herniation of the spinal cord is rare, and not uncommonly misdiagnosed, both clinically and radiologically. We present four patients with a radiological diagnosis of herniation of the thoracic spinal cord, and review the current literature. All affected patients have been adults, typcially presenting with long-standing, unexplained sensory symptoms and eventually developing a Brown-Sequard syndrome, with or without motor changes. Herniation occurs in the upper or midthoracic region, between the T2 and T8 levels. (orig.)

  15. Metabolic Syndrome in Italian Obese Children and Adolescents: Stronger Association with Central Fat Depot than with Insulin Sensitivity and Birth Weight

    Directory of Open Access Journals (Sweden)

    Claudia Brufani

    2011-01-01

    Full Text Available Aim. To evaluate whether body fat distribution, birth weight, and family history for diabetes (FHD were associated with metabolic syndrome (MetS in children and adolescents. Methods. A total of 439 Italian obese children and adolescents (5–18 years were enrolled. Subjects were divided into 2 groups: prepubertal and pubertal. MetS was diagnosed according to the adapted National Cholesterol Education Program criteria. Birth weight percentile, central obesity index (measured by dual-energy X-ray absorptiometry, insulin sensitivity (ISI, and disposition index were evaluated. Multivariate logistic regression models were used to determine variables associated with MetS. Results. The prevalence of MetS was 17%, with higher percentage in adolescents than in children (21 versus 12%. In the overall population, central obesity index was a stronger predictor of MetS than insulin sensitivity and low birth weight. When the two groups were considered, central fat depot remained the strongest predictor of MetS, with ISI similarly influencing the probability of MetS in the two groups and birth weight being negatively associated to MetS only in pubertal individuals. Neither FHD nor degree of fatness was a significant predictor of MetS. Conclusion. Simple clinical parameters like increased abdominal adiposity and low birth weight could be useful tools to identify European obese adolescents at risk for metabolic complications.

  16. Stiff-person syndrome (SPS) and anti-GAD-related CNS degenerations: protean additions to the autoimmune central neuropathies.

    Science.gov (United States)

    Ali, Fatima; Rowley, Merrill; Jayakrishnan, Bindu; Teuber, Suzanne; Gershwin, M Eric; Mackay, Ian R

    2011-09-01

    Stiff Person Syndrome (SPS) is a rare autoimmune neurological disease attributable to autoantibodies to glutamic acid decarboxylase (anti-GAD) more usually associated with the islet beta cell destruction of autoimmune type 1 diabetes (T1D). SPS is characterized by interference in neurons with the synthesis/activity of the inhibitory neurotransmitter gamma amino butyric acid (GABA) resulting in the prototypic progressive spasmodic muscular rigidity of SPS, or diverse neurological syndromes, cerebellar ataxia, intractable epilepsy, myoclonus and several others. Remarkably, a single autoantibody, anti-GAD, can be common to widely different disease expressions, i.e. T1D and SPS. One explanation for these data is the differences in epitope engagement between the anti-GAD reactivity in SPS and T1D: in both diseases, anti-GAD antibody reactivity is predominantly to a conformational epitope region in the PLP- and C-terminal domains of the 65 kDa isoform but, additionally in SPS, there is reactivity to conformational epitope(s) on GAD67, and short linear epitopes in the C-terminal region and at the N-terminus of GAD65. Another explanation for disease expressions in SPS includes ready access of anti-GAD to antigen sites due to immune responsiveness within the CNS itself according to intrathecal anti-GAD-specific B cells and autoantibody. Closer study of the mysterious stiff-person syndrome should enhance the understanding of this disease itself, and autoimmunity in general.

  17. Adult spinal cord ependymal layer: A promising pool of quiescent stem cells to treat spinal cord injury

    Directory of Open Access Journals (Sweden)

    Stavros eMalas

    2013-11-01

    Full Text Available Spinal cord injury is a major health burden and currently there is no effective medical intervention. Research performed over the last decade revealed that cells surrounding the central canal of the adult spinal cord and forming the ependymal layer acquire stem cell properties either in vitro or in response to injury. Following spinal cord injury activated ependymal cells generate progeny cells which migrate to the injury site but fail to produce the appropriate type of cells in sufficient number to limit the damage, rendering this physiological response mainly ineffective. Research is now focusing on the manipulation of ependymal cells to produce cells of the oligodendrocyte lineage which are primarily lost in such a situation leading to secondary neuronal degeneration. Thus, there is a need for a more focused approach to understand the molecular properties of adult ependymal cells in greater detail and develop effective strategies for guiding their response during spinal cord injury.

  18. High-Sensitivity C-Reactive Protein is Related to Central Obesity and the Number of Metabolic Syndrome Components in Jamaican Young Adults

    Science.gov (United States)

    Bennett, Nadia R.; Ferguson, Trevor S.; Bennett, Franklyn I.; Tulloch-Reid, Marshall K.; Younger-Coleman, Novie O. M.; Jackson, Maria D.; Samms-Vaughan, Maureen E.; Wilks, Rainford J.

    2014-01-01

    associated with central obesity and the number of metabolic syndrome components. PMID:26664862

  19. High sensitivity C-reactive protein is related to central obesity and the number of metabolic syndrome components in Jamaican young adults

    Directory of Open Access Journals (Sweden)

    Nadia R Bennett

    2014-12-01

    Full Text Available BackgroundHigh-sensitivity C-reactive protein (hsCRP has been shown to predict cardiovascular disease (CVD endpoints and is associated with CVD risk factors and the metabolic syndrome. This study evaluated the association between hsCRP and CVD risk factors among Afro-Caribbean young adults in Jamaica. MethodsWe conducted a cross-sectional analysis of data from the Jamaica 1986 Birth Cohort Study. Data were collected between 2005 and 2007 when participants were 18-20 years old. All participants completed an interviewer administered questionnaire and had anthropometric and blood pressure (BP measurements performed. Fasting blood samples were collected for measurement of glucose, lipids and hsCRP. Logistic regression models were used to identify factors independently associated with high hsCRP.ResultsAnalyses included 342 men and 404 women with mean age 18.8 ± 0.6 years. Approximately 15% of the participants had high risk hsCRP (>3 mg/L, with a higher prevalence among women (20% vs. 9%; p<0.001. The prevalence of elevated hsCRP increased with BMI category, high waist circumference (WC, high triglycerides, low HDL, and lower parental education among women, but only for high WC and lower parental education among men. In logistic regression models controlling for sex and parental education, high WC was associated with significantly higher odds of high hsCRP (OR 7.8, 95%CI 4.8-12.9, p<0.001. In a similar model high hsCRP was also associated with the number of metabolic syndrome components. Compared to participants with no metabolic syndrome component, having one metabolic syndrome component was associated with a two-fold higher odds of high hsCRP (OR 2.2, 95%CI 1.3-3.8, p=0.005, while having three components was associated with a fourteen-fold higher odds of high hsCRP (OR 13.5, 95%CI 2.4-76.0, p<0.001. ConclusionHigh hsCRP is common among Jamaican young adults and is strongly associated with central obesity and the number of metabolic syndrome

  20. Transient oedema of the cervical spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Sartoretti-Schefer, S.; Kollias, S.; Valavanis, A. [Institute of Neuroradiology, University Hospital of Zuerich (Switzerland)

    2000-04-01

    Transient but very intense oedema of the cervical spinal cord was observed in two patients with obstruction of the cerebrospinal fluid (CSF) pathways. Both presented with hydrocephalus, one due to an infratentorial obstructing mass and the other due to postmeningitic adhesive obstruction of the outlet foramina of the fourth ventricle. In animal experiments with obstruction of CSF pathways (due to outlet foramina obstruction or to downward tentorial herniation) flattening and stretching of the ependymal cells along the central canal is observed, followed by disruption and splitting of the ependymal lining and then by extracellular oedema of the subependymal tissue. Without treatment, frank cavity formation develops in a fourth stage. In our two patients, however, most probably because of appropriate decompressive therapy, the oedema disappeared completely without a residual spinal cord lesion. (orig.)

  1. Immune reconstitution inflammatory syndrome involving the central nervous system in a patient with HIV infection: a case report and review of literature.

    Science.gov (United States)

    Zaffiri, Lorenzo; Verma, Rajanshu; Struzzieri, Kevin; Monterroso, Joanne; Batts, Donald H; Loehrke, Mark E

    2013-01-01

    IRIS is described as a paradoxical deterioration of clinical status upon initiation of combined anti-retroviral therapy (cART) in patients with HIV infection. Immune reconstitution inflammatory syndrome (CNS-IRIS) involving the central nervous system is rarely reported. We describe the case of 57-year-old man who developed a fatal case of CNS- IRIS. A rapid deterioration of neurological status was associated with progression of patchy T2-weighted hyperintensities involving different vascular territories on brain MRI. Diagnosis of CNS-IRIS is based of laboratory and radiologic findings, however brain biopsy is supportive. Despite immune restoration being involved in clinical deterioration, discontinuation of cART is not recommended. The use of corticosteroids is highly controversial. Prompt recognition of CNS-IRIS is crucial for preventing neurological complications and ensuing sequelae.

  2. Sleep apnea syndrome: central sleep apnea and pulmonary hypertension worsened during treatment with auto-CPAP, but improved by adaptive servo-ventilation.

    Science.gov (United States)

    Ono, Hiroshi; Fujimoto, Hiroyuki; Kobayashi, Yoshinori; Kudoh, Shoji; Gemma, Akihiko

    2010-01-01

    In this 71-year-old man diagnosed as obstructive sleep apnea syndrome initially, the apnea-hypopnea index in polysomnography was 31.3/hour. He started auto-adjusted continuous positive airway pressure (auto-CPAP) treatment in July 2005 but developed congestive heart failure in December 2007. Pulmonary arterial pressure (PAP), estimated by echocardiography, was 71 mmHg. In January 2008, during simplified sleep examination with a breath-movement sensor under auto-CPAP, many central-type apneas were recognized. After replacing auto-CPAP with adaptive servo-ventilation (ASV), the apnea-hypopnea index was 5.3/hour and PAP became 36 mmHg after 3 months. It was thought that the increase of PAP was due to long-term inadequate use of auto-CPAP.

  3. Unified-planning, graded-administration, and centralized-controlling: a management modality for treating acquired immune deficiency syndrome with Chinese medicine in Henan Province of China.

    Science.gov (United States)

    Xu, Li-Ran; Guo, Hui-jun; Liu, Zhi-bin; Li, Qiang; Yang, Ji-ping; He, Ying

    2015-04-01

    Henan Province in China has a major epidemic of human immunodeficiency virus/acquired immune deficiency syndrome (HIV/AIDS). Chinese medicine (CM) has been used throughout the last decade, and a management modality was developed, which can be described by unified-planning, graded-administration, and centralized-controlling (UGC). The UGC modality has one primary concept (patient-centered medicine from CM theory), four basic foundations (classifying administrative region, characteristics of CM on disease treatment, health resource conditions, and distribution of patients living with HIV), six important relationships (the "three uniformities and three combinations," and the six relationships therein guide the treatment of AIDS with CM), and four key sections (management, operation, records, and evaluation). In this article, the authors introduce the UGC modality, which could be beneficial to developing countries or resource-limited areas for the management of chronic infectious disease.

  4. [EFFECT OF SYNTHETIC PEPTIDES ON AGING OF PATIENTS WITH CHRONIC POLYMORBIDITY AND ORGANIC BRAIN SYNDROME OF THE CENTRAL NERVOUS SYSTEM IN REMISSION].

    Science.gov (United States)

    Meshchaninov, V N; Tkachenko, E L; Zharkov, S V; Gavrilov, I V; Katyreva, Iu E

    2015-01-01

    We've estimated the cellular and metabolic part of geroprophylactic effects of short synthetic tripeptides vesugen and pinealon for correction of the biological age. 32 people (18 men, 12 women) aged 41-83 years with polymorbidity and the organic brain syndrome in remission participated in the study. The preparations of "Pinealon" and "Vesugen" have had the significant anabolic effect. They have improved the activity of the Central nervous system and other vital organs, which slows the rate of aging by biological age indicators. Vesugen has demonstrated more visible geroprophylactic effect than Pinealon. At the same time we've found the prooxidant activity through chemiluminescence. Decrease of markers CD34+ positive hematopoietic polypotent cells in blood has shown significant inhibition of hemopoiesis. Apparently, the cells have not been involved in the adaptive reactions. Pinealon and Vesugen haven't affected the degree of chromatin condensation, so they are safe on nuclear genetic level. This property should be studied in future. In geriatric practice, we recommend to apply the peptides Pinealon and Vesugen as geroprotectors anabolic neuroprotective and no antioxidant type for reducing the rate of aging in patients with the organic brain syndrome vascular and/or traumatic genesis.

  5. Influence of the Umbilical Cord Insertion Site on the Optimal Individual Birth Weight Achievement

    Directory of Open Access Journals (Sweden)

    Sophie Brouillet

    2014-01-01

    Full Text Available Study Question. To determine whether the umbilical cord insertion site of singleton pregnancies could be linked to the newborn birth weight at term and its individual growth potential achievement. Material and Methods. A cohort study including 528 records of term neonates was performed. Each neonate was assessed for growth adjusted for gestational age according to the infant’s growth potential using the AUDIPOG module. We considered two categories of umbilical cord insertions: central and peripheral. Intrauterine growth restriction was defined as birth weight below the 10th percentile. Statistical analysis was performed using Chi-square, Student’s t test, Wilcoxon test, ANOVA, and logistic regression. Results. We observed a total of 343 centrally inserted cords versus 185 peripheral cords. There were twice as many smokers in the mothers of the peripheral category compared to the centrally inserted ones. More importantly, we demonstrated that only 17/343 (5.0% of infants with central cord insertion were growth restricted, compared to 37/185 (20.0% of the infants born with a peripheral insertion. Neonates with centrally inserted cord were significantly heavier. Conclusion. The umbilical cord insertion site of singleton pregnancies is associated with the newborn’s birth weight at term and its individual growth potential achievement.

  6. 原发性干燥综合征继发中枢神经系统血管炎临床及影像分析%Clinical and Imaging Characteristics of Secondary Central Nervous System Vasculitis Caused by Primary Sjögren's Syndrome

    Institute of Scientific and Technical Information of China (English)

    李伟; 李少武; 张在强

    2015-01-01

    this study. The range age was from 18 to 76 (52±14) years old. Twenty-four patients were involved the central nervous system as initial presentations of PSS. The clinical manifestations of acute cerebral vessel disease were 8 patients (22.86%), including 2 subarachnoid hemorrhage (5.71%), 2 transient ischemic attack (5.71%), 2 arteriovenous fistula (5.71%), 1 cerebral hemorrhage (2.86%), 1 venous sinus thrombosis (2.86%). Other clinical manifestations were including 10 cerebral white matter damage, 6 neuromyelitis optica, 5 spinal cord inlfammations, 3 Parkinson syndrome, 2 trigeminal neuralgia, 1 encephalomyelitis. The imaging ifndings were respectively involved in 3 cases of aneurysm (2 cases of subarachnoid hemorrhage) (8.57%), 2 cases of temporal parietal occipital arteriovenous ifstula (5.71%), 2 case of cerebral arterial stenosis (5.71%) and 1 case of superior sagittal sinus thrombosis (2.86%), 20 cases of paraventricular white matter, 16 cases of subcortical white matter, 8 cases of brainstem, 11 cases of spinal cord, 2 cases of slightly larger ventricle. The single lesion stages (no less than 3) of spinal cord/brainstem were 10 patients.Conclusion The onset of central nervous system involvement in PSS could be form of acute cerebrovascular disease, but the cerebral white matter lesions and spinal cord inflammation are most common. The speciifc imaging characteristic is single lesion involving muti-stages.

  7. Moderate voluntary exercise attenuates the metabolic syndrome in melanocortin-4 receptor-deficient rats showing central dopaminergic dysregulation

    Directory of Open Access Journals (Sweden)

    Silvana Obici

    2015-10-01

    Conclusions: Central dopamine dysregulation during VWR reinforces the link between MC4R function and molecular and behavioral responding to rewards. The data also suggest that exercise can be a successful lifestyle intervention in MC4R-haploinsufficient individuals despite reduced positive reinforcement during exercise training.

  8. Central nervous determination of food storage--a daily switch from conservation to expenditure: implications for the metabolic syndrome.

    Science.gov (United States)

    Kreier, Felix; Kalsbeek, Andries; Ruiter, Marieke; Yilmaz, Ajda; Romijn, Johannes A; Sauerwein, Hans P; Fliers, Eric; Buijs, Ruud M

    2003-11-01

    Here, we present a neuroendocrine concept to review the circularly interacting energy homeostasis system between brain and body. Body-brain interaction is circular because the brain immediately integrates an input to an output, and because part of this response may be that the brain modulates the sensitivity of this perception. First, we describe how the brain senses the body through neurons and blood-borne factors. Direct neuronal connections report the state of various organs. In addition, humoral factors are perceived by the blood-brain barrier and circumventricular organs. We describe how circulating energy carriers are sensed and what signals reach the brain during food intake, exercise and an immune response. We describe that the brain regulates the homeostatic process at two fundamentally different levels during the active and inactive states. The unbalanced output of the brain in the metabolic syndrome is discussed in relation with such circadian rhythms and with regional activity of the autonomic nervous system. In line with the above, we suggest a new approach for the diagnosis and therapy of the metabolic syndrome.

  9. Nestin-positive cells in the spinal cord: a potential source of neural stem cells.

    Science.gov (United States)

    Xu, Renshi; Wu, Chengsi; Tao, Yuhui; Yi, Juan; Yang, Yunzhu; Zhang, Xiong; Liu, Rugao

    2008-11-01

    Some literatures have reported neural precursor cells (NPCs) exist in spinal cord of adult mammal, however, the NPCs distribution feature in spinal cord of adult mice so far is not described in detail. In order to observe and compare the distribution feature of NPCs in various spinal cord regions of adult mice, to research a potential source of neural stem cells (NSCs), we obtained NPCs distribution feature by analyzing the distribution of the nestin-containing cells (NCCs) in spinal cord of adult nestin second-intron enhancer controlled LacZ reporter transgenic mice (pNes-Tg) with LacZ staining and positive cell quantification. The results showed that: NCCs were observed in various regions of spinal cord of adult mice, but amount of NCCs was different in distinct region, the rank order of NCCs amount in various spinal cord regions was dorsal horn region greater than central canal greater than the ventral and lateral horn. NCCs in dorsal horn region mainly distributed in substantia gelatinosa, NCCs in central canal mainly distributed in ependymal zone, on the contrary, NCCs in ventral, lateral horn, medullae, nucleus regions of spinal cord were comparatively less. The rank order of NCCs amount in various spinal cord segments was cervical segment greater than lumbar sacral segment greater than thoracic segment. There was no significantly difference between NCCs amount in the left and right sides, and within cervical 1-7, thoracic 1-12, lumbar 1-5, sacral segment of spinal cord in adult mice. These data collectively indicate that NPCs extensively distribute in various regions of spinal cord of adult mice, especially in substantia gelatinosa and ependymal zone. NPCs in cervical segment are abundant, NPCs in thoracic segment are the least while compared the different spinal cord segment, the NPCs in various regions of spinal cord of adult mice are a potential source of NSCs.

  10. Neuromyelitis optica accompanied by nephrotic syndrome and autoimmune-related pancytopenia.

    Science.gov (United States)

    ZhangBao, Jingzi; Zhou, Lei; Lu, Jiahong; Xi, Jianying; Zhao, Chongbo; Quan, Chao

    2016-05-01

    Neuromyelitis optica (NMO) associated with nephrotic syndrome and autoimmune-related pancytopenia has not been reported previously. We report herein a young woman who initially presented with bilateral blurring of vision and numbness in her hands. MRI disclosed multiple white matter lesions and a long cervical spinal cord lesion extending to the medulla oblongata. Serum aquaporin-4 antibody was positive and the patient was diagnosed with NMO. While in the hospital, she presented with hypoproteinemia and heavy proteinuria, meeting the diagnostic criteria of nephrotic syndrome. After high-dose methylprednisolone treatment, her vision improved significantly and urine protein quantity decreased. However, the patient subsequently developed severe pancytopenia with a positive Coombs' test. Thrombocytopenia finally led to uncontrollable gastrointestinal bleeding as the direct cause of the patient's death. This case illustrates the extremely rare condition of concurrence of NMO, nephrotic syndrome, and autoimmune pancytopenia in one patient, which suggests the involvement of organs beyond the central nervous system in NMO spectrum disorders.

  11. Childhood Central Nervous System Embryonal Tumors Treatment

    Science.gov (United States)

    ... Cord Tumors Treatment Childhood Astrocytomas Treatment Childhood Brain Stem Glioma ... Central nervous system (CNS) embryonal tumors may begin in embryonic (fetal) cells that remain in the brain after birth. ...

  12. Akhirin regulates the proliferation and differentiation of neural stem cells in intact and injured mouse spinal cord.

    Science.gov (United States)

    Abdulhaleem, Felemban Athary M; Song, Xiaohong; Kawano, Rie; Uezono, Naohiro; Ito, Ayako; Ahmed, Giasuddin; Hossain, Mahmud; Nakashima, Kinichi; Tanaka, Hideaki; Ohta, Kunimasa

    2015-05-01

    Although the central nervous system is considered a comparatively static tissue with limited cell turnover, cells with stem cell properties have been isolated from most neural tissues. The spinal cord ependymal cells show neural stem cell potential in vitro and in vivo in injured spinal cord. However, very little is known regarding the ependymal niche in the mouse spinal cord. We previously reported that a secreted factor, chick Akhirin, is expressed in the ciliary marginal zone of the eye, where it works as a heterophilic cell-adhesion molecule. Here, we describe a new crucial function for mouse Akhirin (M-AKH) in regulating the proliferation and differentiation of progenitors in the mouse spinal cord. During embryonic spinal cord development, M-AKH is transiently expressed in the central canal ependymal cells, which possess latent neural stem cell properties. Targeted inactivation of the AKH gene in mice causes a reduction in the size of the spinal cord and decreases BrdU incorporation in the spinal cord. Remarkably, the expression patterns of ependymal niche molecules in AKH knockout (AKH-/-) mice are different from those of AKH+/+, both in vitro and in vivo. Furthermore, we provide evidence that AKH expression in the central canal is rapidly upregulated in the injured spinal cord. Taken together, these results indicate that M-AKH plays a crucial role in mouse spinal cord formation by regulating the ependymal niche in the central canal.

  13. A rare case report of cord entanglement in a post-dated monochorionic monoamniotic twins

    Directory of Open Access Journals (Sweden)

    Kasturi Donimath

    2016-01-01

    Full Text Available Monochorionic Monoamniotic (MCMA twin gestations have been associated perinatal mortality rates as high as 28 to 47%. Umbilical cord entanglements and knots, twin-to-twin transfusion syndrome, congenital anomalies, prematurity and intertwin locking during labour is responsible for their high perinatal morbidity and mortality. We report here a case of Cord entanglement in a post-dated MCMA twin pregnancy without any complications. [Int J Reprod Contracept Obstet Gynecol 2016; 5(1.000: 230-231

  14. Syringomyelia in demyelinating disease of the central nervous system: Report of two cases

    Directory of Open Access Journals (Sweden)

    Savić Dejan

    2011-01-01

    Full Text Available Introduction. Syringomyelia is a cavitary extension inside the spinal cord which can be either symptomatic or congenitally-idiopathic. Syringomyelia during the course of the disease in patients presenting with clinically definite multiple sclerosis was described earlier. Syringomyelia in patients presenting with a clinically isolated syndrome suggestive of multiple sclerosis is unusual. Case Outline. We present two patients presenting with demy-elinating disease of the central nervous system with syringomyelia in the cervical and thoracic spinal cord. We did not find classical clinical signs of syringomyelia in our patients, but we disclosed syringomyelia incidentally during magnetic resonance exploration. Magnetic resonance exploration using the gadolinium contrast revealed the signs of active demyelinating lesions in the spinal cord in one patient but not in the other. Conclusion. Syringomyelia in demyelinating disease of the central nervous system opens the question whether it is a coincidental finding or a part of clinical features of the disease. Differentiation of the significance of syringomyelia finding in these patients plays a role in the choice of treatment concept in such patients.

  15. Central sensitization does not identify patients with carpal tunnel syndrome who are likely to achieve short-term success with physical therapy.

    Science.gov (United States)

    Fernández-de-Las-Peñas, César; Cleland, Joshua A; Ortega-Santiago, Ricardo; de-la-Llave-Rincon, Ana Isabel; Martínez-Perez, Almudena; Pareja, Juan A

    2010-11-01

    The aim of the current study was to identify whether hyperexcitability of the central nervous system is a prognostic factor for individuals with carpal tunnel syndrome (CTS) likely to experience rapid and clinical self-reported improvement following a physical therapy program including soft tissue mobilization and nerve slider neurodynamic interventions. Women presenting with clinical and electrophysiological findings of CTS were involved in a prospective single-arm trial. Participants underwent a standardized examination and then a physical therapy session. The physical therapy sessions included both soft tissue mobilization directed at the anatomical sites of potential median nerve entrapment and a passive nerve slider neurodynamic technique targeted to the median nerve. Pressure pain thresholds (PPT) over the median, radial and ulnar nerves, C5-C6 zygapophyseal joint, carpal tunnel and tibialis anterior muscle were assessed bilaterally. Additionally, thermal detection and pain thresholds were measured over the carpal tunnel and thenar eminence bilaterally to evaluate central nervous system excitability. Subjects were classified as responders (having achieved a successful outcome) or non-responders based on self-perceived recovery. Variables were entered into a stepwise logistic regression model to determine the most accurate variables for determining prognosis. Data from 72 women were included in the analysis, of which 35 experienced a successful outcome (48.6%). Three variables including PPT over the C5-C6 joint affected side 66 points were identified. If 2 out of 3 variables were present (LR + 14.8), the likelihood of success increased from 48.6 to 93.3%. We identified 3 factors that may be associated with a rapid clinical response to both soft tissue mobilization and nerve slider neurodynamic techniques targeted to the median nerve in women presenting with CTS. Our results support that widespread central sensitization may not be present in women with CTS who

  16. Central hypoventilation as the presenting symptom in Hu associated paraneoplastic encephalomyelitis.

    Science.gov (United States)

    Gómez-Choco, Manuel J; Zarranz, Juan J; Saiz, Albert; Forcadas, María I; Graus, Francesc

    2007-10-01

    Central hypoventilation is usually caused by ischaemic or neoplastic lesions of the medulla and upper cervical spinal cord. An autoimmune disorder is not usually considered in the differential diagnosis of this syndrome. We retrospectively identified 14 patients from our database of 202 patients with Hu antibodies who presented with brainstem symptoms. Three were admitted to hospital because of central hypoventilation. All underwent intubation and mechanical ventilation. They could breathe properly while they were awake but suffered deep apnoeas during sleep. Two died, but one is still alive requiring ventilatory assistance during sleep. Autopsy was performed in one of the patients which showed severe inflammatory infiltrates and neuronal loss in the medulla. All patients had normal brain imaging studies and the cause of central hypoventilation was an unsolved problem until Hu antibodies were determined.

  17. Pain following spinal cord injury

    OpenAIRE

    2004-01-01

    The aims of this thesis were to assess and characterise nociceptive and neuropathic pain, the use of pharmacological and non-pharmacological pain treatment, and the influence of pain on the quality of sleep in a population following spinal cord injury (SCI). This thesis is divided into five separate studies: I. Pain in a Swedish spinal cord injury population. II. Gender related differences in pain in spinal cord injured individuals. III. Use of analgesic drugs in indi...

  18. Umbilical cord blood mercury levels in China

    Institute of Scientific and Technical Information of China (English)

    Meiqin Wu,; Chonghuai Yan; Jian Xu; Wei Wu; Hui Li; Xin Zhou

    2013-01-01

    Mercury (Hg) is a well-known neurotoxicant.Hg exposure at high levels can harm individuals of all ages.Even low level exposure to Hg can damage the brain of fetuses and young children,and affect their central nervous system and cognitive development.The aims of our study were to measure total Hg levels in infant umbilical cord blood and to investigate the risk factors associated with total Hg cord blood levels in various cities in China.Our goal was to provide clues for the prevention of Hg exposure in utero.The results indicated that the average cord blood mercury levels (CBMLs) were (1.81 ± 1.93) μg/L,which were lower than those found in most previous studies.The concentrations also differed according to geographic region.The CBMLs were not only associated with family economic and living conditions,but also with diet in pregnant women,especially the intake of marine fish,shellfish,poultry,formula milk and fruits.

  19. Biciliated ependymal cell proliferation contributes to spinal cord growth

    OpenAIRE

    Alfaro-Cervello, Clara; Soriano-Navarro, Mario; Mirzadeh, Zaman; Alvarez-Buylla, Arturo; García Verdugo, José Manuel

    2012-01-01

    Two neurogenic regions have been described in the adult brain, the lateral ventricle subventricular zone and the dentate gyrus subgranular zone. It has been suggested that neural stem cells also line the central canal of the adult spinal cord. Using transmission and scanning electron microscopy and immunostaining, we describe here the organization and cell types of the central canal epithelium in adult mice. The identity of dividing cells was determined by three-dimensional ultrastructural re...

  20. Pain in Down's Syndrome

    Directory of Open Access Journals (Sweden)

    Federica Mafrica

    2006-01-01

    Full Text Available Pain is a homeostatic mechanism that intervenes to protect the organism from harmful stimuli that could damage its integrity. It is made up of two components: the sensory-discriminative component, which identifies the provenance and characteristics of the type of pain; and the affective-motivational component, on which emotional reflexes, following the painful sensation, depend.There is a system for pain control at an encephalic and spinal level, principally made up of the periaqueductal grey matter, the periventricular area, the nucleus raphe magnus, and the pain-inhibition complex situated in the posterior horns of the spinal cord. Through the activation of these pain-control systems, the nervous system suppresses the afference of pain signals. Endogenous opioids represent another analgesic system.In the course of various studies on pain transmission in Down patients, the reduced tolerance of pain and the incapacity to give a qualitative and quantitative description emerged in a powerful way. All of these aspects cause difficulty in evaluating pain. This is linked to several learning difficulties. However, it cannot be excluded that in these anomalies of pain perception, both the anatomical and the neurotransmitter alteration, typical of this syndrome, may hold a certain importance.This fact may have important clinical repercussions that could affect the choice of therapeutic and rehabilitative schemes for treatment of pathologies in which pain is the dominant symptom, such as postoperative pain. It could influence research on analgesics that are more suitable for these patients, the evaluation of the depth of analgesia during surgical operation, and ultimately, absence of obvious pain manifestations. In conclusion, alterations of the central nervous system, neurotransmitters, pain transmission, and all related problems should be considered in the management of pain in patients with Down's syndrome, especially by algologists and

  1. [Pathology of the internal organs and central nervous system in acquired immunodeficiency syndrome (with special reference to opportunistic infections)].

    Science.gov (United States)

    Masini, T; Chinaglia, D; Riviera, L; Capricci, E; Gullotta, F; Spigolon, G; Bauer, A L

    1990-01-01

    Extracerebral and cerebral pathology in AIDS (with particular emphasis on the opportunistic infections). The Authors present the extracerebral pathology of 27 cases of AIDS observed at the Department of Pathology of Milan and the cerebral pathology of 80 cases of AIDS collected by three Institutes (Department of Pathology of Milan, Department of Pathology of Rimini and Department of Neuropathology of Münster) with particular emphasis on the pathology of the opportunistic infections. In the adults' group, the most frequent infections are the protozoan ones (T. gondii) followed with equal incidence by the viral and fungal diseases. In the pediatric group the viral diseases are the most frequently seen. Almost all of the adults show multiple infections in the same organ or in different organs. Diffuse lesions with heavy pathologic fields were observed also without tissue reaction. As to cerebral pathology AIDS' patients with opportunistic infections show focal symptoms, whereas the so called "subacute microglial encephalitis" generally appears as a demential syndrome. In cases with progressive multifocal leukoencephalopathy JC virus was always found and in one case also SV 40 - and BK virus. The diffuse demyelinization in some cases of HIV-Encephalopathy is aspecific. In HIV-positive newborns with cerebral signs, the lesions are characterized by oedema, spongiosis and microcalcifications of the basal ganglia; these are aspecific lesions which can be found in toxic and infectious encephalopathies.

  2. Central sensitization: implications for the diagnosis and treatment of pain.

    Science.gov (United States)

    Woolf, Clifford J

    2011-03-01

    Nociceptor inputs can trigger a prolonged but reversible increase in the excitability and synaptic efficacy of neurons in central nociceptive pathways, the phenomenon of central sensitization. Central sensitization manifests as pain hypersensitivity, particularly dynamic tactile allodynia, secondary punctate or pressure hyperalgesia, aftersensations, and enhanced temporal summation. It can be readily and rapidly elicited in human volunteers by diverse experimental noxious conditioning stimuli to skin, muscles or viscera, and in addition to producing pain hypersensitivity, results in secondary changes in brain activity that can be detected by electrophysiological or imaging techniques. Studies in clinical cohorts reveal changes in pain sensitivity that have been interpreted as revealing an important contribution of central sensitization to the pain phenotype in patients with fibromyalgia, osteoarthritis, musculoskeletal disorders with generalized pain hypersensitivity, headache, temporomandibular joint disorders, dental pain, neuropathic pain, visceral pain hypersensitivity disorders and post-surgical pain. The comorbidity of those pain hypersensitivity syndromes that present in the absence of inflammation or a neural lesion, their similar pattern of clinical presentation and response to centrally acting analgesics, may reflect a commonality of central sensitization to their pathophysiology. An important question that still needs to be determined is whether there are individuals with a higher inherited propensity for developing central sensitization than others, and if so, whether this conveys an increased risk in both developing conditions with pain hypersensitivity, and their chronification. Diagnostic criteria to establish the presence of central sensitization in patients will greatly assist the phenotyping of patients for choosing treatments that produce analgesia by normalizing hyperexcitable central neural activity. We have certainly come a long way since the

  3. Long-lasting beneficial effects of central serotonin receptor 7 stimulation in female mice modeling Rett syndrome

    Directory of Open Access Journals (Sweden)

    Bianca eDe Filippis

    2015-04-01

    Full Text Available Rett syndrome (RTT is a rare neurodevelopmental disorder, characterized by severe behavioral and physiological symptoms. Mutations in the methyl CpG binding protein 2 gene (MECP2 cause more than 95% of classic cases, and currently there is no cure for this devastating disorder. Recently we have demonstrated that specific behavioral and brain molecular alterations can be rescued in MeCP2-308 male mice, a RTT mouse model, by pharmacological stimulation of the brain serotonin receptor 7 (5-HT7R. This member of the serotonin receptor family – crucially involved in the regulation of brain structural plasticity and cognitive processes – can be stimulated by systemic repeated treatment with LP-211, a brain-penetrant selective 5-HT7R agonist. The present study extends previous findings by demonstrating that the LP-211 treatment (0.25 mg/kg, once per day for 7 days rescues RTT-related phenotypic alterations, motor coordination (Dowel test, spatial reference memory (Barnes maze test and synaptic plasticity (hippocampal long-term-potentiation in MeCP2-308 heterozygous female mice, the genetic and hormonal milieu that resembles that of RTT patients. LP-211 also restores the activation of the ribosomal protein S6, the downstream target of mTOR and S6 kinase, in the hippocampus of RTT female mice. Notably, the beneficial effects on neurobehavioral and molecular parameters of a seven-day long treatment with LP-211 were evident up to two months after the last injection, thus suggesting long-lasting effects on RTT-related impairments. Taken together with our previous study, these results provide compelling preclinical evidence of the potential therapeutic value for RTT of a pharmacological approach targeting the brain 5-HT7R.

  4. Development of central nervous system autoimmunity is impaired in the absence of Wiskott-Aldrich syndrome protein.

    Directory of Open Access Journals (Sweden)

    Marita Bosticardo

    Full Text Available Wiskott-Aldrich Syndrome protein (WASP is a key regulator of the actin cytoskeleton in hematopoietic cells. Defective expression of WASP leads to multiple abnormalities in different hematopoietic cells. Despite severe impairment of T cell function, WAS patients exhibit a high prevalence of autoimmune disorders. We attempted to induce EAE, an animal model of organ-specific autoimmunity affecting the CNS that mimics human MS, in Was(-/- mice. We describe here that Was(-/- mice are markedly resistant against EAE, showing lower incidence and milder score, reduced CNS inflammation and demyelination as compared to WT mice. Microglia was only poorly activated in Was(-/- mice. Antigen-induced T-cell proliferation, Th-1 and -17 cytokine production and integrin-dependent adhesion were increased in Was(-/- mice. However, adoptive transfer of MOG-activated T cells from Was(-/- mice in WT mice failed to induce EAE. Was(-/- mice were resistant against EAE also when induced by adoptive transfer of MOG-activated T cells from WT mice. Was(+/- heterozygous mice developed an intermediate clinical phenotype between WT and Was(-/- mice, and they displayed a mixed population of WASP-positive and -negative T cells in the periphery but not in their CNS parenchyma, where the large majority of inflammatory cells expressed WASP. In conclusion, in absence of WASP, T-cell responses against a CNS autoantigen are increased, but the ability of autoreactive T cells to induce CNS autoimmunity is impaired, most probably because of an inefficient T-cell transmigration into the CNS and defective CNS resident microglial function.

  5. Long-lasting beneficial effects of central serotonin receptor 7 stimulation in female mice modeling Rett syndrome.

    Science.gov (United States)

    De Filippis, Bianca; Chiodi, Valentina; Adriani, Walter; Lacivita, Enza; Mallozzi, Cinzia; Leopoldo, Marcello; Domenici, Maria Rosaria; Fuso, Andrea; Laviola, Giovanni

    2015-01-01

    Rett syndrome (RTT) is a rare neurodevelopmental disorder, characterized by severe behavioral and physiological symptoms. Mutations in the methyl CpG binding protein 2 gene (MECP2) cause more than 95% of classic cases, and currently there is no cure for this devastating disorder. Recently we have demonstrated that specific behavioral and brain molecular alterations can be rescued in MeCP2-308 male mice, a RTT mouse model, by pharmacological stimulation of the brain serotonin receptor 7 (5-HT7R). This member of the serotonin receptor family-crucially involved in the regulation of brain structural plasticity and cognitive processes-can be stimulated by systemic repeated treatment with LP-211, a brain-penetrant selective 5-HT7R agonist. The present study extends previous findings by demonstrating that the LP-211 treatment (0.25 mg/kg, once per day for 7 days) rescues RTT-related phenotypic alterations, motor coordination (Dowel test), spatial reference memory (Barnes maze test) and synaptic plasticity (hippocampal long-term-potentiation) in MeCP2-308 heterozygous female mice, the genetic and hormonal milieu that resembles that of RTT patients. LP-211 also restores the activation of the ribosomal protein (rp) S6, the downstream target of mTOR and S6 kinase, in the hippocampus of RTT female mice. Notably, the beneficial effects on neurobehavioral and molecular parameters of a seven-day long treatment with LP-211 were evident up to 2 months after the last injection, thus suggesting long-lasting effects on RTT-related impairments. Taken together with our previous study, these results provide compelling preclinical evidence of the potential therapeutic value for RTT of a pharmacological approach targeting the brain 5-HT7R.

  6. Cutting the Cord-2

    Science.gov (United States)

    2004-01-01

    This animation shows the view from the rear hazard avoidance cameras on the Mars Exploration Rover Spirit as the rover turns 45 degrees clockwise. This maneuver is the first step in a 3-point turn that will rotate the rover 115 degrees to face west. The rover must make this turn before rolling off the lander because airbags are blocking it from exiting from the front lander petal. Before this crucial turn took place, engineers instructed the rover to cut the final cord linking it to the lander. The turn took around 30 minutes to complete.

  7. Cutting the Cord

    Science.gov (United States)

    2004-01-01

    This animation shows the view from the front hazard avoidance cameras on the Mars Exploration Rover Spirit as the rover turns 45 degrees clockwise. This maneuver is the first step in a 3-point turn that will rotate the rover 115 degrees to face west. The rover must make this turn before rolling off the lander because airbags are blocking it from exiting off the front lander petal. Before this crucial turn could take place, engineers instructed the rover to cut the final cord linking it to the lander. The turn took around 30 minutes to complete.

  8. Biomarkers in spinal cord injury.

    NARCIS (Netherlands)

    Pouw, M.H.; Hosman, A.J.F.; Middendorp, J.J. van; Verbeek, M.M.; Vos, P.E.; Meent, H. van de

    2009-01-01

    STUDY DESIGN: Literature review. OBJECTIVES: In traumatic spinal cord injury (SCI), much effort has been put into the evaluation of SCI severity and the prediction of recovery potential. An accurate prediction of the initial damage of the spinal cord that differentiates between the severities of SCI

  9. [Stiff-person syndrome and related autoantibodies].

    Science.gov (United States)

    Tomioka, Ryo; Tanaka, Keiko

    2013-04-01

    Central nervous system hyperexcitability disorders, known as stiff-man/person syndrome (SPS), are thought to be related to the regulatory disturbance of inhibitory synaptic transmission of motor neurons in the brainstem and spinal cord. SPS is characterized by stiffness and spasms of the axis and limbs and is divided into two clinical subgroups: classic SPS, which affects the lumbar, trunk, and proximal limb muscles, and SPS-plus syndrome. The latter comprises (1) the stiff-limb subtype, in which symptom is limited to the lower limbs; (2) jerking stiff-man syndrome, characterized by chronically progressive stiffness and myoclonus; and (3) acute-onset and progressive encephalomyelitis with rigidity and myoclonus. Almost 80% of patients with classic SPS harbor autoantibodies against glutamic acid decarboxylase 65 (GAD65). In approximately 30-40% of patients, SPS accompanies type I diabetes, and anti-GAD65 antibodies are detected frequently in type I diabetes. However, the antibody-recognizing epitopes might be different between SPS and diabetes. Other autoantibodies against glycine receptor α1 (12% of patients with SPS) and GABA(A)-receptor associated protein (70% of patients with SPS) have been reported. In paraneoplastic SPS, anti-amphiphysin antibodies have been shown in patients with breast cancer or small cell lung cancer. One case of mediastinal tumor with anti-gephyrin antibodies has also been reported. However, the roles of these autoantibodies in the pathomechanisms of SPS have not yet been elucidated.

  10. Retraining the injured spinal cord

    Science.gov (United States)

    Edgerton, V. R.; Leon, R. D.; Harkema, S. J.; Hodgson, J. A.; London, N.; Reinkensmeyer, D. J.; Roy, R. R.; Talmadge, R. J.; Tillakaratne, N. J.; Timoszyk, W.; Tobin, A.

    2001-01-01

    The present review presents a series of concepts that may be useful in developing rehabilitative strategies to enhance recovery of posture and locomotion following spinal cord injury. First, the loss of supraspinal input results in a marked change in the functional efficacy of the remaining synapses and neurons of intraspinal and peripheral afferent (dorsal root ganglion) origin. Second, following a complete transection the lumbrosacral spinal cord can recover greater levels of motor performance if it has been exposed to the afferent and intraspinal activation patterns that are associated with standing and stepping. Third, the spinal cord can more readily reacquire the ability to stand and step following spinal cord transection with repetitive exposure to standing and stepping. Fourth, robotic assistive devices can be used to guide the kinematics of the limbs and thus expose the spinal cord to the new normal activity patterns associated with a particular motor task following spinal cord injury. In addition, such robotic assistive devices can provide immediate quantification of the limb kinematics. Fifth, the behavioural and physiological effects of spinal cord transection are reflected in adaptations in most, if not all, neurotransmitter systems in the lumbosacral spinal cord. Evidence is presented that both the GABAergic and glycinergic inhibitory systems are up-regulated following complete spinal cord transection and that step training results in some aspects of these transmitter systems being down-regulated towards control levels. These concepts and observations demonstrate that (a) the spinal cord can interpret complex afferent information and generate the appropriate motor task; and (b) motor ability can be defined to a large degree by training.

  11. Umbilical cord blood transplantation.

    Science.gov (United States)

    Koo, Hong Hoe; Ahn, Hyo Seop

    2012-07-01

    Since the first umbilical cord blood transplantation (CBT) in 1998, cord blood (CB) has now become one of the most commonly used sources of hematopoietic stem cells for transplantation. CBT has advantages of easy procurement, no risk to donor, low risk of transmitting infections, immediate availability and immune tolerance allowing successful transplantation despite human leukocyte antigen disparity. Several studies have shown that the number of cells transplanted is the most important factor for engraftment in CBT, and it limits the wide use of CB in adult patients. New strategies for facilitating engraftment and reducing transplantation-related mortality are ongoing in the field of CBT and include the use of a reduced-intensity conditioning regimen, double-unit CBT, ex vivo expansion of CB, and co-transplantation of CB and mesenchymal stem cells. Recently, the results of two international studies with large sample sizes showed that CB is an acceptable alternative source of hematopoietic stem cells for adult recipients who lack human leukocyte antigen-matched adult donors. Along with the intensive researches, development in banking process of CB will amplify the use of CB and offer the chance for cure in more patients.

  12. Umbilical cord blood transplantation

    Directory of Open Access Journals (Sweden)

    Hong Hoe Koo

    2012-07-01

    Full Text Available Since the first umbilical cord blood transplantation (CBT in 1998, cord blood (CB has now become one of the most commonly used sources of hematopoietic stem cells for transplantation. CBT has advantages of easy procurement, no risk to donor, low risk of transmitting infections, immediate availability and immune tolerance allowing successful transplantation despite human leukocyte antigen disparity. Several studies have shown that the number of cells transplanted is the most important factor for engraftment in CBT, and it limits the wide use of CB in adult patients. New strategies for facilitating engraftment and reducing transplantation-related mortality are ongoing in the field of CBT and include the use of a reduced-intensity conditioning regimen, double-unit CBT, ex vivo expansion of CB, and co-transplantation of CB and mesenchymal stem cells. Recently, the results of two international studies with large sample sizes showed that CB is an acceptable alternative source of hematopoietic stem cells for adult recipients who lack human leukocyte antigen-matched adult donors. Along with the intensive researches, development in banking process of CB will amplify the use of CB and offer the chance for cure in more patients.

  13. Investigation of magnetization transfer ratio-derived pial and subpial abnormalities in the multiple sclerosis spinal cord.

    Science.gov (United States)

    Kearney, Hugh; Yiannakas, Marios C; Samson, Rebecca S; Wheeler-Kingshott, Claudia A M; Ciccarelli, Olga; Miller, David H

    2014-09-01

    Neuropathological studies in multiple sclerosis have suggested that meningeal inflammation in the brain may be linked to disease progression. Inflammation in the spinal cord meninges has been associated with axonal loss, a pathological substrate for disability. Quantitative magnetic resonance imaging facilitates the investigation of spinal cord microstructure by approximating histopathological changes. We acquired structural and quantitative imaging of the cervical spinal cord from which we calculated magnetization transfer ratio in the outer spinal cord-an area corresponding to the expected location of the pia mater and subpial region-and in spinal cord white and grey matter. We studied 26 healthy controls, 22 people with a clinically isolated syndrome, 29 with relapsing-remitting, 28 with secondary-progressive and 28 with primary-progressive multiple sclerosis. Magnetization transfer ratio values in the outermost region of the spinal cord were higher than the white matter in controls and patients: controls (51.35 ± 1.29 versus 49.87 ± 1.45, P spinal cord magnetization transfer ratio values were seen in controls than all patient groups: clinically isolated syndrome (coefficient = -0.32, P = 0.03), relapsing-remitting (coefficient = -0.48, P < 0.01), secondary-progressive (coefficient = -0.51, P < 0.01) and primary-progressive multiple sclerosis (coefficient = -0.38, P < 0.01). In a regression analysis correcting for age and cord area, magnetization transfer ratio values in the outer cord were lower in relapsing-remitting multiple sclerosis compared with clinically isolated syndrome (coefficient = -0.28, P = 0.02), and both primary and secondary-progressive compared to relapsing-remitting multiple sclerosis (coefficients = -0.29 and -0.24, respectively, P = 0.02 for both). In the clinically isolated syndrome and relapsing-remitting multiple sclerosis groups, outer cord magnetization transfer ratio was decreased in the absence of significant cord atrophy. In a

  14. Microglia and Spinal Cord Synaptic Plasticity in Persistent Pain

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    Sarah Taves

    2013-01-01

    Full Text Available Microglia are regarded as macrophages in the central nervous system (CNS and play an important role in neuroinflammation in the CNS. Microglial activation has been strongly implicated in neurodegeneration in the brain. Increasing evidence also suggests an important role of spinal cord microglia in the genesis of persistent pain, by releasing the proinflammatory cytokines tumor necrosis factor-alpha (TNFα, Interleukine-1beta (IL-1β, and brain derived neurotrophic factor (BDNF. In this review, we discuss the recent findings illustrating the importance of microglial mediators in regulating synaptic plasticity of the excitatory and inhibitory pain circuits in the spinal cord, leading to enhanced pain states. Insights into microglial-neuronal interactions in the spinal cord dorsal horn will not only further our understanding of neural plasticity but may also lead to novel therapeutics for chronic pain management.

  15. Localization of cholecystokininlike immunoreactivity in the rat spinal cord, with particular reference to the autonomic innervation of the pelvic organs

    DEFF Research Database (Denmark)

    Schrøder, H D

    1983-01-01

    The distribution of cholecystokinin in the spinal cord was investigated by immunohistochemistry. Throughout the length of the spinal cord cholecystokinin immunoreactivity was found in laminae I and II, in the spinal reticular nucleus, and in the surroundings of the central canal. On the basis of ...

  16. Reconstitution of the central and peripheral nervous system during salamander tail regeneration.

    Science.gov (United States)

    McHedlishvili, Levan; Mazurov, Vladimir; Grassme, Kathrin S; Goehler, Kerstin; Robl, Bernhard; Tazaki, Akira; Roensch, Kathleen; Duemmler, Annett; Tanaka, Elly M

    2012-08-21

    We show that after tail amputation in Ambystoma mexicanum (Axolotl) the correct number and spacing of dorsal root ganglia are regenerated. By transplantation of spinal cord tissue and nonclonal neurospheres, we show that the central spinal cord represents a source of peripheral nervous system cells. Interestingly, melanophores migrate from preexisting precursors in the skin. Finally, we demonstrate that implantation of a clonally derived spinal cord neurosphere can result in reconstitution of all examined cell types in the regenerating central spinal cord, suggesting derivation of a cell with spinal cord stem cell properties.

  17. HERC 1 Ubiquitin Ligase Mutation Affects Neocortical, CA3 Hippocampal and Spinal Cord Projection Neurons: An Ultrastructural Study.

    Science.gov (United States)

    Ruiz, Rocío; Pérez-Villegas, Eva María; Bachiller, Sara; Rosa, José Luis; Armengol, José Angel

    2016-01-01

    The spontaneous mutation tambaleante is caused by the Gly483Glu substitution in the highly conserved N terminal RCC1-like domain of the HERC1 protein, which leads to the increase of mutated protein levels responsible for cerebellar Purkinje cell death by autophagy. Until now, Purkinje cells have been the only central nervous neurons reported as being targeted by the mutation, and their degeneration elicits an ataxic syndrome in adult mutant mice. However, the ultrastructural analysis performed here demonstrates that signs of autophagy, such as autophagosomes, lysosomes, and altered mitochondria, are present in neocortical pyramidal, CA3 hippocampal pyramidal, and spinal cord motor neurons. The main difference is that the reduction in the number of neurons affected in the tambaleante mutation in the neocortex, the hippocampus, and the spinal cord is not so evident as the dramatic loss of cerebellar Purkinje cells. Interestingly, signs of autophagy are absent in both interneurons and neuroglia cells. Affected neurons have in common that they are projection neurons which receive strong and varied synaptic inputs, and possess the highest degree of neuronal activity. Therefore, because the integrity of the ubiquitin-proteasome system is essential for protein degradation and hence, for normal protein turnover, it could be hypothesized that the deleterious effects of the misrouting of these pathways would depend directly on the neuronal activity.

  18. HERC 1 ubiquitin ligase mutation affects neocortical, CA3 hippocampal and spinal cord projection neurons. An ultrastructural study

    Directory of Open Access Journals (Sweden)

    Rocío eRuiz

    2016-04-01

    Full Text Available The spontaneous mutation tambaleante is caused by the Gly483Glu substitution in the highly conserved N terminal RCC1-like domain of the HERC1 protein, which leads to the increase of mutated protein levels responsible for cerebellar Purkinje cell death by autophagy. Until now, Purkinje cells have been the only central nervous neurons reported as being targeted by the mutation, and their degeneration elicits an ataxic syndrome in adult mutant mice. However, the ultrastructural analysis performed here demonstrates that signs of autophagy, such as autophagosomes, lysosomes, and altered mitochondria, are present in neocortical pyramidal, CA3 hippocampal pyramidal, and spinal cord motor neurons. The main difference is that the reduction in the number of neurons affected in the tambaleante mutation in the neocortex, the hippocampus, and the spinal cord is not so evident as the dramatic loss of cerebellar Purkinje cells. Interestingly, signs of autophagy are absent in both interneurons and neuroglia cells. Affected neurons have in common that they are projection neurons which receive strong and varied synaptic inputs, and possess the highest degree of neuronal activity. Therefore, because the integrity of the ubiquitin-proteasome system is essential for protein degradation and, hence, for normal protein turnover, it could be hypothesized that the deleterious effects of the misrouting of these pathways would depend directly on the neuronal activity.

  19. The astrocytic transporter SLC7A10 (Asc-1) mediates glycinergic inhibition of spinal cord motor neurons

    Science.gov (United States)

    Ehmsen, Jeffrey T.; Liu, Yong; Wang, Yue; Paladugu, Nikhil; Johnson, Anna E.; Rothstein, Jeffrey D.; du Lac, Sascha; Mattson, Mark P.; Höke, Ahmet

    2016-01-01

    SLC7A10 (Asc-1) is a sodium-independent amino acid transporter known to facilitate transport of a number of amino acids including glycine, L-serine, L-alanine, and L-cysteine, as well as their D-enantiomers. It has been described as a neuronal transporter with a primary role related to modulation of excitatory glutamatergic neurotransmission. We find that SLC7A10 is substantially enriched in a subset of astrocytes of the caudal brain and spinal cord in a distribution corresponding with high densities of glycinergic inhibitory synapses. Accordingly, we find that spinal cord glycine levels are significantly reduced in Slc7a10-null mice and spontaneous glycinergic postsynaptic currents in motor neurons show substantially diminished amplitudes, demonstrating an essential role for SLC7A10 in glycinergic inhibitory function in the central nervous system. These observations establish the etiology of sustained myoclonus (sudden involuntary muscle movements) and early postnatal lethality characteristic of Slc7a10-null mice, and implicate SLC7A10 as a candidate gene and auto-antibody target in human hyperekplexia and stiff person syndrome, respectively. PMID:27759100

  20. Research Progress of the Effect of Metabolic Syndrome and Exercise Intervention on Central Obesity%代谢综合征与运动干预对中心性肥胖影响的研究进展

    Institute of Scientific and Technical Information of China (English)

    李婷; 杨烨

    2014-01-01

    代谢综合征(metabolic syndrome,MS)是一系列代谢异常成分在同一个体聚集的临床综合症状,中心性肥胖是其重要的发病危险因素和临床表现之一。此外,有诸多研究显示中心性肥胖还是胰岛素抵抗(insulin resistance,IR)的一个重要发病基础。中心性肥胖症患者实施运动干预,对MS、IR有着显著的影响。本文通过文献资料法就中心性肥胖的危害和运动干预对中心性肥胖的影响进行综述。%Metabolic syndrome(MS) is a clinical syndrome of a series of abnormal metabolic factors or components clustered together in one individual. Central obesity is one of the important risk factors and clinical manifestations. Besides, various researches have shown that central obesity is also an important pathogenic basis of insulin resistance. It has been found that exercise intervention in patients with central obesity has signiifcant effect on MS and IR . By the method of literature study, the paper discusses the harm of central obesity and the effect of exercise intervention on central obesity .

  1. Neurogenic bladder in Hunter's syndrome.

    Science.gov (United States)

    Koyama, K; Moda, Y; Sone, A; Tanaka, H; Hino, Y

    1994-01-01

    We encountered a rare patient with Hunter's syndrome who exhibited urinary retention as a result of a neurogenic bladder, uninhibited detrusor contractions, and detrusor-sphincter dyssynergia. Neurological findings were consistent with cervical myelopathy and cervical MR imaging showed very narrow segments at the cord level C2-4. We speculate that this Hunter's syndrome patient has cervical myelopathy and that this neurological dysfunction causes the neurogenic bladder. PMID:8014981

  2. Central cholinergic regulation of respiration: nicotinic receptors

    Institute of Scientific and Technical Information of China (English)

    Xuesi M SHAO; Jack L FELDMAN

    2009-01-01

    Nicotinic acetylcholine receptors (nAChRs) are expressed in brainstem and spinal cord regions involved in the control of breathing. These receptors mediate central cholinergic regulation of respiration and effects of the exogenous ligand nicotine on respiratory pattern. Activation of a4* nAChRs in the preBotzinger Complex (preBotC), an essential site for normal respiratory rhythm generation in mammals, modulates excitatory glutamatergic neurotransmission and depolarizes preBotC inspiratory neurons, leading to increases in respiratory frequency. nAChRs are also present in motor nuclei innervating respiratory muscles. Activation of post- and/or extra-synaptic a4* nAChRs on hypoglossal (XII) motoneurons depolarizes these neurons, potentiating tonic and respiratory-related rhythmic activity. As perinatal nicotine exposure may contribute to the pathogenesis of sudden infant death syndrome (SIDS), we discuss the effects of perinatal nicotine exposure on development of the cholinergic and other neurotransmitter systems involved in control of breathing. Advances in understanding of the mechanisms underlying central cholinergic/nicotinic modulation of respiration provide a pharmacological basis for exploiting nAChRs as therapeutic targets for neurological disorders related to neural control of breathing such as sleep apnea and SIDS.

  3. Central centrifugal cicatricial alopecia

    Directory of Open Access Journals (Sweden)

    Collin Blattner

    2013-01-01

    Full Text Available Central centrifugal cicatricial alopecia is a common cause of progressive permanent apical alopecia. This unique form of alopecia includes entities previously know as "hot comb alopecia," "follicular degeneration syndrome," "pseudopelade" in African Americans and "central elliptical pseudopelade" in Caucasians. The etiology appears to be multifactorial and the condition occurs in all races.

  4. International Spinal Cord Injury

    DEFF Research Database (Denmark)

    Dvorak, M F; Itshayek, E; Fehlings, M G;

    2015-01-01

    of the completion of the intervention or surgical closure; (6) Surgical procedure-open reduction, (7) Surgical procedure-direct decompression of neural elements, and (8 and 9) Surgical procedure-stabilization and fusion (spinal segment number and level). All variables are coded using numbers or characters. Each...... spinal intervention and procedure is coded (variables 1 through 7) and the spinal segment level is described (variables 8 and 9). Sample clinical cases were developed to illustrate how to complete it. CONCLUSION: The International SCI Spinal Interventions and Surgical Procedures Basic Data Set......STUDY DESIGN: Survey of expert opinion, feedback and final consensus. OBJECTIVE: To describe the development and the variables included in the International Spinal Cord Injury (SCI) Spinal Interventions and Surgical Procedures Basic Data set. SETTING: International working group. METHODS...

  5. Maladaptive spinal plasticity opposes spinal learning and recovery in spinal cord injury

    Directory of Open Access Journals (Sweden)

    Adam R Ferguson

    2012-10-01

    Full Text Available Synaptic plasticity within the spinal cord has great potential to facilitate recovery of function after spinal cord injury (SCI. Spinal plasticity can be induced in an activity-dependent manner even without input from the brain after complete SCI. The mechanistic basis for these effects is provided by research demonstrating that spinal synapses have many of the same plasticity mechanisms that are known to underlie learning and memory in the brain. In addition, the lumbar spinal cord can sustain several forms of learning and memory, including limb-position training. However, not all spinal plasticity promotes recovery of function. Central sensitization of nociceptive (pain pathways in the spinal cord may emerge with certain patterns of activity, demonstrating that plasticity within the spinal cord may contribute to maladaptive pain states. In this review we discuss interactions between adaptive and maladaptive forms of activity-dependent plasticity in the spinal cord. The literature demonstrates that activity-dependent plasticity within the spinal cord must be carefully tuned to promote adaptive spinal training. Stimulation that is delivered in a limb position-dependent manner or on a fixed interval can induce adaptive plasticity that promotes future spinal cord learning and reduces nociceptive hyper-reactivity. On the other hand, stimulation that is delivered in an unsynchronized fashion, such as randomized electrical stimulation or peripheral skin injuries, can generate maladaptive spinal plasticity that undermines future spinal cord learning, reduces recovery of locomotor function, and promotes nociceptive hyper-reactivity after spinal cord injury. We review these basic phenomena, discuss the cellular and molecular mechanisms, and discuss implications of these findings for improved rehabilitative therapies after spinal cord injury.

  6. Effect of rehabilitation nursing on postoperative functional recovery of neurogenic bladder in patients with tethered cord syndrome%康复护理对脊髓栓系综合征术后神经源性膀胱功能恢复的影响

    Institute of Scientific and Technical Information of China (English)

    王熠平; 黄天雯; 杨秀玉; 苏霞; 蔡丽娥

    2012-01-01

    Objective To explore the effect of rehabilitation nursing on postoperative functional recovery of neurogenic bladder in patients with tethered cord syndrome (TCS). Methods From June 2008 to August 2011, 11 patients with neurogenic bladder from TCS accepted the rehabilitation nursing with urinary catheterization, intermittent urethral catheterication, training of bladder function, electric stimulation and health education. The functional recovery of neurogenic bladder before the rehabilitation nursing was compared with that after the rehabilitative care. Results The bladder volume after withdrawing urinary catheter was more than that on admission by training of bladder function and electric stimulation during continuous urinary catheterization (P < 0.01). The bladder volume was larger and the residual urine volume was small than that at the time of withdrawing urinary catheter after performing intermittent urethral catheterication for one month (P < 0.01). Conclusion Rehabilitative nursing intervention is good for the reconstruction of bladder function of the TCS patients with neurogenic bladder as well as for the improvement of the therapeutic quality.%目的 探讨康复护理对脊髓栓系综合征(tethered cord syndrome,TCS)术后神经源性膀胱功能恢复的影响.方法 选择2008年6月~2011年6月本院收治的11例TCS术后神经源性膀胱患者,住院期间接受持续留置尿管、间歇导尿、膀胱功能训练、电刺激及健康教育等康复护理措施,比较应用康复护理措施前后患者膀胱功能的恢复情况.结果 患者在留置尿管期间使用膀胱功能训练和电刺激等康复措施,拔尿管时膀胱容量大于人院时(P<0.01);患者接受间歇导尿1个月后,膀胱容量大于拔管时,残余尿量少于拔管时(均P< 0.01).结论 康复护理措施有利于TCS伴神经源性膀胱患者术后重建膀胱功能,提高患者的治疗质量.

  7. Propitious Therapeutic Modulators to Prevent Blood-Spinal Cord Barrier Disruption in Spinal Cord Injury.

    Science.gov (United States)

    Kumar, Hemant; Ropper, Alexander E; Lee, Soo-Hong; Han, Inbo

    2016-05-18

    The blood-spinal cord barrier (BSCB) is a specialized protective barrier that regulates the movement of molecules between blood vessels and the spinal cord parenchyma. Analogous to the blood-brain barrier (BBB), the BSCB plays a crucial role in maintaining the homeostasis and internal environmental stability of the central nervous system (CNS). After spinal cord injury (SCI), BSCB disruption leads to inflammatory cell invasion such as neutrophils and macrophages, contributing to permanent neurological disability. In this review, we focus on the major proteins mediating the BSCB disruption or BSCB repair after SCI. This review is composed of three parts. Section 1. SCI and the BSCB of the review describes critical events involved in the pathophysiology of SCI and their correlation with BSCB integrity/disruption. Section 2. Major proteins involved in BSCB disruption in SCI focuses on the actions of matrix metalloproteinases (MMPs), tumor necrosis factor alpha (TNF-α), heme oxygenase-1 (HO-1), angiopoietins (Angs), bradykinin, nitric oxide (NO), and endothelins (ETs) in BSCB disruption and repair. Section 3. Therapeutic approaches discusses the major therapeutic compounds utilized to date for the prevention of BSCB disruption in animal model of SCI through modulation of several proteins.

  8. Acute spinal cord injury and neurogenic shock in pregnancy.

    Science.gov (United States)

    Gilson, G J; Miller, A C; Clevenger, F W; Curet, L B

    1995-07-01

    A case of a pregnant woman with a subluxation of C-6 on C-7 with acute quadriplegia and sensory loss to the T-10 dermatome is described. Hemodynamic and fetal monitoring during the 3-week period of neurogenic shock resulted in good maternal and fetal outcomes. Pulmonary complications and anesthetic issues are important aspects of the care of these critically ill patients. Major trauma is a common cause of death and disability in young adults and may contribute to as much as 15 percent of nonobstetric maternal deaths. Spinal cord injuries involve young women in 15 percent of cases. The literature is replete with information on the obstetric management of patients with preexisting spinal cord injury (1-4) but there is little on the management and special problems of the pregnant patient with acute spinal cord trauma. We report here the management of a case of acute cord transection accompanied by spinal shock and discuss the specific maternal as well as fetal considerations in this syndrome.

  9. Spinal cord toxoplasmosis in AIDS; Toxoplasmose medullaire et sida

    Energy Technology Data Exchange (ETDEWEB)

    Carteret, M.; Petit, E.; Granat, O.; Marichez, M.; Gilquin, J. [Hopital Saint-Joseph, 69 - Lyon (France)

    1995-07-01

    Toxoplasmosis is the most common brain parasitic infection in acquired immunodeficiency syndrome (AIDS). Spinal cord localizations are still rare (2 cases with cerebral involvement, 2 cases without). A case of both spinal cord and cerebral involvement is reported. Magnetic resonance imaging (MR imaging) was performed because of sensory level (L 1). A focal conus medullaris enlargement was seen, iso intense on T 1 weighted images. This lesion was hyperintense on T 2 weighted sequence, and was homogeneously enhanced after Gadolinium on T 1 weighted images. A medullary oedema was noted. A toxoplasmosis treatment was initiated, without cortico therapy. MR imaging performed one month later (D 30), while important clinical improvements were seen, pointed out normal thickness of conus medullaris, without enhancement after Gadolinium. Disease lesions in AIDS with focal spinal cord processes are reviewed, and diagnostic work-up is discussed. Spinal cord single lesion, associated or not with brain involvements should be treated as a toxoplasmic infection, with MR imaging follow up. This work up should avoid medullary biopsy, still required in case of treatment failure. Cerebral involvements, with multiples lesions can mask medullary localization. (authors). 8 refs., 2 figs.

  10. Intraspinal microstimulation A novel technique for the functional recovery of spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Hua Zhang; Liqun Feng; Yuping Wang

    2010-01-01

    Nonspecific neuronal activity elicited by intraspinal microstimulation in the intermediate and ventral gray matter of thoracic spinal segments caudal to a complete spinal cord transection significantly increased the rat hindlimb Basso,Beattie,Bresnahan locomotor score by activating the central pattern generator located in the lumbar spinal cord.However,the best region for intraspinal microstimulation is unclear.Using an incomplete spinal cord injury model at T8,we compared the use of intraspinal microstimulation to activate the spinal cord in rats with a spontaneous recovery group.The intraspinal microstimulation group recovered sooner and showed three kinds of movement: the left hindlimb,the left hindlimb toes,and the paraspinal muscles and tails.These had different microstimulation thresholds.There was mild hyperplasia of the astrocytes surrounding the tips of the microelectrodes and slight inflammatory reactions nearby.These results indicate that implantation of microelectrodes was relatively safe and induced minimal damage to the lumbar-sacral spinal cord.Intraspinal microstimulation in the lumbar sacral spinal cord may improve leg movements after spinal cord injury.Non-specific intraspinal microstimulation may be a novel technique for the recovery of spinal cord injuries.

  11. The meningeal sheath of the regenerating spinal cord of the eel, Anguilla.

    Science.gov (United States)

    Dervan, Adrian G; Roberts, Barry L

    2003-09-01

    We describe here the meningeal sheath that encloses the spinal cord, and the sheath that develops when the cord regenerates after a total transection. This description is derived from electron and light microscopy. The sheath of the uninjured cord was found to be a single structure of two parts: an outer, thin melanocyte layer and an inner, thicker layer of 2 to 10 rows of fibroblasts, closely associated with collagen and elastic fibers. Soon after cord transection, the injured axons re-grow and, together with the reforming central canal, create a bridge that links the transected cord within 8 days of injury. This bridge is covered at first by a rudimentary meningeal sheath, formed of fibroblasts and macrophages, that later progressively thickens and becomes more compact. By about day 20, the fibroblasts are arranged as 16 to 20 loose rows that include bundles of collagen, oriented along the rostro-caudal axis of the cord. Even after 144 days, the meninx, although substantially thicker than normal because of the numerous fibroblast rows (20 to 30), still lacks the melanocyte layer. In cases in which the meninx at the transection site was mechanically and pharmacologically (6-hydroxydopamine) disrupted, bridge formation was essentially unchanged, and axonal regrowth continued; some regrowing axons, however, extruded from the denuded cord. Accordingly, our findings indicate that although the meningeal sheath is not essential for cord regeneration to take place, it may well facilitate recovery by providing mechanical guidance and support to the regrowing axons.

  12. Depression and Spinal Cord Injury

    Science.gov (United States)

    ... About Us Patient Care Resources Information & Education SCI Empowerment Project Projects & Research FAQ © 2017 University of Washington ... Ave., Seattle WA 98104 Spinal Cord Injury Clinic nurses: 206-744-5862 University of Washington Medical Center ...

  13. Multiple System Atrophy Manifested by Bilateral Vocal Cord Palsy as an Initial Sign

    Directory of Open Access Journals (Sweden)

    Yuri Seo

    2015-05-01

    Full Text Available A 71-year-old male initially presented with vocal cord palsy and underwent tracheostomy. After thorough examination, urogenital dysfunction, orthostatic hypotension, and Parkinsonism were found, which led to the diagnosis of multiple system atrophy (MSA. After the tracheostomy, bi-level positive airway pressure ventilation was required during the night due to nocturnal hypoxemia. Nighttime hypoxemia is related to central sleep apnea, which is one of the manifestations of MSA. This is the first case of MSA manifested by bilateral vocal cord palsy as an initial sign in Korea. This case supports the notion that MSA should be taken into consideration when vocal cord paralysis is observed.

  14. Chronic Pain Following Spinal Cord Injury: The Role of Immunogenetics and Time of Injury Pain Treatment

    Science.gov (United States)

    2015-10-01

    central sensitization to nociceptive stimuli culminates in profound debilitating pain that serves no adaptive purpose for the sufferer. It is now...Award Number: W81XWH-11-1-0806 TITLE: Chronic Pain Following Spinal Cord Injury: The Role of Immunogenetics and Time of Injury Pain Treatment...29Sep2015 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER W81XWH-11-1-0806 Chronic Pain Following Spinal Cord Injury: The Role of Immunogenetics and Time

  15. 应用 iTRAQ 对唐氏综合征胎儿脐带血的蛋白质组学研究%Proteomics study of cord blood in Down′s syndrome fetuses by iTRAQ

    Institute of Scientific and Technical Information of China (English)

    锦丽; 林秀华; 郭辉; 李武县; 林琳华; 戴勇

    2015-01-01

    目的:筛选并鉴定唐氏综合征胎儿与健康对照者差异性表达蛋白质,以寻找唐氏综合征胎儿的生物标记物。方法应用同位素标记相对和绝对蛋白定量法和功能聚类分析法筛查和分析唐氏综合征胎儿脐带血和正常胎儿脐带血的差异表达蛋白。结果共筛选鉴定到506种差异表达蛋白,其中最显著的3种蛋白分别是基质蛋白‐3、胸腺肽β10和骨桥蛋白。结论唐氏综合征胎儿和正常胎儿脐带血血清蛋白存在较多差异表达蛋白,其中基质蛋白‐3、胸腺肽β10和骨桥蛋白等显著差异蛋白可能作为潜在的临床筛查生物标志物。%Objective To identify the differentially expressed proteins between fetuses with Down′s syndrome and healthy control ,so as to screen the potential biomarkers of fetuses with Down′s syndrome .Methods To investi‐gate the differentially expressed proteins in cord blood of fetus with Down′s syndrome and normal fetus using isobaric tagging for relative and absolute protein quantification and gene gene ontology analysis .Results A total of 506 dif‐ferentiate proteins were identified ,in which 3 proteins (Matrin‐3 ,Thymosin beta 10 and Osteopontin) were signifi‐cant .Conclusion The proteomics of the serum are significant different among normal fetus and fetus with Down′s syndrome .Some of the protein ,such as Matrin‐3 ,Thymosin beta 10 and Osteopontin ,may have potential applying value in clinic screening pratice .

  16. A report of high triglyceride level in cord blood of Iranian newborns

    Directory of Open Access Journals (Sweden)

    Seyed Ali Naghi Kazemi

    2013-01-01

    Conclusion: The mean and 95 th percentiles of triglyceride levels in cord blood of our newborn infants were higher than other reports. We recommend that larger studies should be conducted in this area to establish preventive ways for increasing epidemic of the metabolic syndrome.

  17. Biciliated ependymal cell proliferation contributes to spinal cord growth.

    Science.gov (United States)

    Alfaro-Cervello, Clara; Soriano-Navarro, Mario; Mirzadeh, Zaman; Alvarez-Buylla, Arturo; Garcia-Verdugo, Jose Manuel

    2012-10-15

    Two neurogenic regions have been described in the adult brain, the lateral ventricle subventricular zone and the dentate gyrus subgranular zone. It has been suggested that neural stem cells also line the central canal of the adult spinal cord. Using transmission and scanning electron microscopy and immunostaining, we describe here the organization and cell types of the central canal epithelium in adult mice. The identity of dividing cells was determined by 3D ultrastructural reconstructions of [(3) H]thymidine-labeled cells and confocal analysis of bromodeoxyuridine labeling. The most common cell type lining the central canal had two long motile (9+2) cilia and was vimentin+, CD24+, FoxJ1+, Sox2+, and CD133+, but nestin- and glial fibrillary acidic protein (GFAP)-. These biciliated ependymal cells of the central canal (Ecc) resembled E2 cells of the lateral ventricles, but their basal bodies were different from those of E2 or E1 cells. Interestingly, we frequently found Ecc cells with two nuclei and four cilia, suggesting they are formed by incomplete cytokinesis or cell fusion. GFAP+ astrocytes with a single cilium and an orthogonally oriented centriole were also observed. The majority of dividing cells corresponded to biciliated Ecc cells. Central canal proliferation was most common during the active period of spinal cord growth. Pairs of labeled Ecc cells were observed within the central canal in adult mice 2.5 weeks post labeling. Our work suggests that the vast majority of postnatal dividing cells in the central canal are Ecc cells and their proliferation is associated with the growth of the spinal cord.

  18. Median nerve conduction velocity and central conduction time measured with somatosensory evoked potentials in thyroxine-treated infants with Down syndrome

    NARCIS (Netherlands)

    van Trotsenburg, A. S. Paul; Smit, Bert J.; Koelman, Johannes H. T. M.; Sloot, Marijke Dekker-van der; Ridder, Jeannette C. D.; Tijssen, Jan G. P.; de Vijlder, Jan J. M.; Vulsma, Thomas

    2006-01-01

    OBJECTIVE. The aim of this study was to determine whether thyroxine treatment would improve nerve conduction in infants with Down syndrome. METHODS. A single-center, nationwide, randomized, double-blind, clinical trial was performed. Neonates with Down syndrome were assigned randomly to thyroxine (N

  19. Spinal cord involvement in a child with familial hemophagocytic lymphohistiocytosis

    Directory of Open Access Journals (Sweden)

    Muge Gokce

    2012-01-01

    Full Text Available The involvement of the central nervous system (CNS in familial hemophagocytic lymphohistiocytosis (FHL has known to be limited to the brain, brain stem, and cerebellum. Herein, we report an 11-year-old boy who presented with neurological symptoms and was diagnosed as FHL by molecular diagnosis. The hemophagocytic lesions in the CNS were shown to extend to the thoracal level of spinal cord which completely disappeared after the completion of hemophagocytic lymphohistiocytosis-2004 protocol.

  20. Immunotherapy strategies for spinal cord injury.

    Science.gov (United States)

    Wang, Yong-Tang; Lu, Xiu-Min; Chen, Kai-Ting; Shu, Ya-Hai; Qiu, Chun-Hong

    2015-01-01

    Regeneration in the central nervous system (CNS) of adult mammalian after traumatic injury is limited, which often causes permanent functional motor and sensory loss. After spinal cord injury (SCI), the lack of regeneration is mainly attributed to the presence of a hostile microenvironment, glial scarring, and cavitation. Besides, inflammation has also been proved to play a crucial role in secondary degeneration following SCI. The more prominent treatment strategies in experimental models focus mainly on drugs and cell therapies, however, only a few strategies applied in clinical studies and therapies still have only limited effects on the repair of SCI. Recently, the interests in immunotherapy strategies for CNS are increasing in number and breadth. Immunotherapy strategies have made good progresses in treating many CNS degenerative disorders, such as Alzheimer's disease (AD), Parkinson's disease (PD), stroke, and multiple sclerosis (MS). However, the strategies begin to be considered to the treatment of SCI and other neurological disorders in recent years. Besides anti-inflamatory therapy, immunization with protein vaccines and DNA vaccines has emerged as a novel therapy strategy because of the simplicity of preparation and application. An inflammatory response followed by spinal cord injury, and is controled by specific signaling molecules, such as some cytokines playing a crucial role. As a result, appropriate immunoregulation, the expression of pro-inflammatory cytokines and anti-inflammatory cytokines may be an effective therapy strategy for earlier injury of spinal cord. In addition, myelinassociated inhibitors (MAIs) in the injured spinal cord, such as Nogo, myelin-associated glycoprotein (MAG) and oligodendrocyte- myelin glycoprotein (OMgp) are known to prevent axonal regeneration through their co-receptors, and to trigger demyelinating autoimmunity through T cell-mediated harmful autoimmune response. The antagonism of the MAIs through vaccinating with

  1. Transplantation of olfactory ensheathing cells for promoting regeneration following spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Kaijun Liu

    2007-01-01

    OBJECTIVE: To investigate the status of olfactory ensheathing cells (OECs) transplantation in facilitating the regeneration of spinal cord injury.DATA SOURCES: Articles about OECs transplantation in treating spinal cord injury were searched in Pubmed database published in English from January 1981 to December 2005 by using the keywords of "olfactory ensheathing cells, transplantation, spinal cord injury".STUDY SELECTION: The data were checked primarily, literatures related to OECs transplantation and the regeneration of spinal cord injury were selected, whereas the repetitive studies and reviews were excluded.DATA EXTRACTION: Totally 43 articles about OECs transplantation and the regeneration and repair of spinal cord injury were collected, and the repetitive ones were excluded.DATA SYNTHESIS: There were 35 articles accorded with the criteria. OECs are the olfactory ensheathing glias isolated from olfactory bulb and olfactory nerve tissue. OECs have the characters of both Schwann cells in central nervous system and peripheral astrocytes. The transplanted OECs can migrate in the damaged spinal cord of host, can induce and support the regeneration, growth and extension of damaged neuritis.Besides, transgenic technique can enable it to carry some exogenous genes that promote neuronal regeneration, and express some molecules that can facilitate neural regeneration, so as to ameliorate the internal environment of nerve injury, induce the regeneration of damaged spinal cord neurons, which can stimulate the regeneration potential of the damaged spinal cord to reach the purpose of spinal cord regeneration and functional recovery.CONCLUSION: OECs are the glial cells with the energy for growth at mature phase, they can myelinize axons, secrete various biological nutrition factors, and then protect and support neurons, also facilitate neural regeneration. OECs have been successfully isolated from nasal olfactory mucosa and olfactory nerve.Therefore, autologous transplantation

  2. Bancos de cordón umbilical Umbilical cord banks

    OpenAIRE

    2009-01-01

    La utilización de sangre de cordón como fuente de precursores hematológicos se remonta a 1983 cuando Boyse apuntó el potencial en progenitores existente en la sangre de cordón, realizándose un año más tarde las primeras experiencias sobre modelos murinos. Tuvieron que pasar más de cinco años para que Gluckman realizara la primera experiencia en humanos. Un niño afecto de anemia de Fanconi fue trasplantado con progenitores de sangre de cordón umbilical de su hermana HLA idéntica, realizándose ...

  3. Glial implications in transplantation therapy of spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    CHEN Shi-wen; XIE Yu-feng

    2009-01-01

    Spinal cord injuries are damages that result in complete or partial loss of sensation and/or mobility and affect the life qualities of many patients. Their pathophysiology in-cludes primary and secondary processes, which are related with the activation of astrocytes and microgliacytes and the degeneration of oligodendrocytes. Although transplan-tation of embryonic stem cells or neural progenitor cells is an attractive strategy for repair of the injured central ner-vous system (CNS), transplantation of these cells alone for acute spinal cord injuries has not resulted in robust axon regeneration beyond the injury sites. This may be due to the progenitor cells differentiating to the cell types that sup-port axon growth poorly and/or their inability to modify the inhibitory environment of adult CNS after injury. Recent studies indicate that transplantation of glial progenitor cells has exhibited beneficial effects on the recovery and promis-ing future for the therapy strategy of spinal cord injury. In this review, we summarized the data from recent literature regarding glial implications in transplantation therapy of spinal cord injury.

  4. Spinal Cord Injury Model System Information Network

    Science.gov (United States)

    ... Go New to Website Managing Bowel Function After Spinal Cord Injury Resilience, Depression and Bouncing Back after SCI Getting ... the UAB-SCIMS Contact the UAB-SCIMS UAB Spinal Cord Injury Model System Newly Injured Health Daily Living Consumer ...

  5. Suicide in a spinal cord injured population

    DEFF Research Database (Denmark)

    Hartkopp, A; Brønnum-Hansen, Henrik; Seidenschnur, A M;

    1998-01-01

    To determine the relation between functional status and risk of suicide among individuals with spinal cord injury (SCI).......To determine the relation between functional status and risk of suicide among individuals with spinal cord injury (SCI)....

  6. 脐带间充质干细胞移植对不同证型系统性红斑狼疮患者的效果分析%Therapeutic effect of umbilical cord-derived mesenchymal stem cell transplantation in systemic lupus erythematosus patients with different patterns of syndromes

    Institute of Scientific and Technical Information of China (English)

    汤郁; 刘瑞霞; 裘影影; 芮金兵; 李晶

    2015-01-01

    BACKGROUND:Systemic lupus erythematosus (SLE) is classified into four types, and the major treatment is to tonify kidney and nourishyin, clear blood stasis and toxin by the traditional Chinese medicine (TCM). Even though, there are stil many patients with poor efficacy. Mesenchymal stem cels have the capacity of multiple differentiation, hematopoietic support and immune regulation, thus having been used for the treatment of refractory, recurrent SLE and achieving good effects. OBJECTIVE:To investigate the therapeutic effect of umbilical cord-derived mesenchymal stem cel transplantation on SLE patients with different patterns of syndromes. METHODS: Twenty-one SLE patients were clustered to four syndrome types of TCM, including heat-toxin,yin deficiency of liver and kidney,yang deficiency of spleen and kidney, andqi stagnation and blood stasis. The changes in clinical and laboratory indicators were analyzed statisticaly before and after cel transplantation. RESULTS AND CONCLUSION:The level of 24-hour proteinuria and SLE disease activity index scores in SLE patients were significantly decreased at 1, 3, 6 months after cel transplantation (P < 0.01). Umbilical cord-derived mesenchymal stem cel transplantation could significantly reduce the 24-hour proteinuria in SLE patients withyin deficiency of liver and kidney at 1, 3 and 6 months (P < 0.01), while slightly reduce the 24-hour proteinuria in SLE patients with heat-toxin andqi stagnation and blood stasis at 1, 3 months (P < 0.05) as wel as in SLE patients withyang deficiency of spleen and kidney at 1 month (P < 0.05). Additionaly, umbilical cord-derived mesenchymal stem cel transplantation could increase the serum albumin levels in al the SLE patients (P < 0.01), although the changes in patients with heat-toxin were moderate (P < 0.05). Al the SLE patients of four types had an increasing trend of their platelet counting after cel transplantation, but there was no statistical difference before and after cel

  7. Dexmedetomidine for an awake fiber-optic intubation of a parturient with Klippel-Feil syndrome, Type I Arnold Chiari malformation and status post released tethered spinal cord presenting for repeat cesarean section

    Directory of Open Access Journals (Sweden)

    Tanmay H. Shah

    2011-08-01

    Full Text Available Patients with Klippel-Feil Syndrome (KFS have congenital fusion of their cervical vertebrae due to a failure in the normal segmentation of the cervical vertebrae during the early weeks of gestation and also have myriad of other associated anomalies. Because of limited neck mobility, airway management in these patients can be a challenge for the anesthesiologist. We describe a unique case in which a dexmedetomidine infusion was used as sedation for an awake fiber-optic intubation in a parturient with Klippel-Feil Syndrome, who presented for elective cesarean delivery. A 36-yearold female, G2P1A0 with KFS (fusion of cervical vertebrae who had prior cesarean section for breech presentation with difficult airway management was scheduled for repeat cesarean delivery. After obtaining an informed consent, patient was taken in the operating room and non-invasive monitors were applied. Dexmedetomidine infusion was started and after adequate sedation, an awake fiberoptic intubation was performed. General anesthetic was administered after intubation and dexmedetomidine infusion was continued on maintenance dose until extubation. Klippel-Feil Syndrome (KFS is a rare congenital disorder for which the true incidence is unknown, which makes it even rare to see a parturient with this disease. Patients with KFS usually have other congenital abnormalities as well, sometimes including the whole thoraco-lumbar spine (Type III precluding the use of neuraxial anesthesia for these patients. Obstetric patients with KFS can present unique challenges in administering anesthesia and analgesia, primarily as it relates to the airway and dexmedetomidine infusion has shown promising result to manage the airway through awake fiberoptic intubation without any adverse effects on mother and fetus.

  8. Timing of Surgery After Spinal Cord Injury.

    Science.gov (United States)

    Piazza, Matthew; Schuster, James

    2017-01-01

    Although timing for surgical intervention after spinal cord injury remains controversial, there is accumulating evidence suggesting that early surgery may improve neurologic outcomes, particularly with incomplete spinal cord injury, and may reduce non-neurologic complications and health care resource utilization. Moreover, even in patients with complete spinal cord injury, minor improvement in neurologic function can lead to significant changes in quality of life. This article reviews the experimental and clinical data examining surgical timing after spinal cord injury.

  9. The role of propriospinal interneurons in recovery from spinal cord injury.

    Science.gov (United States)

    Flynn, Jamie R; Graham, Brett A; Galea, Mary P; Callister, Robert J

    2011-04-01

    Over one hundred years ago, Sir Charles Sherrington described a population of spinal cord interneurons (INs) that connect multiple spinal cord segments and participate in complex or 'long' motor reflexes. These neurons were subsequently termed propriospinal neurons (PNs) and are known to play a crucial role in motor control and sensory processing. Recent work has shown that PNs may also be an important substrate for recovery from spinal cord injury (SCI) as they contribute to plastic reorganisation of spinal circuits. The location, inter-segmental projection pattern and sheer number of PNs mean that after SCI, a significant number of them are capable of 'bridging' an incomplete spinal cord lesion. When these properties are combined with the capacity of PNs to activate and coordinate locomotor central pattern generators (CPGs), it is clear they are ideally placed to assist locomotor recovery. Here we summarise the anatomy, organisation and function of PNs in the uninjured spinal cord, briefly outline the pathophysiology of SCI, describe how PNs contribute to recovery of motor function, and finally, we discuss the mechanisms that underlie PN plasticity. We propose there are two major challenges for PN research. The first is to learn more about ways we can promote PN plasticity and manipulate the 'hostile' micro-environment that limits regeneration in the damaged spinal cord. The second is to study the cellular/intrinsic properties of PNs to better understand their function in both the normal and injured spinal cord. This article is part of a Special Issue entitled 'Synaptic Plasticity & Interneurons'.

  10. [MR investigation of spinal cord herniation in the thoracic spine].

    Science.gov (United States)

    Kenéz, József; Barsi, Péter; Várallyay, György; Bobest, Mátyás; Veres, Róbert

    2002-05-20

    Transdural herniation of the spinal cord is thought to be previously extremely rare and very often misdiagnosed. Possible reasons may be iatrogenic and traumatic or in about one third of cases it may be unknown, where the probable origin might be a congenital dural defect. The pathology may show characteristic and misleading MR patterns of the thoracic spine, emphasising the importance of these patterns. This anomaly can lead to progressive Brown-Sequard syndrome. Surgical intervention, consisting the repair of the dural defect may result in improvement or even complete regression of the neurologic deficits.

  11. MRI in Lyme disease of the spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Mantienne, C.; Catalaa, I.; Sevely, A.; Cognard, C.; Manelfe, C. [Dept. of Diagnostic and Therapeutic Neuroradiology, Hopital Purpan, Toulouse (France); Albucher, J.F. [Dept. of Neurology, Hopital Purpan, Toulouse (France)

    2001-06-01

    We report a case of Lyme myelitis in a 31-year-old man, presenting with a conus medullaris syndrome. MRI demonstrated contrast enhancement on the pial surface of the lower thoracic cord and conus medullaris. Elevated blood immunoglobulins and IgM antibodies against Borrelia burgdorferi in the cerebrospinal fluid (CSF) were found. Leptomeningitis may be the first stage of spinal infection in Lyme disease, preceding parenchymal infection leading to myelitis. Vasculitis is probably the major mechanism. MRI findings are nonspecific and the diagnosis is given by serum and CSF analyses. Early treatment with antibiotics and high doses steroids may result in complete recovery, as in this case. (orig.)

  12. Split cervical spinal cord malformation and vertebral dysgenesis.

    Science.gov (United States)

    Andro, C; Pecquery, R; De Vries, P; Forlodou, P; Fenoll, B

    2009-11-01

    We report a case of vertebral malformation associated with diplomyelia believed to be a type II split cord malformation. Cervicothoracic level cases are exceptional. This article reports the case of an 11-year-old boy with no neurological symptoms who had not undergone surgery. The diagnosis was made during pregnancy by prenatal screening with ultrasound and MRI. Several embryological theories have been offered to provide an explanation for this syndrome. Close follow-up is mandatory. Surgery must only be considered if neurological deterioration occurs.

  13. Augmentation of Voluntary Locomotor Activity by Transcutaneous Spinal Cord Stimulation in Motor-Incomplete Spinal Cord-Injured Individuals.

    Science.gov (United States)

    Hofstoetter, Ursula S; Krenn, Matthias; Danner, Simon M; Hofer, Christian; Kern, Helmut; McKay, William B; Mayr, Winfried; Minassian, Karen

    2015-10-01

    The level of sustainable excitability within lumbar spinal cord circuitries is one of the factors determining the functional outcome of locomotor therapy after motor-incomplete spinal cord injury. Here, we present initial data using noninvasive transcutaneous lumbar spinal cord stimulation (tSCS) to modulate this central state of excitability during voluntary treadmill stepping in three motor-incomplete spinal cord-injured individuals. Stimulation was applied at 30 Hz with an intensity that generated tingling sensations in the lower limb dermatomes, yet without producing muscle reflex activity. This stimulation changed muscle activation, gait kinematics, and the amount of manual assistance required from the therapists to maintain stepping with some interindividual differences. The effect on motor outputs during treadmill-stepping was essentially augmentative and step-phase dependent despite the invariant tonic stimulation. The most consistent modification was found in the gait kinematics, with the hip flexion during swing increased by 11.3° ± 5.6° across all subjects. This preliminary work suggests that tSCS provides for a background increase in activation of the lumbar spinal locomotor circuitry that has partially lost its descending drive. Voluntary inputs and step-related feedback build upon the stimulation-induced increased state of excitability in the generation of locomotor activity. Thus, tSCS essentially works as an electrical neuroprosthesis augmenting remaining motor control.

  14. Evaluation of spinal cord injury animal models

    Institute of Scientific and Technical Information of China (English)

    Ning Zhang; Marong Fang; Haohao Chen; Fangming Gou; Mingxing Ding

    2014-01-01

    Because there is no curative treatment for spinal cord injury, establishing an ideal animal model is important to identify injury mechanisms and develop therapies for individuals suffering from spinal cord injuries. In this article, we systematically review and analyze various kinds of animal models of spinal cord injury and assess their advantages and disadvantages for further studies.

  15. Psychological Aspects of Spinal Cord Injury

    Science.gov (United States)

    Cook, Daniel W.

    1976-01-01

    Reviewing literature on the psychological impact of spinal cord injury suggests: (a) depression may not be a precondition for injury adjustment; (b) many persons sustaining cord injury may have experienced psychological disruption prior to injury; and (c) indexes of rehabilitation success need to be developed for the spinal cord injured. (Author)

  16. Spinal Cord Ventral Horns and Lymphoid Organ Involvement in Powassan Virus Infection in a Mouse Model

    Directory of Open Access Journals (Sweden)

    Rodrigo I. Santos

    2016-08-01

    Full Text Available Powassan virus (POWV belongs to the family Flaviviridae and is a member of the tick-borne encephalitis serogroup. Transmission of POWV from infected ticks to humans has been documented in the USA, Canada, and Russia, causing fatal encephalitis in 10% of human cases and significant neurological sequelae in survivors. We used C57BL/6 mice to investigate POWV infection and pathogenesis. After footpad inoculation, infected animals exhibited rapid disease progression and 100% mortality. Immunohistochemistry and immunofluorescence revealed a very strong neuronal tropism of POWV infection. The central nervous system infection appeared as a meningoencephalitis with perivascular mononuclear infiltration and microglial activation in the brain, and a poliomyelitis-like syndrome with high level of POWV antigen at the ventral horn of the spinal cord. Pathological studies also revealed substantial infection of splenic macrophages by POWV, which suggests that the spleen plays a more important role in pathogenesis than previously realized. This report provides a detailed description of the neuroanatomical distribution of the lesions produced by POWV infection in C57BL/6 mice.

  17. Cryptic organisation within an apparently irregular rostrocaudal distribution of interneurons in the embryonic zebrafish spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Wells, Simon, E-mail: simon.wells@adelaide.edu.au [Discipline of Genetics, School of Molecular and Biomedical Sciences, University of Adelaide, Adelaide, South Australia 5005 (Australia); The Special Research Centre for the Molecular Genetics of Development, University of Adelaide, Adelaide, South Australia 5005 (Australia); Conran, John G., E-mail: john.conran@adelaide.edu.au [Ecology and Evolutionary Biology, School of Earth and Environmental Sciences, University of Adelaide, Adelaide, South Australia 5005 (Australia); Tamme, Richard, E-mail: rtamme@ttu.ee [Discipline of Genetics, School of Molecular and Biomedical Sciences, University of Adelaide, Adelaide, South Australia 5005 (Australia); Gaudin, Arnaud, E-mail: a.gaudin@uq.edu.au [School of Biomedical Sciences, University of Queensland, Brisbane, Queensland 4072 (Australia); Webb, Jonathan, E-mail: jonathan.webb@worc.ox.ac.uk [Discipline of Genetics, School of Molecular and Biomedical Sciences, University of Adelaide, Adelaide, South Australia 5005 (Australia); Lardelli, Michael, E-mail: michael.lardelli@adelaide.edu.au [Discipline of Genetics, School of Molecular and Biomedical Sciences, University of Adelaide, Adelaide, South Australia 5005 (Australia); The Special Research Centre for the Molecular Genetics of Development, University of Adelaide, Adelaide, South Australia 5005 (Australia)

    2010-11-15

    The molecules and mechanisms involved in patterning the dorsoventral axis of the developing vertebrate spinal cord have been investigated extensively and many are well known. Conversely, knowledge of mechanisms patterning cellular distributions along the rostrocaudal axis is relatively more restricted. Much is known about the rostrocaudal distribution of motoneurons and spinal cord cells derived from neural crest but there is little known about the rostrocaudal patterning of most of the other spinal cord neurons. Here we report data from our analyses of the distribution of dorsal longitudinal ascending (DoLA) interneurons in the developing zebrafish spinal cord. We show that, although apparently distributed irregularly, these cells have cryptic organisation. We present a novel cell-labelling technique that reveals that DoLA interneurons migrate rostrally along the dorsal longitudinal fasciculus of the spinal cord during development. This cell-labelling strategy may be useful for in vivo analysis of factors controlling neuron migration in the central nervous system. Additionally, we show that DoLA interneurons persist in the developing spinal cord for longer than previously reported. These findings illustrate the need to investigate factors and mechanisms that determine 'irregular' patterns of cell distribution, particularly in the central nervous system but also in other tissues of developing embryos.

  18. The stress ulcer syndrome

    NARCIS (Netherlands)

    H.A. van Essen

    1986-01-01

    textabstractThe stress ulcer syndrome is described in this thesis. This syndrome is seen in patients admitted to intensive care departments or being treated in field hospitals, in disaster areas, or battle fields. Acute mucosal lesions associated with burns (Curling's ulcers) and central nervous sys

  19. Comparison of standard, prone and cine MRI in the evaluation of tethered cord

    Energy Technology Data Exchange (ETDEWEB)

    Singh, Sukhjinder [Cohen Children' s Medical Center, Department of Radiology, New Hyde Park, NY (United States); Kline-Fath, Beth; Racadio, Judy M. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Bierbrauer, Karin [Cincinnati Children' s Hospital Medical Center, Department of Neurosurgery, Cincinnati, OH (United States); Salisbury, Shelia; Macaluso, Maurizio [Cincinnati Children' s Hospital Medical Center, Division of Biostatistics and Epidemiology, Cincinnati, OH (United States); Jackson, Elizabeth C. [Cincinnati Children' s Hospital Medical Center, Department of Pediatrics, Division of Nephrology, Cincinnati, OH (United States); Egelhoff, John C. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Phoenix Children' s Hospital, Department of Radiology, Phoenix, AZ (United States)

    2012-06-15

    Tethered cord syndrome (TCS) is defined by abnormal traction on the spinal cord that confines its movement. Surgical cord release usually stops neurological deterioration; therefore, early and accurate neuroradiological diagnosis is important. Supine MRI is the imaging modality of choice, but prone MRI and cine MRI can demonstrate cord movement. We compared the diagnostic accuracies of standard MRI, prone MRI and cine MRI in patients with clinical suspicion of TCS and evaluated inter-reader reliability for MR imaging. Children who underwent MRI for suspicion of TCS were retrospectively identified. Supine, prone and cine MRI studies were re-read by two pediatric neuroradiologists. Conus level, filum appearance and cord movement were documented. Thirteen of 49 children had tethered cord documented at surgery. Conus level had the highest diagnostic accuracy (sensitivity 69-77%, specificity 94%, positive predictive value 82-83%, negative predictive value 89-92%, correct diagnosis 88-90%) and highest between-reader concordance (98%). Prone and cine MRI did not add to the accuracy of the supine imaging. Conus level provides the highest diagnostic accuracy and inter-reader reliability in TCS. Until a larger series is evaluated, it remains questionable whether prone or cine MRI provides enough additional diagnostic information to warrant routine use. (orig.)

  20. Cysticercosis of the nervous system: less frequent clinical forms III- spinal cord forms

    Directory of Open Access Journals (Sweden)

    Horacio M. Canelas

    1963-06-01

    Full Text Available The spinal forms of cysticercosis are rather rare (2.7% of 296 cases of neurocysticercosis recorded in the Department of Neurology of the University of São Paulo Medical School. In a survey of the literature only 42 cases were found, most of them associated with cerebral symptoms. The reasons for this low incidence, as well as the possible routes followed by the parasite in its approach to the spinal cord, are discussed. After a review of the first cases reported in the literature, the authors refer the main syndromes (meningomyelitides, tabetiform pictures and spinal cord compressions and some of the clinico-pathologic features of spinal cysticercosis. Nine cases of spinal cysticercosis are reported. The diagnosis was based on laboratorial data (mainly the complement fixation test for cysticercosis in the cerebrospinal fluid or in the results of surgical therapy. Other cerebrospinal fluid findings (presence of eosinophile cells, protein contents, and the results of the manometric tests are discussed. Myelographic block was demonstrated in 5 cases. Three of these patients were submitted to laminectomy, with variable results. The prevailing neurological picture was that of spinal cord and/or root compression (4 cases. Two patients showed a dorsal funiculi syndrome closely simulating tabes dorsalis. Two other patients presented a picture of meningomyelitis with no systematization. One patient had a syndrome suggestive of subacute combined degeneration of the spinal cord, but the presence of cerebral symptoms and the laboratorial data pointed to cysti-cercosis as the main disease process.

  1. Reelin inhibits migration of sympathetic preganglionic neurons in the spinal cord of the chick.

    Science.gov (United States)

    Yip, Yee Ping; Zhou, Guangdou; Kubo, Ken-Ichiro; Nakajima, Kazunori; Yip, Joseph W

    2011-07-01

    The present study examined the effects of Reelin in the migration of sympathetic preganglionic neurons (SPN) in the spinal cord of the chick. SPN in the chick first migrate from the neuroepithelium to the ventrolateral spinal cord. They then undergo a secondary migration to cluster adjacent to the central canal, forming the column of Terni (CT). During secondary migration, abundant Reelin is found in large areas of the ventral spinal cord; the only areas devoid of Reelin are areas occupied by SPN or somatic motor neurons and the pathway along which SPN migrate. Ectopic expression of Reelin in the pathway of SPN through electroporation of full-length Reelin DNA stopped SPN migration toward their destination. The spatiotemporal pattern of Reelin expression, along with the inhibition of SPN migration by exogenous Reelin, suggests that Reelin functions as a barrier to SPN migration during normal development of the spinal cord.

  2. Vitamin B(12) dependent changes in mouse spinal cord expression of vitamin B(12) related proteins and the epidermal growth factor system

    DEFF Research Database (Denmark)

    Mutti, Elena; Lildballe, Dorte L; Kristensen, Lise

    2013-01-01

    Chronic vitamin B(12) (cobalamin) deficiency in the mammalian central nervous system causes degenerative damage, especially in the spinal cord. Previous studies have shown that cobalamin status alters spinal cord expression of epidermal growth factor (EGF) and its receptor in rats. Employing a mo...

  3. Spinal cord projections to the cerebellum in the mouse.

    Science.gov (United States)

    Sengul, Gulgun; Fu, YuHong; Yu, You; Paxinos, George

    2015-09-01

    The projections from the spinal cord to the cerebellar cortex were studied using retrograde neuronal tracers. Thus far, no study has shown the detailed topographic mapping of the projections from the spinal neuron clusters to the cerebellar cortex regions for experimental animals, and there are no studies for the mouse. Tracers Fluoro-Gold and cholera toxin B were injected into circumscribed regions of the cerebellar cortex, and retrogradely labeled spinal cord neurons were mapped throughout the spinal cord. Spinal projections to the cerebellar cortex were mainly from five neuronal columns--central cervical nucleus, dorsal nucleus, lumbar and sacral precerebellar nuclei, and lumbar border precerebellar cells--and from scattered neurons located in the deep dorsal horn and laminae 6-8. The spinocerebellar projections to the cortex were mainly to the vermis. All five precerebellar cell columns projected to both anterior and posterior parts of the cerebellar cortex. Results of this study provide an amendment to the known rostral and caudal boundaries of the precerebellar cell columns in the mouse. Scattered precerebellar neurons in the most caudal deep dorsal horn and laminae 6-8 projected exclusively to the anterior part of the cerebellar cortex. In this study, no labeled spinal neurons were found to project to the lobules 6 and 7 of the cerebellar vermis, the flocculus, and the paraflocculus. Spinocerebellar neurons were located bilaterally, but the majority of the projections were contralateral for the central cervical nucleus, and ipsilateral for the remaining spinal precerebellar neuronal clusters.

  4. MRI of central nervous system anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Izawa, M.; Oikawa, A.; Matoba, A.

    1987-05-01

    MRI was very useful in the evaluation of congenital anomalies of central nervous system as well as other nervous system disease with three-dimensional spatial resolution. We had experienced MRI of central nervous system anomalies, demonstrated characterisitic findings in each anomaly. MRI is useful to observe the coronal, horizontal and sagittal images of the brain and spinal cord in order to discuss the etiological mechanisms of spinal dysraphysm and its associated anomalies. In case of spina bifida cystica MRI was available to decide operative indication for radical operation and tetherd cord developed from postoperative scar or accompanied intraspinal lesions.

  5. TRUNCATION ARTIFACT IN MAGNETIC RESONANCE IMAGES OF THE CANINE SPINAL CORD.

    Science.gov (United States)

    Gregori, Tommaso; Lam, Richard; Priestnall, Simon L; Lamb, Christopher R

    2016-11-01

    The truncation artifact in magnetic resonance (MR) images is a line of abnormal signal intensity that occurs parallel to an interface between tissues of markedly different signal intensity. In order to demonstrate the truncation artifact in sagittal images of the canine spinal cord and the effect of changing spatial resolution, we conducted an experimental in vitro study. A section of fixed canine spinal cord was imaged using a 1.5T magnet. Spatial resolution was increased by increasing the acquisition matrix and reconstruction matrix, producing series of T2-weighted (T2w) images with the following pixel sizes: A, 1.6 (vertical) × 2.2 mm(2) (horizontal); B, 1.2 × 1.7 mm(2) ; C, 0.8 × 1.1 mm(2) ; D, 0.4 × 0. 6 mm(2) . Plots of mean pixel value across the cord showed variations in signal intensity compatible with truncation artifact, which appeared as a single, wide central hyperintense zone in low-resolution images and as multiple narrower zones in high spatial resolution images. Even in images obtained using the highest spatial resolution available for the MR system, the edge of the spinal cord was not accurately defined and the central canal was not visible. The experiment was repeated using an unfixed spinal cord specimen with focal compression applied to mimic a pathologic lesion. Slight hyperintensity was observed within the spinal cord at the site of compression although the cord was normal histologically. Results of this study suggest that caution should be applied when interpreting hyperintensity affecting the spinal cord in T2w sagittal images of clinical patients because of the possibility that the abnormal signal could represent a truncation artifact.

  6. Matrix metalloproteinase production in regenerating axolotl spinal cord.

    Science.gov (United States)

    Chernoff, E A; O'Hara, C M; Bauerle, D; Bowling, M

    2000-01-01

    In urodele amphibian spinal cord regeneration, the ependymal cells lining the central canal remodel the lesion site to favor axonal regrowth. We profiled the production of matrix metalloproteinases by injury-reactive mesenchymal ependymal cells in vivo and in vitro and found that matrix metalloproteinases are involved in this remodeling process in the axolotl (Ambystoma mexicanum). The production of cell-associated matrix metalloproteinases in vivo was shown to be identical to that in our cultured ependymal cell model system. Activated and zymogen forms of matrix metalloproteinases were identified using zymography, chemical inhibitors of matrix metalloproteinases, and cleavage of propeptides by organomercurials. The principal cellular proteinases consisted of matrix metalloproteinase-2 (gelatinase A) and matrix metalloproteinase-1 (type I collagenase), which display characteristic shifts in molecular weight following proenzyme processing by organomercurials. In addition, ependymal cell conditioned medium contained secreted forms of the enzyme undetectable in situ. Matrix metalloproteinase-9 (gelatinase B) as well as matrix metalloproteinase-2 and matrix metalloproteinase-1 were secreted and casein substrate zymography showed the presence of a small amount of a very high molecular weight matrix metalloproteinase-3 (prostromelysin) secreted into the culture medium. Matrix metalloproteinases were still present at 4 weeks post-lesioning when the ependymal cells have just re-epithelialized, but decreased near the completion of regeneration (8 weeks post-lesioning). Zymography showed no detectable matrix metalloproteinases in unlesioned cord but the presence of tissue inhibitor of metalloproteinase-1 in intact cord was seen by Western blotting. This study shows that matrix metalloproteinases are associated with urodele spinal cord regeneration and validates the use of our ependymal cell tissue culture model system to evaluate ependymal cell behavior during spinal cord

  7. Spinal cord injury at birth

    DEFF Research Database (Denmark)

    Fenger-Gron, Jesper; Kock, Kirsten; Nielsen, Rasmus G;

    2008-01-01

    UNLABELLED: A case of perinatally acquired spinal cord injury (SCI) is presented. The foetus was vigorous until birth, the breech presented and delivery was performed by a non-traumatic Caesarean section. The infant displayed symptoms of severe SCI but diagnosis was delayed due to severe co...

  8. Pain and spinal cord imaging measures in children with demyelinating disease

    Directory of Open Access Journals (Sweden)

    Nadia Barakat

    2015-01-01

    Full Text Available Pain is a significant problem in diseases affecting the spinal cord, including demyelinating disease. To date, studies have examined the reliability of clinical measures for assessing and classifying the severity of spinal cord injury (SCI and also to evaluate SCI-related pain. Most of this research has focused on adult populations and patients with traumatic injuries. Little research exists regarding pediatric spinal cord demyelinating disease. One reason for this is the lack of reliable and useful approaches to measuring spinal cord changes since currently used diagnostic imaging has limited specificity for quantitative measures of demyelination. No single imaging technique demonstrates sufficiently high sensitivity or specificity to myelin, and strong correlation with clinical measures. However, recent advances in diffusion tensor imaging (DTI and magnetization transfer imaging (MTI measures are considered promising in providing increasingly useful and specific information on spinal cord damage. Findings from these quantitative imaging modalities correlate with the extent of demyelination and remyelination. These techniques may be of potential use for defining the evolution of the disease state, how it may affect specific spinal cord pathways, and contribute to the management of pediatric demyelination syndromes. Since pain is a major presenting symptom in patients with transverse myelitis, the disease is an ideal model to evaluate imaging methods to define these regional changes within the spinal cord. In this review we summarize (1 pediatric demyelinating conditions affecting the spinal cord; (2 their distinguishing features; and (3 current diagnostic and classification methods with particular focus on pain pathways. We also focus on concepts that are essential in developing strategies for the detection, monitoring, treatment and repair of pediatric myelitis.

  9. Shedding light on restoring respiratory function after spinal cord injury

    Directory of Open Access Journals (Sweden)

    Warren J Alilain

    2009-10-01

    Full Text Available Loss of respiratory function is one of the leading causes of death following spinal cord injury. Because of this, much work has been done in studying ways to restore respiratory function following SCI - including pharmacological and regeneration strategies. With the emergence of new and powerful tools from molecular neuroscience, new therapeutically relevant alternatives to these approaches have become available, including expression of light sensitive proteins called channelrhodopsins. In this article we briefly review the history of various attempts to restore breathing after C2 hemisection, and focus on our recent work using the activation of light sensitive channels to restore respiratory function after experimental spinal cord injury. We also discuss how such light induced activity can help shed light on the inner workings of the central nervous system respiratory circuitry that controls diaphragmatic function.

  10. Macrophage and microglial plasticity in the injured spinal cord.

    Science.gov (United States)

    David, S; Greenhalgh, A D; Kroner, A

    2015-10-29

    Macrophages in the injured spinal cord arise from resident microglia and from infiltrating peripheral myeloid cells. Microglia respond within minutes after central nervous system (CNS) injury and along with other CNS cells signal the influx of their peripheral counterpart. Although some of the functions they carry out are similar, they appear to be specialized to perform particular roles after CNS injury. Microglia and macrophages are very plastic cells that can change their phenotype drastically in response to in vitro and in vivo conditions. They can change from pro-inflammatory, cytotoxic cells to anti-inflammatory, pro-repair phenotypes. The microenvironment of the injured CNS importantly influences macrophage plasticity. This review discusses the phagocytosis and cytokine-mediated effects on macrophage plasticity in the context of spinal cord injury.

  11. Central pattern generator for locomotion: anatomical, physiological, and pathophysiological considerations.

    Science.gov (United States)

    Guertin, Pierre A

    2012-01-01

    This article provides a perspective on major innovations over the past century in research on the spinal cord and, specifically, on specialized spinal circuits involved in the control of rhythmic locomotor pattern generation and modulation. Pioneers such as Charles Sherrington and Thomas Graham Brown have conducted experiments in the early twentieth century that changed our views of the neural control of locomotion. Their seminal work supported subsequently by several decades of evidence has led to the conclusion that walking, flying, and swimming are largely controlled by a network of spinal neurons generally referred to as the central pattern generator (CPG) for locomotion. It has been subsequently demonstrated across all vertebrate species examined, from lampreys to humans, that this CPG is capable, under some conditions, to self-produce, even in absence of descending or peripheral inputs, basic rhythmic, and coordinated locomotor movements. Recent evidence suggests, in turn, that plasticity changes of some CPG elements may contribute to the development of specific pathophysiological conditions associated with impaired locomotion or spontaneous locomotor-like movements. This article constitutes a comprehensive review summarizing key findings on the CPG as well as on its potential role in Restless Leg Syndrome, Periodic Leg Movement, and Alternating Leg Muscle Activation. Special attention will be paid to the role of the CPG in a recently identified, and uniquely different neurological disorder, called the Uner Tan Syndrome.

  12. Central Pattern Generator for Locomotion: Anatomical, Physiological and Pathophysiological Considerations

    Directory of Open Access Journals (Sweden)

    Pierre A. Guertin

    2013-02-01

    Full Text Available This article provides a perspective on major innovations over the past century in research on the spinal cord and, specifically, on specialized spinal circuits involved in the control of rhythmic locomotor pattern generation and modulation. Pioneers such as Charles Sherrington and Thomas Graham Brown have conducted experiments in the early twentieth century that changed our views of the neural control of locomotion. Their seminal work supported subsequently by several decades of evidence has led to the conclusion that walking, flying and swimming are largely controlled by a network of spinal neurons generally referred to as the central pattern generator (CPG for locomotion. It has been subsequently demonstrated across all vertebrate species examined, from lampreys to humans, that this CPG is capable, under some conditions, to self-produce, even in absence of descending or peripheral inputs, basic rhythmic and coordinated locomotor movements. Recent evidence suggests, in turn, that plasticity changes of some CPG elements may contribute to the development of specific pathophysiological conditions associated with impaired locomotion or spontaneous locomotor-like movements. This article constitutes a comprehensive review summarizing key findings on the CPG as well as on its potential role in Restless Leg Syndrome (RLS, Periodic Leg Movement (PLM, and Alternating Leg Muscle Activation (ALMA. Special attention will be paid to the role of the CPG in a recently identified, and uniquely different neurological disorder, called the Uner Tan Syndrome.

  13. Dumping Syndrome

    Science.gov (United States)

    ... System & How it Works Digestive Diseases A-Z Dumping Syndrome What is dumping syndrome? Dumping syndrome occurs when food, especially sugar, ... the colon and rectum—and anus. What causes dumping syndrome? Dumping syndrome is caused by problems with ...

  14. Neuroimaging features of Cornelia de Lange syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Whitehead, Matthew T. [Department of Radiology, Washington, DC (United States); Nagaraj, Usha D. [Department of Radiology, Washington, DC (United States); Cincinnati Children' s Hospital, Department of Radiology, Cincinnati, OH (United States); Pearl, Phillip L. [Department of Radiology, Washington, DC (United States); Boston Children' s Hospital, Department of Neurology, Boston, MA (United States)

    2015-08-15

    Cornelia de Lange syndrome is a rare genetic disease characterized by distinctive facial dysmorphia and dwarfism. Multiple organ system involvement is typical. Various central nervous system (CNS) aberrations have been described in the pathology literature; however, the spectrum of neuroimaging manifestations is less well documented. To present neuroimaging findings from a series of eight patients with Cornelia de Lange syndrome. The CT/MR database at a single academic children's hospital was searched for the terms ''Cornelia'', ''Brachmann'' and ''de Lange.'' The search yielded 18 exams from 16 patients. Two non-CNS and six exams without available images were excluded. Ten exams from eight patients were evaluated by a board-certified neuroradiologist. All patients had skull base dysplasia, most with an unusual coronal basioccipital cleft (7/8). All brain MR exams showed microcephaly, volume loss and gyral simplification (5/5). Six patients had an absent massa intermedia. Four patients had small globe anterior segments; three had optic pathway hypoplasia. Basilar artery fenestration was present in two patients; vertebrobasilar hypoplasia was present in one patient. The inner ear vestibules were dysplastic in two patients. One patient had pachymeningeal thickening. Spinal anomalies included scoliosis, segmentation anomalies, endplate irregularities, basilar invagination, foramen magnum stenosis and tethered spinal cord. Typical imaging manifestations of Cornelia de Lange syndrome include skull base dysplasia with coronal clival cleft, cerebral and brainstem volume loss, and gyral simplification. Membranous labyrinth dysplasia, anterior segment and optic pathway hypoplasia, basilar artery fenestration, absent massa intermedia and spinal anomalies may also be present. (orig.)

  15. Adult spinal cord ependymal layer: a promising pool of quiescent stem cells to treat spinal cord injury.

    Science.gov (United States)

    Panayiotou, Elena; Malas, Stavros

    2013-11-28

    Spinal cord injury (SCI) is a major health burden and currently there is no effective medical intervention. Research performed over the last decade revealed that cells surrounding the central canal of the adult spinal cord and forming the ependymal layer acquire stem cell properties either in vitro or in response to injury. Following SCI activated ependymal cells generate progeny cells which migrate to the injury site but fail to produce the appropriate type of cells in sufficient number to limit the damage, rendering this physiological response mainly ineffective. Research is now focusing on the manipulation of ependymal cells to produce cells of the oligodendrocyte lineage which are primarily lost in such a situation leading to secondary neuronal degeneration. Thus, there is a need for a more focused approach to understand the molecular properties of adult ependymal cells in greater detail and develop effective strategies for guiding their response during SCI.

  16. Experimental Neuromyelitis Optica Induces a Type I Interferon Signature in the Spinal Cord

    DEFF Research Database (Denmark)

    Oji, Satoru; Nicolussi, Eva-Maria; Kaufmann, Nathalie;

    2016-01-01

    Neuromyelitis optica (NMO) is an acute inflammatory disease of the central nervous system (CNS) which predominantly affects spinal cord and optic nerves. Most patients harbor pathogenic autoantibodies, the so-called NMO-IgGs, which are directed against the water channel aquaporin 4 (AQP4) on astr......Neuromyelitis optica (NMO) is an acute inflammatory disease of the central nervous system (CNS) which predominantly affects spinal cord and optic nerves. Most patients harbor pathogenic autoantibodies, the so-called NMO-IgGs, which are directed against the water channel aquaporin 4 (AQP4...... not profit from such treatments. How is I-IFN involved in NMO pathogenesis? To address this question, we made gene expression profiles of spinal cords from Lewis rat models of experimental neuromyelitis optica (ENMO) and experimental autoimmune encephalomyelitis (EAE). We found an upregulation of I...

  17. Unusual presentation in Axenfeld-Rieger syndrome

    Directory of Open Access Journals (Sweden)

    Rajul S Parikh

    2011-01-01

    Full Text Available We report an unusual presentation of a case of Axenfeld-Rieger (A-R syndrome. A 14-year-old male presented with gradual dimness of vision for 1 year and redness of left eye for 3 days. The patient had megalocornea with Haab′s striae in the right eye and posterior embryotoxon in both the eyes. In the left eye, there was a white cord-like structure traversing the anterior chamber with adhesions to iris tissue along its course. On two antiglaucoma medications, his intraocular pressure (IOP was 22 mm Hg in the right eye and 18 mm Hg in the left eye. Gonioscopy revealed a cord-like structure originating at the level of Schwalbe′s line. He underwent right eye trabeculectomy with mitomycin-C. This case highlights a rare presentation of a strange cord-like structure, a rare presentation of A-R syndrome.

  18. Tumor necrosis factor α sensitizes spinal cord TRPV1 receptors to the endogenous agonist N-oleoyldopamine

    OpenAIRE

    2010-01-01

    Abstract Modulation of synaptic transmission in the spinal cord dorsal horn is thought to be involved in the development and maintenance of different pathological pain states. The proinflamatory cytokine, tumor necrosis factor α (TNFα), is an established pain modulator in both the peripheral and the central nervous system. Up-regulation of TNFα and its receptors (TNFR) in dorsal root ganglion (DRG) cells and in the spinal cord has been shown to play an important role in neuropathic and inflam...

  19. Carpenter syndrome: report of two siblings.

    Science.gov (United States)

    Işlek, I; Küçüködük, S; Incesu, L; Selçuk, M B; Aygün, D

    1998-07-01

    Carpenter syndrome consists of acrocephaly, soft tissue syndactyly, short fingers, preaxial polydactyly, congenital heart disease, hypogenitalism, cryptorchidism, obesity, umbilical hernia and mental retardation. Here we report two affected sibs (IQs were 80 and 93) presenting various cerebrospinal malformations, i.e. frontal lobe deformity, narrowed foramen magnum, hypoplastic posterior fossa, kinked spinal cord, and syrinx cavitation demonstrated by magnetic resonance imaging.

  20. Establishment and evaluation of a rat model of complete transected spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Xuejun Li; Chunhai Huang; Shangming Liu; Xianrui Yuan

    2008-01-01

    BACKGROUND: The establishment of a rat model of complete transected spinal cord injury lacks technological specifications. The current models lack concordance and reliability, and the death rate of the experimental animals is high. Therefore, there is a great need for a reliable model to apply clinical applications of therapy.OBJECTIVE: To construct a rat model of complete transected spinal cord injury characterized by stability, reproducibility, and a high animal survival rate. DESIGN: Completely randomized controlled study.SETTING: Department of Neurosurgery, Xiangya Hospital of Central South University.MATERIALS: Fifty-five healthy specific pathogen free grade adult female Sprague Dawley rats were provided by the Experimental Animal Department, Xiangya Medical College, Central South University. Olympus BX51 imaging collecting analytic system was provided by Olympus Company, Japan; and SEN-7203 Nihon-Kohden electrical stimulator by Nihon Kohden, Japan. METHODS: This study was performed at the Laboratory of Neurosurgery, Xiangya Hospital of Central South University from April to June 2006. Experimental grouping: 55 rats were randomly divided into model group (n = 40) and sham surgery group (n = 15). In the model group, a self-made sliver hook was passed through the ventral side to support the spinal cord at the T12 segment and to shear it off. A complete transected spinal cord, 2 mm in length, was resected. In the sham surgery group, the spinal cord was identically exposed. The dura mater of the spinal cord was cut open, but the spinal cord was not damaged. MAIN OUTCOME MEASURES: Histopathological changes after spinal cord injury at L2 segment were observed subsequent to hematoxylin and eosin staining under optical microscopy. Olympus BX51 imaging collecting analytic system was used to count spinal cord ventral horn neurons. Motor function of rat hindlimb was evaluated with the Basso, Beattie and Bresnahan (BBB) scale. Paraplegia was evaluated as 0 point, and

  1. Temporal response of endogenous neural progenitor cells following injury to the adult rat spinal cord

    Directory of Open Access Journals (Sweden)

    Yilin eMao

    2016-03-01

    Full Text Available A pool of endogenous neural progenitor cells found in the ependymal layer and the sub-ependymal area of the spinal cord are reported to upregulate nestin in response to traumatic spinal cord injury. These cells could potentially be manipulated within a critical time period offering one innovative approach to the repair of spinal cord injury. However, little is known about the temporal response of endogenous neural progenitor cells following spinal cord injury. This study used a mild contusion injury in rat spinal cord and immunohistochemistry to determine the temporal response of ependymal neural progenitor cells following injury and their correlation to astrocyte activation at the lesion site. The results from the study demonstrated that Nestin staining intensity at the central canal peaked at 24 hours post-injury and then gradually declined over time. Reactive astrocytes double labelled by Nestin and GFAP were found at the lesion edge and commenced to form the glial scar from 1 week after injury. We conclude that the critical time period for manipulating endogenous neural progenitor cells following a spinal cord injury in rats is between 24 hrs when nestin expression in ependymal cells is increased and 1 week when astrocytes are activated in large numbers.

  2. Sustained delivery of bioactive neurotrophin-3 to the injured spinal cord.

    Science.gov (United States)

    Elliott Donaghue, Irja; Tator, Charles H; Shoichet, Molly S

    2015-01-01

    Spinal cord injury is a debilitating condition that currently lacks effective clinical treatment. Neurotrophin-3 (NT-3) has been demonstrated in experimental animal models to induce axonal regeneration and functional improvements, yet its local delivery remains challenging. For ultimate clinical translation, a drug delivery system is required for localized, sustained, and minimally invasive release. Here, an injectable composite drug delivery system (DDS) composed of biodegradable polymeric nanoparticles dispersed in a hyaluronan/methyl cellulose hydrogel was injected into the intrathecal space to achieve acute local delivery to the spinal cord after a thoracic clip compression injury. NT-3 was encapsulated in the DDS and released in vitro for up to 50 d. With a single injection of the DDS into the intrathecal space of the injured spinal cord, NT-3 diffused ventrally through the cord and was detectable in the spinal cord for at least 28 d therein. Delivery of NT-3 resulted in significant axon growth with no effect on the astroglial response to injury in comparison with vehicle and injury controls. NT-3 treatment promoted functional improvements at 21 d according to the Basso Beattie Bresnahan locomotor scale in comparison with the DDS alone. The sustained delivery of bioactive NT-3 to the injured spinal cord achieved in this study demonstrates the promise of this DDS for central nervous system repair.

  3. Intramedullary spinal cord ganglioglioma presenting as hyperhidrosis: unique symptoms and magnetic resonance imaging findings: case report.

    Science.gov (United States)

    Murakami, Tomohiro; Koyanagi, Izumi; Kaneko, Takahisa; Yoneta, Akihiro; Keira, Yoshiko; Wanibuchi, Masahiko; Hasegawa, Tadashi; Mikuni, Nobuhiro

    2013-02-01

    Hyperhidrosis is caused by a sympathetic dysfunction of the central or peripheral nervous system. Intramedullary spinal cord lesions can be a cause of hyperhidrosis. The authors report a rare case of intramedullary thoracic spinal cord ganglioglioma presenting as hyperhidrosis. This 16-year-old boy presented with abnormal sweating on the right side of the neck, chest, and the right arm that had been occurring for 6 years. Neurological examination revealed mild motor weakness of the right lower extremity and slightly decreased sensation in the left lower extremity. Hyperhidrosis was observed in the right C3-T8 dermatomes. Magnetic resonance imaging showed an intramedullary tumor at the right side of the spinal cord at the T2-3 level. The tumor showed partial enhancement after Gd administration. The patient underwent removal of the tumor via hemilaminectomy of T2-3. Only subtotal resection was achieved because the margins of the tumor were unclear. Histopathological examination revealed ganglioglioma. Hyperhidrosis gradually improved after surgery. Hyperhidrosis is a rare clinical manifestation of intramedullary spinal cord tumors, and only a few cases have been reported in the literature. The location of the tumor origin, around the right gray matter of the lateral spinal cord, may account for the hyperhidrosis as the initial symptom in this patient. Physicians should examine the spinal cord using MRI studies when a patient has hyperhidrosis with some motor or sensory symptoms of the extremities.

  4. Vascularized peripheral nerve trunk autografted in the spinal cord: a new experimental model in adult rats

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective: To investigate the effect of vascularized peripheral nerve trunk autografted in spinal cord. Methods: With modern microsurgical technique,vascularized peripheral median and ulnar nerve trunk autografted in the upper thoracic region of the spinal cord were established in 20 female adult rats. The origin and the termination of axons in the graft were studied by retrograde neuronal labeling with horseradish peroxidase (HRP).Cord, nerve grafts and some normal median and ulnar nerves in the right upper limb were removed and sectioned for Bielschowsky's silver stain and haematoxylin and eosin (H&E) stain. Light and electron microscopic examination and electrophysiological examination were applied.Results: The grafts were innervated by many new fibers. Studies with HRP indicated that new axons in graft were originated from intrinsic central nervous system (CNS) neurons with their cell bodies from brain stem to sacral segments of spinal cord. Other axons arose from dorsal root ganglia at the level of graft and at least 19 distal segments to them. Together with electron microscopy, electrophysiological examination, silver and H&E stain, the results demonstrated that vascularized peripheral nerve trunk grafted in spinal cord attracted many neurons to grow into the nerve grafts.Conclusions: The findings implicate that CNS is able to regenerate much better in vascularized nerve autografted in spinal cord.

  5. Pyramidal tract abnormalities in the human fetus and infant with trisomy 18 syndrome.

    Science.gov (United States)

    Miyata, Hajime; Miyata, Mio; Ohama, Eisaku

    2014-06-01

    Trisomy 18 or Edwards syndrome is known to exhibit various developmental abnormalities in the central nervous system. We report dominant uncrossed pyramidal tract in trisomy 18 syndrome, based on the postmortem neuropathologic study of eight consecutive autopsied fetuses and infants with trisomy 18 ranging in age from 16 to 39 weeks of gestation, including six males and two females, along with autopsy cases of a stillborn triploid infant with 69XXX and two stillborn infants without chromosomal or neurodevelopmental abnormalities. Five out of eight cases with trisomy 18 showed a larger proportion of uncrossed than crossed pyramidal tract. All of these cases were male, and the anterior corticospinal tract on one side was constantly larger than the contralateral lateral corticospinal tract in the spinal cord on both sides, while the pyramidal tract was hypoplastic in female cases with trisomy 18 and a case with 69XXX. Abnormal pyramidal decussation has been found in cases with posterior fossa malformations such as occipital encephaloceles, Dandy-Walker malformation, Joubert syndrome and Möbius syndrome, but has not been described in cases with trisomy 18. Our data, together with the previous reports describing uncrossed aberrant ipsilateral pyramidal tract in patients with congenital mirror movements caused by DCC gene mutation in chromosome 18, and hypolasia and hyperplasia of the pyramidal tract in X-linked recessive disorders caused by L1CAM and Kal1 gene mutations, respectively, suggest a role of trisomy 18 in association with X-chromosome in the abnormal development of the pyramidal tract.

  6. Activity-Based Restorative Therapies: Concepts and Applications in Spinal Cord Injury-Related Neurorehabilitation

    Science.gov (United States)

    Sadowsky, Cristina L.; McDonald, John W.

    2009-01-01

    Physical rehabilitation following spinal cord injury-related paralysis has traditionally focused on teaching compensatory techniques, thus enabling the individual to achieve day-to-day function despite significant neurological deficits. But the concept of an irreparable central nervous system (CNS) is slowly being replaced with evidence related to…

  7. Spinal cord ependymoma presenting with neurological deficits in the setting of trauma.

    Science.gov (United States)

    Saad, Amin F; Nickell, Larry T; Finn, S Sam; Opatowsky, Michael J

    2014-07-01

    Ependymomas represent 4% of all primary central nervous system neoplasms in adults, with 30% occurring in the spinal cord. We describe a young man with neurological deficits following a motor vehicle accident who was found to have an intramedullary cervicothoracic ependymoma.

  8. Immunocytochemical localization of zinc transporter 3 in the ependyma of the mouse spinal cord.

    Science.gov (United States)

    Danscher, Gorm; Wang, Zhanyou; Kim, Yong Kuk; Kim, Sung Joo; Sun, Yuanjie; Jo, Seung Mook

    2003-05-15

    We report, for the first time, the light microscopical and ultrastructural appearance of ZnT3-immunoreactivities in the ependymal cells of the central canal of the mouse spinal cord. Light microscopy revealed the presence of ZnT3-immunoreactive (Ir) ependymal cells in 1 microm thick epon sections stained by the ABC method. The ZnT3-Ir cells were observed at all levels of the spinal cord, but were a little more numerous in lumbosacral segments than in cervicothoracic segments. The ZnT3-Ir cells had large, ovoid nuclei with abundant cytoplasm, and protruded into the lumen of the central canal. Our ultrastructural findings suggest that the ZnT3-Ir ependymal cells possess secretory activity directed towards the central canal. We propose that they may play a role in the trans-ependymal mechanism responsible for zinc homeostasis between cerebrospinal fluid and the central area of the gray matter.

  9. Role of plasma membrane calcium ATPase 2 in spinal cord pathology

    Institute of Scientific and Technical Information of China (English)

    Amanda; Kathleen; Fakira; Stella; Elkabes

    2010-01-01

    A number of studies have indicated that plasma membrane calcium ATPases(PMCAs) are expressed in the brain and spinal cord and could play important roles not only in the maintenance of cellular calcium homeostasis but also in the survival and function of central nervous system cells under pathological conditions.The different regional and cellular distributions of the various PMCA isoforms and splice variants in the nervous system and the diverse phenotypes of PMCA knockout mice support the notion that each isoform might play a distinct role. Especially in the spinal cord,the survival of neurons and,in particular,motor neurons could be dependent on PMCA2.This is indicated by the knockdown of PMCA2 in pure spinal cord neuronal cultures that leads to cell death via a decrease in collapsing response mediator protein 1 levels.Moreover,the progressive decline in the number of motor neurons in PMCA2-null mice andheterozygous mice further supports this notion.Therefore,the reported reduction in PMCA2 mRNA and protein levels in the inflamed spinal cord of mice affected by experimental autoimmune encephalomyelitis(EAE) ,an animal model of multiple sclerosis,and after spinal cord contusion injury,suggests that changes in PMCA2 expression could be a cause of neuronal pathology and death during inflammation and injury.Glutamate excitotoxicity mediated via kainate receptors has been implicated in the neuropathology of both EAE and spinal cord injury,and has been identified as a trigger that reduces PMCA2 levels in pure spinal cord neuronal cultures through degradation of the pump by calpain without affecting PMCA2 transcript levels.It remains to be determined which other stimuli modulate PMCA2 mRNA expression in the aforementioned pathological conditions of the spinal cord.

  10. Major vault protein promotes locomotor recovery and regeneration after spinal cord injury in adult zebrafish.

    Science.gov (United States)

    Pan, Hong-Chao; Lin, Jin-Fei; Ma, Li-Ping; Shen, Yan-Qin; Schachner, Melitta

    2013-01-01

    In contrast to mammals, adult zebrafish recover locomotor functions after spinal cord injury (SCI), in part due to axonal regrowth and regeneration permissivity of the central nervous system. Upregulation of major vault protein (MVP) expression after spinal cord injury in the brainstem of the adult zebrafish prompted us to probe for its contribution to recovery after SCI. MVP is a multifunctional protein expressed not only in many types of tumours but also in the nervous system, where its importance for regeneration is, however, unclear. Using an established zebrafish SCI model, we found that MVP mRNA and protein expression levels were increased in ependymal cells in the spinal cord caudal to the lesion site at 6 and 11 days after SCI. Double immunolabelling showed that MVP was co-localised with Islet-1 or tyrosine hydroxylase around the central canal of the spinal cord in sham-injured control fish and injured fish 11 days after surgery. MVP co-localised with the neural stem cell marker nestin in ependymal cells after injury. By using an in vivo morpholino-based knock-down approach, we found that the distance moved by MVP morpholino-treated fish was reduced at 4, 5 and 6 weeks after SCI when compared to fish treated with standard control morpholino. Knock-down of MVP resulted in reduced regrowth of axons from brainstem neurons into the spinal cord caudal to the lesion site. These results indicate that MVP supports locomotor recovery and axonal regrowth after SCI in adult zebrafish.

  11. NEW TRENDS IN STEEL CORD DEVELOPMENT

    Directory of Open Access Journals (Sweden)

    A. V. Vedeneev

    2011-01-01

    Full Text Available The analysis of directions of metal cord constructions developments depending on the place of application in tires of different function is carried out. the requirements to metal cord, which are necessary to be taken into account at development of its new type, are given. the peculiarities of perspective types of reinforcing agents for tires, and also advantages of new types of metal cord over the existing ones are shown.

  12. Distinct Presentations of Hernia of Umbilical Cord

    Science.gov (United States)

    Mirza, Bilal; Ali, Waqas

    2016-01-01

    Hernia of umbilical cord is a well-known entity which presents with herniation of small bowel into the proximal part of umbilical cord. It has very good prognosis after surgical repair. Occasionally, it can have distinct presentations and varied malformations at the umbilicus which have bearing on the course of treatment and final outcome. Herein, we describe various presentations and malformations associated with hernia of umbilical cord. Embryological extrapolation is attempted for the malformations at umbilicus. PMID:27896161

  13. Somatostatinergic nerves in the cervical spinal cord of the monkey.

    Science.gov (United States)

    Burnweit, C; Forssmann, W G

    1979-08-03

    Somatostatinergic nerves in the spinal cord of the monkey were investigated utilizing immunohistochemistry with various antibodies against synthetic somatostatin. In contrast to earlier investigations, it is shown that somatostatinergic nerve endings occur in most of the areas of the grey matter of the spinal cord. The somatostatinergic axons are, however, characteristically distributed in three main regions: (1) Densely-packed endings are seen in lamina II of the substantia gelatinosa, forming a crescent-shaped pattern in the columna dorsalis. Somatostatin immunoreactivity is also seen in lamina I and in the Lissauer tract. (2) A fine network of fibers is observed around the central canal; the endings are concentrated on special cell bodies. Some single perikarya are also stained in this region. (3) A loose network of single fibers is found ending on perikarya of the columna lateralis or ventralis. The perikarya of the nerve axons, with the exception of those terminating in the columna dorsalis, have as yet not been identified. In order to better understand the somatostatinergic system of the spinal cord, these newly-detected somatostatinergic nerves must be studied and their exact pathways analyzed.

  14. Spinal cord compression due to ethmoid adenocarcinoma.

    Science.gov (United States)

    Johns, D R; Sweriduk, S T

    1987-10-15

    Adenocarcinoma of the ethmoid sinus is a rare tumor which has been epidemiologically linked to woodworking in the furniture industry. It has a low propensity to metastasize and has not been previously reported to cause spinal cord compression. A symptomatic epidural spinal cord compression was confirmed on magnetic resonance imaging (MRI) scan in a former furniture worker with widely disseminated metastases. The clinical features of ethmoid sinus adenocarcinoma and neoplastic spinal cord compression, and the comparative value of MRI scanning in the neuroradiologic diagnosis of spinal cord compression are reviewed.

  15. Cord blood stem cell banking and transplantation.

    Science.gov (United States)

    Dhot, P S; Nair, V; Swarup, D; Sirohi, D; Ganguli, P

    2003-12-01

    Stem cells have the ability to divide for indefinite periods in culture and to give rise to specialized cells. Cord blood as a source of hematopoietic stem cells (HSC) has several advantages as it is easily available, involves non-invasive collection procedure and is better tolerated across the HLA barrier. Since the first cord blood transplant in 1988, over 2500 cord blood HSC transplants have been done world wide. Since then, the advantages of cord blood as a source of hematopietic stem cells for transplantation have become clear. Firstly, the proliferative capacity of HSC in cord blood is superior to that of cells in bone marrow or blood from adults. A 100 ml unit of cord blood contains 1/10th the number of nucleated cells and progenitor cells (CD34+ cells) present in 1000 ml of bone marrow, but because they proliferate rapidly, the stem cell in a single unit of cord blood can reconstitute the entire haematopoietic system. Secondly, the use of cord blood reduces the risk of graft vs host disease. Cord Blood Stem Cell banks have been established in Europe and United States to supply HSC for related and unrelated donors. Currently, more than 65,000 units are available and more than 2500 patients have received transplants of cord blood. Results in children have clearly shown that the number of nucleated cells in the infused cord blood influences the speed of recovery of neutrophils and platelets after myeloablative chemotherapy. The optimal dose is about 2 x 10(7) nucleated cells/kg of body weight. The present study was carried out for collection, separation, enumeration and cryopreservation of cord blood HSC and establishing a Cord Blood HSC Bank. 172 samples of cord blood HSC were collected after delivery of infant prior to expulsion of placenta. The average cord blood volume collected was 101.20 ml. Mononuclear cell count ranged from 7.36 to 25.6 x 10(7)/ml. Viability count of mononuclear cells was 98.1%. After 1 year of cryopreservation, the viability count on

  16. Serotonin syndrome

    Science.gov (United States)

    Hyperserotonemia; Serotonergic syndrome; Serotonin toxicity; SSRI - serotonin syndrome; MAO - serotonin syndrome ... two medicines that affect the body's level of serotonin are taken together at the same time. The ...

  17. Multifocal Dysembryoplastic Neuroepithelial Tumour with Intradural Spinal Cord Lipomas: Report of a Case

    Directory of Open Access Journals (Sweden)

    Richard D. White

    2011-01-01

    Full Text Available We report a case of temporal lobe epilepsy and incomplete Brown-Sequard syndrome of the thoracic cord. Computed tomography and magnetic resonance (MR imaging showed multiple supratentorial masses with the classical radiological appearances of multifocal dysembryoplastic neuroepithelial tumour (DNET. Spinal MR imaging revealed intradural lipomas, not previously reported in association with multifocal DNET. Presentation and imaging findings are discussed along with classification and natural history of the tumour.

  18. Effects of electroacupuncture on c-Fos expression in the spinal cord and brain of rats with chronic visceral hypersensitivity

    Institute of Scientific and Technical Information of China (English)

    Xiaomei Wang; Huirong Liu; Guanghong Ding; Yunfei Chen; Huangan Wu; Na Li; Enhua Zhou; Xiudi Qin; Lingsong Yuan

    2009-01-01

    BACKGROUND: Visceral hypersensitivity is the main cause of irritable bowel syndrome, c-Fos is a marker of visceral hypersensitivity in the central nervous system. Electroacupuncture can relieve chronic visceral hypersensitivity in rats, but the mechanism is still unknown.OBJECTIVE: To identify c-Fos expression in the spinal cord and cerebral cortex of rats with chronic visceral hypersensitivity, and to test the effects of electroacupuncture on pain sensitivity in rats with chronic visceral hypersensitivity.DESIGN, TIME AND SETTING: A randomized controlled animal experiment was performed at the Animal Experimental Center, Shanghai University of Traditional Chinese Medicine, from January to April, 2007.MATERIALS: A total of 24 neonatal, male, Sprague Dawley rats, aged five days old, were equally and randomly assigned into a normal group, a model group, and an electroacupuncture group. Rabbit anti-rat c-Fos antibody and Evision secondary antibody kits (Sigma, USA), diaminobenzidine kit (Dako, Denmark), and an LD202H electroacupuncture apparatus (Huawei, Beijing, China) were used in this study.METHODS: Neonatal rats from the model and electroacupuncture groups were used to establish rat models of chronic visceral hypersensitivity by the saccule stimulation method. After model establishment, 0.25 mm diameter electric needles were inserted into Tianshu (ST 25) and Shangjuxu (ST37) at a depth of approximately 0.5 cm, with an square wave (alternating current frequency at 100/20 Hz, amplitude ranged 0.2-0.6 ms, intensify at 1 mA) once for 20 minutes, once a day, for seven days. Rats in the normal and model groups were not treated.MAIN OUTCOME MEASURES: Following 7 days of treatment, c-Fos expression in the spinal cord and cerebral cortex was detected by immunohistochemistry. After the first electroacupuncture treatment, abdominal withdrawal reflex scores were investigated to evaluate the pain threshold for chronic visceral hypersensitivity in rats.RESULTS: Visceral

  19. Segmentation of the human spinal cord.

    Science.gov (United States)

    De Leener, Benjamin; Taso, Manuel; Cohen-Adad, Julien; Callot, Virginie

    2016-04-01

    Segmenting the spinal cord contour is a necessary step for quantifying spinal cord atrophy in various diseases. Delineating gray matter (GM) and white matter (WM) is also useful for quantifying GM atrophy or for extracting multiparametric MRI metrics into specific WM tracts. Spinal cord segmentation in clinical research is not as developed as brain segmentation, however with the substantial improvement of MR sequences adapted to spinal cord MR investigations, the field of spinal cord MR segmentation has advanced greatly within the last decade. Segmentation techniques with variable accuracy and degree of complexity have been developed and reported in the literature. In this paper, we review some of the existing methods for cord and WM/GM segmentation, including intensity-based, surface-based, and image-based methods. We also provide recommendations for validating spinal cord segmentation techniques, as it is important to understand the intrinsic characteristics of the methods and to evaluate their performance and limitations. Lastly, we illustrate some applications in the healthy and pathological spinal cord. One conclusion of this review is that robust and automatic segmentation is clinically relevant, as it would allow for longitudinal and group studies free from user bias as well as reproducible multicentric studies in large populations, thereby helping to further our understanding of the spinal cord pathophysiology and to develop new criteria for early detection of subclinical evolution for prognosis prediction and for patient management. Another conclusion is that at the present time, no single method adequately segments the cord and its substructure in all the cases encountered (abnormal intensities, loss of contrast, deformation of the cord, etc.). A combination of different approaches is thus advised for future developments, along with the introduction of probabilistic shape models. Maturation of standardized frameworks, multiplatform availability, inclusion

  20. Noninvasive transcutaneous bionic baroreflex system prevents severe orthostatic hypotension in patients with spinal cord injury.

    Science.gov (United States)

    Yoshida, Masayoshi; Murayama, Yoshinori; Chishaki, Akiko; Sunagawa, Kenji

    2008-01-01

    Central baroreflex failure in patients with spinal cord injury results in serious orthostatic hypotension. We examined if transcutaneous electrical stimulation regulates arterial pressure in those patients. We identified skin regions capable of increasing arterial pressure and determined respective transfer function. Using the transfer function, we designed the feedback regulator (i.e., bionic baroreflex system) to control arterial pressure. Orthostatic stress decreased arterial pressure profoundly. Activation of bionic regulator restored and maintained arterial pressure at pre-specified levels. We conclude that the transcutaneous bionic system is noninvasive and capable of stabilizing arterial pressure in patients with spinal cord injury.

  1. Toxoplasmosis in cord blood transplantation recipients.

    Science.gov (United States)

    Bautista, G; Ramos, A; Forés, R; Regidor, C; Ruiz, E; de Laiglesia, A; Navarro, B; Bravo, J; Portero, F; Sanjuan, I; Fernández, M N; Cabrera, R

    2012-10-01

    Toxoplasmosis is a devastating opportunistic infection that can affect immunocompromised patients such as cord blood transplantation (CBT) recipients. The clinical characteristics of 4 toxoplasmosis CBT patients treated at our institution are reviewed, together with 5 cases collected from the literature. The rate of toxoplasmosis in our hospital was 6% in CBT recipients and 0.2% in other types of allogeneic hematopoietic stem cell transplantation (P < 0.001). Five patients (56%) presented disseminated toxoplasmosis and 4 patients (44%) had localized infection in the central nervous system. In 5 of the 9 patients considered (56%), cytomegalovirus viral replication had been detected before the clinical onset of toxoplasmosis. Seven patients (78%) had previously developed graft-versus-host disease. All patients who exhibited disseminated disease died due to Toxoplasma infection. Pre-transplant serology was positive in 1 patient, negative in 3 patients, and not performed in another. Only 1 of these 5 patients with disseminated disease had received Toxoplasma prophylaxis with cotrimoxazole. It could be concluded that mortality in CBT patients with disseminated toxoplasmosis is unacceptably high. The negative results of serology in the majority of these cases, and its unspecific clinical presentation, makes diagnosis exceedingly difficult. Better diagnostic tests and prophylaxis strategy are needed in CBT recipients.

  2. High-fat simple carbohydrate feeding impairs central and peripheral monoamine metabolic pathway triggering the onset of metabolic syndrome in C57Bl/6J mice

    Directory of Open Access Journals (Sweden)

    Serena S D'Souza

    2016-01-01

    Conclusion: HFSC diet impairs the central and peripheral dopaminergic and noradrenergic pathways in mice as evidenced by the disturbances in their hypothalamic, plasma, and urine levels and this might be one of the early factors contributing towards the development of the MetS.

  3. Synaptogenesis and Myelination in the Nucleus/Tractus Solitarius: Potential Role in Apnea of Prematurity, Congenital Central Hypoventilation, and Sudden Infant Death Syndrome.

    Science.gov (United States)

    Sarnat, Harvey B; Flores-Sarnat, Laura

    2016-05-01

    Fetuses as early as 15 weeks' gestation exhibit rhythmical respiratory movements shown by real-time ultrasonography. The nucleus/tractus solitarius is the principal brainstem respiratory center; other medullary nuclei also participate. The purpose was to determine temporal maturation of synaptogenesis. Delayed synaptic maturation may explain neurogenic apnea or hypoventilation of prematurity and some cases of sudden infant death syndrome. Sections of medulla oblongata were studied from 30 human fetal and neonatal brains 9 to 41 weeks' gestation. Synaptophysin demonstrated the immunocytochemical sequence of synaptogenesis. Other neuronal markers and myelin stain also were applied. The nucleus/tractus solitarius was similarly studied in fetuses with chromosomopathies, metabolic encephalopathies, and brain malformations. Synapse formation in the nucleus solitarius begins at about 12 weeks' gestation and matures by 15 weeks; myelination initiated at 33 weeks. Synaptogenesis was delayed in 3 fetuses with different conditions, but was not specific for only nucleus solitarius. Delayed synaptogenesis or myelination in the nucleus solitarius may play a role in neonatal hypoventilation, especially in preterm infants and in some sudden infant death syndrome cases.

  4. Ambulation and spinal cord injury.

    Science.gov (United States)

    Hardin, Elizabeth C; Kobetic, Rudi; Triolo, Ronald J

    2013-05-01

    Walking is possible for many patients with a spinal cord injury. Avenues enabling walking include braces, robotics and FES. Among the benefits are improved musculoskeletal and mental health, however unrealistic expectations may lead to negative changes in quality of life. Use rigorous assessment standards to gauge the improvement of walking during the rehabilitation process, but also yearly. Continued walking after discharge may be limited by challenges, such as lack of accessibility in and outside the home, and complications, such as shoulder pain or injuries from falls. It is critical to determine the risks and benefits of walking for each patient.

  5. Large-scale synchronized activity in the embryonic brainstem and spinal cord

    Directory of Open Access Journals (Sweden)

    Yoko eMomose-Sato

    2013-04-01

    Full Text Available In the developing central nervous system, spontaneous activity appears well before the brain responds to external sensory inputs. One of the earliest activities is observed in the hindbrain and spinal cord, which is detected as rhythmic electrical discharges of cranial and spinal motoneurons or oscillations of Ca2+- and voltage-related optical signals. Shortly after the initial expression, the spontaneous activity appearing in the hindbrain and spinal cord exhibits a large-scale correlated wave that propagates over a wide region of the central nervous system, maximally extending to the lumbosacral cord and to the forebrain. In this review, we describe several aspects of this synchronized activity by focusing on the basic properties, development, origin, propagation pattern, pharmacological characteristics, and possible mechanisms underlying the generation of the activity. These profiles differ from those of the respiratory and locomotion pattern generators observed in the mature brainstem and spinal cord, suggesting that the wave is primordial activity that appears during a specific period of embryonic development and plays some important roles in the development of the central nervous system.

  6. Pulmonary mucormycosis presenting with vocal cord paralysis

    OpenAIRE

    Gayathri Devi, H. J.; Mohan Rao, K.N.; K M Prathima; Moideen, Riyaz

    2013-01-01

    Pulmonary mucormycosis is a relatively uncommon infection. It can present in various forms. Very few cases of pulmonary mucormycosis presenting as vocal cord paralysis have been described in the literature. We report a case of pulmonary mucormycosis presenting as vocal cord paralysis in an uncontrolled diabetic patient.

  7. [Neurogenic bladder caused by spinal cord traction].

    Science.gov (United States)

    Garat, J M; Aragona, F; Martinez, E

    1985-01-01

    A neurogenic bladder was the presenting syndrome in three cases of spinal cord traction. Of the typical symptomatic triad: neuro-orthopedic, cutaneous and urologic, the latter was of primary importance. Symptoms in the first case were incomplete bladder retention with distention of upper urinary tract, right-sided vesicorenal reflux and renal insufficiency. Six months after excision of a sacral lipoma and freeing of the filum terminale, micturition had become normal without residue, and renal function normalized. Right-sided reflux was corrected by submucosal advancement surgery with good results. The clinical history was more suggestive in the second case. Although inaugural symptoms were mictional, there was foot paralysis and a retrosacral lipoma above an abnormal hairy tuft in the upper part of the gluteal cleft. Operation revealed the presence of a dermoid cyst and a lipoma. Their excision combined with section of the filum terminale allowing ascension of the medullary cone. Marked clinical and urodynamic improvement was obtained with normal micturition and disappearance of incontinence. An anti-reflux operation suppressed residual reflux with good urographic results. Marked improvement in mictional disorders was obtained also in the 3rd case after excision of a sacral extradural lipoma and section of the filum terminale, allowing objective ascension of the medullary cone by 4 cm. A very detailed analysis was conducted of similar cases reported in the literature, about 2% of neurogenic bladders in children being affected. The importance of early diagnosis is emphasized as well as the essential need to establish a precise diagnosis of the lipoma of cauda equina and of medullary fixation. Early neurosurgery is justified by the high frequency of improvement in cases treated in this way.

  8. KBG syndrome

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    Brancati Francesco

    2006-12-01

    Full Text Available Abstract KBG syndrome is a rare condition characterised by a typical facial dysmorphism, macrodontia of the upper central incisors, skeletal (mainly costovertebral anomalies and developmental delay. To date, KBG syndrome has been reported in 45 patients. Clinical features observed in more than half of patients that may support the diagnosis are short stature, electroencephalogram (EEG anomalies (with or without seizures and abnormal hair implantation. Cutaneous syndactyly, webbed short neck, cryptorchidism, hearing loss, palatal defects, strabismus and congenital heart defects are less common findings. Autosomal dominant transmission has been observed in some families, and it is predominantly the mother, often showing a milder clinical picture, that transmits the disease. The diagnosis is currently based solely on clinical findings as the aetiology is unknown. The final diagnosis is generally achieved after the eruption of upper permanent central incisors at 7–8 years of age when the management of possible congenital anomalies should have been already planned. A full developmental assessment should be done at diagnosis and, if delays are noted, an infant stimulation program should be initiated. Subsequent management and follow-up should include an EEG, complete orthodontic evaluation, skeletal investigation with particular regard to spine curvatures and limb asymmetry, hearing testing and ophthalmologic assessment.

  9. Galactorrhea: a complication of spinal cord injury.

    Science.gov (United States)

    Yarkony, G M; Novick, A K; Roth, E J; Kirschner, K L; Rayner, S; Betts, H B

    1992-09-01

    Galactorrhea, a secretion of milk or milk-like products from the breast in the absence of parturition, has been reported to occur in women with spinal cord injuries in association with amenorrhea and hyperprolactinemia. Four cases of galactorrhea in association with spinal cord injury are reported. Galactorrhea developed in four spinal cord injured women who had thoracic paraplegia. The onset of galactorrhea was from one month to five months after injury. Although the onset of galactorrhea may have been related to prescribed medications in all four cases, insufficient data exist to draw conclusions. The three women whose galactorrhea persisted declined treatment and galactorrhea continuing for more than two years in one instance. We conclude that galactorrhea with or without amenorrhea may develop after a spinal cord injury and that spinal cord injured women may have an enhanced sensitivity to medication-induced galactorrhea.

  10. Spinal cord ischemia secondary to hypovolemic shock.

    Science.gov (United States)

    Oh, Jacob Yl; Kapoor, Siddhant; Koh, Roy Km; Yang, Eugene Wr; Hee, Hwan-Tak

    2014-12-01

    A 44-year-old male presented with symptoms of spinal cord compression secondary to metastatic prostate cancer. An urgent decompression at the cervical-thoracic region was performed, and there were no complications intraoperatively. Three hours postoperatively, the patient developed acute bilateral lower-limb paralysis (motor grade 0). Clinically, he was in class 3 hypovolemic shock. An urgent magnetic resonance imaging (MRI) was performed, showing no epidural hematoma. He was managed aggressively with medical therapy to improve his spinal cord perfusion. The patient improved significantly, and after one week, he was able to regain most of his motor functions. Although not commonly reported, spinal cord ischemia post-surgery should be recognized early, especially in the presence of hypovolemic shock. MRI should be performed to exclude other potential causes of compression. Spinal cord ischemia needs to be managed aggressively with medical treatment to improve spinal cord perfusion. The prognosis depends on the severity of deficits, and is usually favorable.

  11. Templated agarose scaffolds for the support of motor axon regeneration into sites of complete spinal cord transection.

    Science.gov (United States)

    Gao, Mingyong; Lu, Paul; Bednark, Bridget; Lynam, Dan; Conner, James M; Sakamoto, Jeff; Tuszynski, Mark H

    2013-02-01

    Bioengineered scaffolds have the potential to support and guide injured axons after spinal cord injury, contributing to neural repair. In previous studies we have reported that templated agarose scaffolds can be fabricated into precise linear arrays and implanted into the partially injured spinal cord, organizing growth and enhancing the distance over which local spinal cord axons and ascending sensory axons extend into a lesion site. However, most human injuries are severe, sparing only thin rims of spinal cord tissue in the margins of a lesion site. Accordingly, in the present study we examined whether template agarose scaffolds seeded with bone marrow stromal cells secreting Brain-Derived Neurotrophic Factor (BDNF) would support regeneration into severe, complete spinal cord transection sites. Moreover, we tested responses of motor axon populations originating from the brainstem. We find that templated agarose scaffolds support motor axon regeneration into a severe spinal cord injury model and organize axons into fascicles of highly linear configuration. BDNF significantly enhances axonal growth. Collectively, these findings support the feasibility of scaffold implantation for enhancing central regeneration after even severe central nervous system injury.

  12. Ontogeny of the collar cord: neurulation in the hemichordate Saccoglossus kowalevskii.

    Science.gov (United States)

    Kaul, Sabrina; Stach, Thomas

    2010-10-01

    The chordate body plan is characterized by a central notochord, a pharynx perforated by gill pores, and a dorsal central nervous system. Despite progress in recent years, the evolutionary origin of each of theses characters remains controversial. In the case of the nervous system, two contradictory hypotheses exist. In the first, the chordate nervous system is derived directly from a diffuse nerve net; whereas, the second proposes that a centralized nervous system is found in hemichordates and, therefore, predates chordate evolution. Here, we document the ontogeny of the collar cord of the enteropneust Saccoglossus kowalevskii using transmission electron microscopy and 3D-reconstruction based on completely serially sectioned stages. We demonstrate that the collar cord develops from a middorsal neural plate that is closed in a posterior to anterior direction. Transversely oriented ependymal cells possessing myofilaments mediate this morphogenetic process and surround the remnants of the neural canal in juveniles. A mid-dorsal glandular complex is present in the collar. The collar cord in juveniles is clearly separated into a dorsal saddle-like region of somata and a ventral neuropil. We characterize two cell types in the somata region, giant neurons and ependymal cells. Giant neurons connect via a peculiar cell junction that seems to function in intercellular communication. Synaptic junctions containing different vesicle types are present in the neuropil. These findings support the hypotheses that the collar cord constitutes a centralized element of the nervous system and that the morphogenetic process in the ontogeny of the collar cord is homologous to neurulation in chordates. Moreover, we suggest that these similarities are indicative of a close phylogenetic relationship between enteropneusts and chordates.

  13. Experimental autoimmune prostatitis induces microglial activation in the spinal cord

    Science.gov (United States)

    Wong, Larry; Done, Joseph D.; Schaeffer, Anthony J.; Thumbikat, Praveen

    2014-01-01

    Background The pathogenesis of chronic prostatitis/chronic pelvic pain syndrome is unknown and factors including the host’s immune response and the nervous system have been attributed to the development of CP/CPPS. We previously demonstrated that mast cells and chemokines such as CCL2 and CCL3 play an important role in mediating prostatitis. Here, we examined the role of neuroinflammation and microglia in the CNS in the development of chronic pelvic pain. Methods Experimental autoimmune prostatitis (EAP) was induced using a subcutaneous injection of rat prostate antigen. Sacral spinal cord tissue (segments S4–S5) was isolated and utilized for immunofluorescence or QRT-PCR analysis. Tactile allodynia was measured at baseline and at various points during EAP using Von Frey fibers as a function for pelvic pain. EAP mice were treated with minocycline after 30 days of prostatitis to test the efficacy of microglial inhibition on pelvic pain. Results Prostatitis induced the expansion and activation of microglia and the development of inflammation in the spinal cord as determined by increased expression levels of CCL3, IL-1β, Iba1, and ERK1/2 phosphorylation. Microglial activation in mice with prostatitis resulted in increased expression of P2X4R and elevated levels of BDNF, two molecular markers associated with chronic pain. Pharmacological inhibition of microglia alleviated pain in mice with prostatitis and resulted in decreased expression of IL-1β, P2X4R, and BDNF. Conclusion Our data shows that prostatitis leads to inflammation in the spinal cord and the activation and expansion of microglia, mechanisms that may contribute to the development and maintenance of chronic pelvic pain. PMID:25263093

  14. Minimally invasive tethered cord release in children: A technical note

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    S. Kağan Başarslan

    2014-03-01

    Full Text Available Tethered cord release is commonly performed in pediatric neurosurgery. Nowadays, minimally invasive procedures are created growing interest due to its highly tolerable nature for surgery. It has been main purpose a minimal damaging on access route and maximum protection of normal structures in surgery. We present a surgical treatment of tethered cord syndrome, by which is provided the cord releasing unlike the many methods being applied with tissue removal. The main advantage of performing this surgery through 2 cm hole is to avoid removing ligamentum flavum and bony structure like lamina in addition to reduce the length of the incision and the related scar tissue. J Clin Exp Invest 2014; 5 (1: 115-117 Technical note: the patient was taken on the operating table in the sitting-prone position, and L5-S1 distance was determined by fluoroscopy. The skin and subcutaneous tissues was passed via a 2 cm vertical incision settled in 0.5 cm laterally from midline. L5-S1 distance and its covering ligamentum flavum are displayed by the guidance of L5 lamina. Williams’s retractor was placed in the distance after fetching microscope. The foregoing procedures are the same with microdiscectomic surgery. By a vertical incision made on the flavum, its both layer was lifted up and hanged with simple suture on the back tissue for a comfortable exposure of the Dura. Thecal sac was opened by 0.5 cm long vertical incision on the Dura after obtaining secure CSF drainage with the help of yellow-tipped syringe needle. With finding by a nerve hook, the phylum was burned and released securely. Then the Dura was sutured primarily for the closure by means of microsurgery instruments, and flavum was laid on it again.

  15. Ependymal cell proliferation and apoptosis following acute spinal cord injury in the adult rat

    Institute of Scientific and Technical Information of China (English)

    Xu Wang; Jun Qian; Yanchao Ma; Guoxin Nan; Shuanke Wang; Yayi Xia; Youcheng Zhang

    2008-01-01

    BACKGROUND: Studies have reported that spinal cord injury can induce the reactive proliferation of ependymal cells and secondarily cause the apoptosis of nerve cells. However, there is no generally accepted theory on the apoptotic characteristics of ependymal cells in the injured spinal cord.OBJECTIVE: To observe the reactive proliferation and apoptosis of ependymal cells in adult rats following acute spinal cord injury.DESIGN, TIME AND SETTING: A randomized control study based on neuropathology was performed in the Third Military Medical University of Chinese PLA between 2005 and 2007.MATERIALS: Forty healthy, adult, Wistar rats were included in the present study.METHODS: Moderate spinal cord injury was established in twenty rats using Feeney's method, while the remaining 20 rats served as controls and were only treated with laminectomy. All rats were injected intraperitoneally with 1.25 mL of BrdU solution (10 mg BrdU/mL saline) 3 times at 4 hours intervals during the 12 hours prior to sacrifice.MAIN OUTCOME MEASURES: Ependymal cell proliferation and apoptosis in the rat spinal cord were determined by BrdU and nestin immunofluorescence double-labeling, as well as the TUNEL method, at 1, 3, 7, and 14 days after operation.RESULTS: In the moderate spinal cord injury rats, nestin expression was observed in the cytoplasm of ependymal cells. One day immediately following surgery, ependymal cells were BrdU-labeled. The number of BrdU-positive cells increased at 3 days, reached a peak at 7 days, and gradually reduced thereafter. The ependyma developed ti'om a constitutive monolayer cells to a multi-layer cell complex. Some BrdU/Nestin double-positive ependymal cells migrated out from the ependyma. TUNEL-positive cells were also detected in the ependyma in the central region, as well as ischemic regions of the injured spinal cord. In addition, TUNEL-positive cells were visible in the ependyma. No TUNEL-positive ependymal cells were observed in the normal spinal cord

  16. The MRI manifestations of intramedullary germinoma of the spinal cord: a case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Feng Chen; Tao Liu; Jianjun Li; Chuanzi Li

    2009-01-01

    Primary germinoma in the spinal cord is very rare. Preoperative diagnosis is important because germinomas are sensitive to radiation therapy and chemotherapy so that treatment trends and prognosis differ from other intramedullary spinal cord tumors. Preoperative radiologic diagnosis is very difficult because of this rarity. In this case a 22-year-old woman with a 4-month history of gradual numbness and weakness of both lower extremities was found to have a solid tumor in the thoracic cord between the T9 and T11 vertebral bodies. No other neoplastic lesion was found inside or outside the central nervous system. The patient underwent surgery, and the intramedullary lesion was almost totally resected. Serum HCG was elevated postoperatively without pregnancy. The pathological diagnosis was conclusively that of a germinoma. The previous 23 cases of primary spinal cord germinoma were reviewed for comparison.

  17. Advances in regenerative therapies for spinal cord injury:a biomaterials approach

    Institute of Scientific and Technical Information of China (English)

    Magdalini Tsintou; Kyriakos Dalamagkas; Alexander Marcus Seifalian

    2015-01-01

    Spinal cord injury results in the permanent loss of function, causing enormous personal, social and economic problems. Even though neural regeneration has been proven to be a natural mech-anism, central nervous system repair mechanisms are ineffective due to the imbalance of the inhibitory and excitatory factors implicated in neuroregeneration. Therefore, there is growing re-search interest on discovering a novel therapeutic strategy for effective spinal cord injury repair. To this direction, cell-based delivery strategies, biomolecule delivery strategies as well as scaf-fold-based therapeutic strategies have been developed with a tendency to seek for the answer to a combinatorial approach of all the above. Here we review the recent advances on regenerative/neural engineering therapies for spinal cord injury, aiming at providing an insight to the most promising repair strategies, in order to facilitate future research conduction.

  18. Advances in regenerative therapies for spinal cord injury: a biomaterials approach

    Directory of Open Access Journals (Sweden)

    Magdalini Tsintou

    2015-01-01

    Full Text Available Spinal cord injury results in the permanent loss of function, causing enormous personal, social and economic problems. Even though neural regeneration has been proven to be a natural mechanism, central nervous system repair mechanisms are ineffective due to the imbalance of the inhibitory and excitatory factors implicated in neuroregeneration. Therefore, there is growing research interest on discovering a novel therapeutic strategy for effective spinal cord injury repair. To this direction, cell-based delivery strategies, biomolecule delivery strategies as well as scaffold-based therapeutic strategies have been developed with a tendency to seek for the answer to a combinatorial approach of all the above. Here we review the recent advances on regenerative/neural engineering therapies for spinal cord injury, aiming at providing an insight to the most promising repair strategies, in order to facilitate future research conduction.

  19. A clinicopathological analysis of unusual extraventricular neurocytoma of spinal cord

    Directory of Open Access Journals (Sweden)

    LI Zhi

    2013-08-01

    attach the dura mater and invade the surrounding tissues. The tumor was removed totally. Microscopic examination showed that a moderate cellular tumor was composed of uniform cells and arranged in sheets. The tumor cells had round nuclei, finely speckled chromatin and clear cytoplasm. Some cells had perinuclear haloes resembling oligodendroglioma. In some areas, the tumor cells with elongated cytoplasmic processes arranged radially around the blood vessels with myxoid degeneration, forming a structure of "perivascular pseudorosette", resembling the ependymoma. Mitotic activity and necrotic area were not observed. The tumor cells were strongly immunopositive for Syn, focally positive for NSE, S-100 and Oligo-2, but negative for Vim, CK, EMA, NeuN and GFAP. Ki-67 index was less than 1% in our case. Based on clinical presentation and histological findings, a final histological diagnosis of primary EVN in spinal cord was made according to the criteria of WHO classification. The patient has not received radiotherapy and attended follow-up for 6 months, without any neurological deficit or signs of recurrence. Conclusion Spinal EVN is extremely rare and there are no more than 20 bona fide cases reported previously all over the world. It might originate from neuronal precursor cells surrounding the region of central canal in fetal life. The definite diagnosis of this tumor should be made under the microscopical examination because the preoperatively radiological appearance of the tumor does not differ from other tumors occurring in spinal cord. Although good prognosis obtained from gross total resection in most of reported patients with this tumor, adjuvant radiotherapy is recommend for those tumors with atypical histological features to avoid the tumor recurrence. Due to the rarity of its site, the tumor can easily be confused with other tumors of spinal cord with clear cells features and ependymoma-like structure. The strictly differential diagnosis should be made when the

  20. Muscle fibers in the central nervous system of nemerteans: spatial organization and functional role.

    Science.gov (United States)

    Petrov, A A; Zaitseva, O V

    2012-08-01

    The system of muscle fibers associated with the brain and lateral nerve cords is present in all major groups of enoplan nemerteans. Unfortunately, very little is known about the functional role and spatial arrangement of these muscles of the central nervous system. This article examines the architecture of the musculature of the central nervous system in two species of monostiliferous nemerteans (Emplectonema gracile and Tetrastemma cf. candidum) using phalloidin staining and confocal microscopy. The article also briefly discusses the body-wall musculature and the muscles of the cephalic region. In both species, the lateral nerve cords possess two pairs of cardinal muscles that run the length of the nerve cords and pass through the ventral cerebral ganglia. A system of peripheral muscles forms a meshwork around the lateral nerve cords in E. gracile. The actin-rich processes that ramify within the nerve cords in E. gracile (transverse fibers) might represent a separate population of glia-like cells or sarcoplasmic projections of the peripheral muscles of the central nervous system. The lateral nerve cords in T. cf. candidum lack peripheral muscles but have muscles similar in their position and orientation to the transverse fibers. The musculature of the central nervous system is hypothesized to function as a support system for the lateral nerve cords and brain, preventing rupturing and herniation of the nervous tissue during locomotion. The occurrence of muscles of the central nervous system in nemerteans and other groups and their possible relevance in taxonomy are discussed.

  1. The syndrome of central hypothyroidism and macroorchidism: IGSF1 controls TRHR and FSHB expression by differential modulation of pituitary TGFβ and Activin pathways

    Science.gov (United States)

    García, Marta; Barrio, Raquel; García-Lavandeira, Montserrat; Garcia-Rendueles, Angela R.; Escudero, Adela; Díaz-Rodríguez, Esther; Gorbenko Del Blanco, Darya; Fernández, Ana; de Rijke, Yolanda B.; Vallespín, Elena; Nevado, Julián; Lapunzina, Pablo; Matre, Vilborg; Hinkle, Patricia M.; Hokken-Koelega, Anita C. S.; de Miguel, María P.; Cameselle-Teijeiro, José Manuel; Nistal, Manuel; Alvarez, Clara V.; Moreno, José C.

    2017-01-01

    IGSF1 (Immunoglobulin Superfamily 1) gene defects cause central hypothyroidism and macroorchidism. However, the pathogenic mechanisms of the disease remain unclear. Based on a patient with a full deletion of IGSF1 clinically followed from neonate to adulthood, we investigated a common pituitary origin for hypothyroidism and macroorchidism, and the role of IGSF1 as regulator of pituitary hormone secretion. The patient showed congenital central hypothyroidism with reduced TSH biopotency, over-secretion of FSH at neonatal minipuberty and macroorchidism from 3 years of age. His markedly elevated inhibin B was unable to inhibit FSH secretion, indicating a status of pituitary inhibin B resistance. We show here that IGSF1 is expressed both in thyrotropes and gonadotropes of the pituitary and in Leydig and germ cells in the testes, but at very low levels in Sertoli cells. Furthermore, IGSF1 stimulates transcription of the thyrotropin-releasing hormone receptor (TRHR) by negative modulation of the TGFβ1-Smad signaling pathway, and enhances the synthesis and biopotency of TSH, the hormone secreted by thyrotropes. By contrast, IGSF1 strongly down-regulates the activin-Smad pathway, leading to reduced expression of FSHB, the hormone secreted by gonadotropes. In conclusion, two relevant molecular mechanisms linked to central hypothyroidism and macroorchidism in IGSF1 deficiency are identified, revealing IGSF1 as an important regulator of TGFβ/Activin pathways in the pituitary. PMID:28262687

  2. Extensive neuronal differentiation of human neural stem cell grafts in adult rat spinal cord.

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    Jun Yan

    2007-02-01

    Full Text Available BACKGROUND: Effective treatments for degenerative and traumatic diseases of the nervous system are not currently available. The support or replacement of injured neurons with neural grafts, already an established approach in experimental therapeutics, has been recently invigorated with the addition of neural and embryonic stem-derived precursors as inexhaustible, self-propagating alternatives to fetal tissues. The adult spinal cord, i.e., the site of common devastating injuries and motor neuron disease, has been an especially challenging target for stem cell therapies. In most cases, neural stem cell (NSC transplants have shown either poor differentiation or a preferential choice of glial lineages. METHODS AND FINDINGS: In the present investigation, we grafted NSCs from human fetal spinal cord grown in monolayer into the lumbar cord of normal or injured adult nude rats and observed large-scale differentiation of these cells into neurons that formed axons and synapses and established extensive contacts with host motor neurons. Spinal cord microenvironment appeared to influence fate choice, with centrally located cells taking on a predominant neuronal path, and cells located under the pia membrane persisting as NSCs or presenting with astrocytic phenotypes. Slightly fewer than one-tenth of grafted neurons differentiated into oligodendrocytes. The presence of lesions increased the frequency of astrocytic phenotypes in the white matter. CONCLUSIONS: NSC grafts can show substantial neuronal differentiation in the normal and injured adult spinal cord with good potential of integration into host neural circuits. In view of recent similar findings from other laboratories, the extent of neuronal differentiation observed here disputes the notion of a spinal cord that is constitutively unfavorable to neuronal repair. Restoration of spinal cord circuitry in traumatic and degenerative diseases may be more realistic than previously thought, although major

  3. Pain modulation by nitric oxide in the spinal cord.

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    Marco Aurelio M Freire

    2009-09-01

    Full Text Available Nitric oxide (NO is a versatile messenger molecule first associated with endothelial relaxing effects. In the central nervous system (CNS, NO synthesis is primarily triggered by activation of N-methyl-D-aspartate (NMDA receptors and has a Janus face, with both beneficial and harmful properties, depending on concentration and the identity of its synthetic enzyme isoform. There are three isoforms of the NO synthesizing enzyme nitric oxide synthase (NOS: neuronal (nNOS, endothelial (eNOS, and inducible nitric oxide synthase (iNOS, each one involved with specific events in the brain. In CNS, nNOS is involved with modulation of synaptic transmission through long-term potentiation in several regions, including nociceptive circuits in the spinal cord. Here, we review the role played by NO on central pain sensitization.

  4. Genetics Home Reference: Williams syndrome

    Science.gov (United States)

    ... 2006 Jan 31. Citation on PubMed or Free article on PubMed Central Eckert MA, Galaburda AM, Mills DL, Bellugi U, Korenberg JR, Reiss AL. The neurobiology of Williams syndrome: cascading influences of visual system ...

  5. Gorlin syndrome: A case report

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    Patil K

    2005-01-01

    Full Text Available Gorlin syndrome is an autosomal dominant inherited condition that exhibits high penetrance and variable expressivity. It is characterized mainly by Basal cell carcinomas, Odontogenic keratocysts and skeletal anomalies. However, medical literature documents both common and lesser known manifestations of the disorder involving the skin, central nervous system, skeletal system etc. Diagnosis of the syndrome in childhood is basically through oral abnormalities. A case of Gorlin syndrome has been reported here, with review of literature.

  6. A Clinical Perspective and Definition of Spinal Cord Injury.

    Science.gov (United States)

    Kretzer, Ryan M

    2016-04-01

    Spinal cord injury (SCI) can be complete or incomplete. The level of injury in SCI is defined as the most caudal segment with motor function rated at greater than or equal to 3/5, with pain and temperature preserved. The standard neurological classification of SCI provided by the American Spinal Injury Association (ASIA) assigns grades from ASIA A (complete SCI) through ASIA E (normal sensory/motor), with B, C, and D representing varying degrees of injury between these extremes. The most common causes of SCI include trauma (motor vehicle accidents, sports, violence, falls), degenerative spinal disease, vascular injury (anterior spinal artery syndrome, epidural hematoma), tumor, infection (epidural abscess), and demyelinating processes (). (SDC Figure 1, http://links.lww.com/BRS/B91)(Figure is included in full-text article.).

  7. Dental approach to craniofacial syndromes

    DEFF Research Database (Denmark)

    Kjær, Inger

    2012-01-01

    is essential for insight into craniofacial syndromes. The dentition, thus, becomes central in diagnostics and evaluation of the pathogenesis. Developmental fields can explore and advance the concept of dental approaches to craniofacial syndromes. Discussion. As deviations in teeth persist and do not reorganize...

  8. Robust GM/WM segmentation of the spinal cord with iterative non-local statistical fusion.

    Science.gov (United States)

    Asman, Andrew J; Smith, Seth A; Reich, Daniel S; Landman, Bennett A

    2013-01-01

    New magnetic resonance imaging (MRI) sequences are enabling clinical study of the in vivo spinal cord's internal structure. Yet, low contrast-to-noise ratio, artifacts, and imaging distortions have limited the applicability of tissue segmentation techniques pioneered elsewhere in the central nervous system. Recently, methods have been presented for cord/non-cord segmentation on MRI and the feasibility of gray matter/white matter tissue segmentation has been evaluated. To date, no automated algorithms have been presented. Herein, we present a non-local multi-atlas framework that robustly identifies the spinal cord and segments its internal structure with submillimetric accuracy. The proposed algorithm couples non-local fusion with a large number of slice-based atlases (as opposed to typical volumetric ones). To improve performance, the fusion process is interwoven with registration so that segmentation information guides registration and vice versa. We demonstrate statistically significant improvement over state-of-the-art benchmarks in a study of 67 patients. The primary contributions of this work are (1) innovation in non-volumetric atlas information, (2) advancement of label fusion theory to include iterative registration/segmentation, and (3) the first fully automated segmentation algorithm for spinal cord internal structure on MRI.

  9. Label-free imaging of rat spinal cords based on multiphoton microscopy

    Science.gov (United States)

    Liao, Chenxi; Wang, Zhenyu; Zhou, Linquan; Zhu, Xiaoqin; Liu, Wenge; Chen, Jianxin

    2016-10-01

    As an integral part of the central nervous system, the spinal cord is a communication cable between the body and the brain. It mainly contains neurons, glial cells, nerve fibers and fiber tracts. The recent development of the optical imaging technique allows high-resolution imaging of biological tissues with the great potential for non-invasively looking inside the body. In this work, we evaluate the imaging capacity of multiphoton microscopy (MPM) based on second harmonic generation (SHG) and two-photon excited fluorescence (TPEF) for the cells and extracellular matrix in the spinal cord at molecular level. Rat spinal cord tissues were sectioned and imaged by MPM to demonstrate that MPM is able to show the microstructure including white matter, gray matter, ventral horns, dorsal horns, and axons based on the distinct intrinsic sources in each region of spinal cord. In the high-resolution and high-contrast MPM images, the cell profile can be clearly identified as dark shadows caused by nuclei and encircled by cytoplasm. The nerve fibers in white matter region emitted both SHG and TPEF signals. The multiphoton microscopic imaging technique proves to be a fast and effective tool for label-free imaging spinal cord tissues, based on endogenous signals in biological tissue. It has the potential to extend this optical technique to clinical study, where the rapid and damage-free imaging is needed.

  10. Projections from the brain to the spinal cord in the mouse.

    Science.gov (United States)

    Liang, Huazheng; Paxinos, George; Watson, Charles

    2011-01-01

    The cells that project from the brain to the spinal cord have previously been mapped in a wide range of mammalian species, but have not been comprehensively studied in the mouse. We have mapped these cells in the mouse using retrograde tracing after large unilateral Fluoro-Gold (FG) and horseradish peroxidase (HRP) injections in the C1 and C2 spinal cord segments. We have identified over 30 cell groups that project to the spinal cord, and have confirmed that the pattern of major projections from the cortex, diencephalon, midbrain, and hindbrain in the mouse is typically mammalian, and very similar to that found in the rat. However, we report two novel findings: we found labeled neurons in the precuneiform area (an area which has been associated with the midbrain locomotor center in other species), and the epirubrospinal nucleus. We also found labeled cells in the medial division of central nucleus of the amygdala in a small number of cases. Our findings should be of value to researchers engaged in evaluating the impact of spinal cord injury and other spinal cord pathologies on the centers which give rise to descending pathways.

  11. The corticospinal tract lesion of amyotrophic lateral sclerosis. Magnetic resonance imaging of the spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Terao, Shin-ichi; Sobue, Gen; Mitsuma, Terunori (Aichi Medical Univ., Nagakute (Japan)); Yasuda, Takeshi; Kachi, Teruhiko

    1994-09-01

    Magnetic resonance imaging by gradient echo method demonstrated lesions of the lateral corticospinal tract at cervical cord levels in three ALS patients. Patient 1 was a 43-year-old woman with common from of ALS. She developed right-side predominant pyramidal signs, and right-side predominant prolongation of central motor conduction time. MRI showed hypersignal intensity areas in the dorsal region of the lateral column at the 4th and 5th cervical segments with right-side predominacy. Patient 2 was a 65-year-old man with pseudopolyneuritic from of ALS, who showed lower motor neuron signs without a pyramidal sign. MRI of the 3rd and 4th cervical cord segments demonstrated bilateral hypersignal intensity areas in the dorsal part of the lateral column. Patient 3 was a 62-year-old man with common form of ALS, who showed marked bilateral pyramidal signs with Babinski's sign. MRI of the 5th cervical spinal cord segment demonstrated bilateral hypersignal intensity areas in the dorsolateral column. MR images of the spinal cord thus obtained corresponded well to the postmortem confirmed degeneration of the spinal corticospinal tract. MRI of the spinal cord performed by gradient echo method would provide additional information on the upper motor neuron involvement in ALS. (author).

  12. Therapeutic approaches for spinal cord injury

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    Alexandre Fogaça Cristante

    2012-10-01

    Full Text Available This study reviews the literature concerning possible therapeutic approaches for spinal cord injury. Spinal cord injury is a disabling and irreversible condition that has high economic and social costs. There are both primary and secondary mechanisms of damage to the spinal cord. The primary lesion is the mechanical injury itself. The secondary lesion results from one or more biochemical and cellular processes that are triggered by the primary lesion. The frustration of health professionals in treating a severe spinal cord injury was described in 1700 BC in an Egyptian surgical papyrus that was translated by Edwin Smith; the papyrus reported spinal fractures as a ''disease that should not be treated.'' Over the last biological or pharmacological treatment method. Science is unraveling the mechanisms of cell protection and neuroregeneration, but clinically, we only provide supportive care for patients with spinal cord injuries. By combining these treatments, researchers attempt to enhance the functional recovery of patients with spinal cord injuries. Advances in the last decade have allowed us to encourage the development of experimental studies in the field of spinal cord regeneration. The combination of several therapeutic strategies should, at minimum, allow for partial functional recoveries for these patients, which could improve their quality of life.

  13. Atypical presentations of Wolframs syndrome

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    S Saran

    2012-01-01

    Full Text Available Background: Wolfram syndrome is a rare hereditary or sporadic neurodegenerative disorder also known as DIDMOAD. The classically described presentation is of insulin-dependent diabetes, followed by optic atrophy, central diabetes insipidus, and sensory neural deafness. Also included are less well-described presentations of Wolframs syndrome. We here present three cases of atypical presentation of this syndrome. Case 1: A 15-year-old boy with insulin-dependent diabetes was presented for evaluation of depressive symptoms associated with suicidal tendency. Neuropsychiatric manifestations are described with Wolframs syndrome, and wolframin gene, in recessive inheritance, is associated with psychiatric illnesses without other manifestations of Wolframs syndrome. Case 2: A 17-year-old diabetic boy on insulin with good control of blood sugar presented for evaluation of delayed puberty. Central hypogonadism and other anterior pituitary hormone dysfunctions are the less publicized hormone dysfunctions in Wolframs syndrome. Case 3: A 23-year-old female who was on insulin for diabetes for the past 14 years, got admitted for evaluation of sudden loss of vision. This patient had developed a vitreous hemorrhage and, on evaluation, was found to have optic atrophy, sensory neural hearing loss, and diabetes insipidus, and presented differently from the gradual loss of vision described in Wolframs syndrome. Conclusion: Wolframs syndrome being a multisystem degenerative disorder can have myriad other manifestations than the classically described features. Neuropsychiatric manifestations, depression with suicidal risk, central hypogonadism, and secondary adrenal insufficiency are among the less well-described manifestations of this syndrome.

  14. Distensión abdominal y edemas por quiste del cordón espermático Abdomina distention and edema due to spermatic cord cyst

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    Guillermo A. Keller

    2006-04-01

    Full Text Available La distensión abdominal es un síntoma común, siendo en general la presentación inicial de enfermedades sistémicas o desórdenes gastrointestinales. Otras causas son infrecuentes. Los quistes del cordón espermático son poco frecuentes, pero aún más su ubicación intraabdominal, su tamaño habitual es insuficiente para producir distensión. El paciente presentado en este caso es un varón con criptorquidia bilateral admitido por distensión abdominal, interpretada inicialmente como síndrome ascítico edematoso. La ecografía interpretó la distensión como ascitis tabicada, y la tomografía computada como debida a un gran quiste. En la exploración quirúrgica se diagnosticó un quiste gigante del cordón espermático de ubicación abdominal.Abdominal distention is a frequent symptom, being often the initial presentation of systemic diseases or gastrointestinal disorders. Other causes are uncommon. Spermatic cord cysts are infrequent, abdominal location is even rarer, and the size of the cysts is usually not enough to produce abdominal distention. In our case a man with bilateral cryptorchidism was admitted with abdominal distention and edema of the lower extremities initially interpreted as ascitic-edematous syndrome. Ultrasonography interpreted abdominal distention as septate ascites, computed tomography as a giant cyst. Exploratory surgery showed a giant spermatic cord cyst in the left spermatic cord.

  15. [Von Hippel-Lindau syndrome].

    Science.gov (United States)

    Reich, H; Hollwich, F

    1984-06-01

    The von Hippel-Lindau syndrome is an autosomal dominant condition that comprises, apart from angiomas of the retina, the cerebellum, the spinal cord, and the cerebrum, also cystic and blastomatous dysplasias resulting from maldevelopment, namely cystic kidney and pancreas, hypernephroma, and pheochromocytoma. Early observers of the syndrome were the English neurologist John Hughlings Jackson (1872) and the German ophthalmologist Hugo Magnus (1874). The typical association of angiomas of the retina with the cerebellum was first described in 1905 by the Prague ophthalmologist Wilhelm Czermak, long before Lindau (1926). The fact that hypernephromas and pheochromocytomas may form parts of it characterizes the syndrome as a polyneoplastic hereditary disease and the sufferers as members of families at risk. Since the ophthalmologist is often the first to recognize this disease by direct inspection of the fundi, he is responsible for ensuring proper medical care for the affected person and his or her entire family.

  16. Human umbilical cord mesenchymal stem cells and the treatment of spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    CAO Fu-jiang; FENG Shi-qing

    2009-01-01

    Objective To review the recent studies about human umbilical cord mesenchymal stem cells (hUCMSCs) and advances in the treatment of spinal cord injury, Data sources Published articles (1983-2007) about hUCMSCs and spinal cord injury were selected using Medline. Study selection Articles selected were relevant to development of mesenchymal stem cells (MSCs) for transplantation in spinal cord injury therapy. Of 258 originally identifiied arises 51 were selected that specifically addressed the stated purpose. Results Recent work has revealed that hUCMSCs share most of the characteristics with MSCs derived from bone marrow and are more appropriate to transplantation for cell based therapies. Conclusions Human umbilical cord could be regarded as a source of MSCs for experimental and clinical needs. In addition, as a peculiar source of stem cells, hUCMSCs may play an important role in the treatment of spinal cord injury.

  17. Central nervous system abnormalities in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome Anormalidades do sistema nervoso central em pacientes com espectro óculo-aurículo-vertebral (síndrome de Goldenhar

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    Rafael Fabiano Machado Rosa

    2010-02-01

    Full Text Available OBJECTIVE: To describe the central nervous system (CNS alterations present in a sample of oculo-auriculo-vertebral spectrum (OAVS patients, trying to correlate them with other clinical features. METHOD: Seventeen patients with diagnosis of OAVS were evaluated. All presented radiological evaluation of the CNS, normal GTG-Banding karyotype and clinical features involving at least two from the four following areas: oro-cranio-facial, ocular, auricular and vertebral. RESULTS: CNS alterations were verified in eight from seventeen patients (47%. Diffuse cerebral hypoplasia, dilated lateral cerebral ventricles (asymptomatic hydrocephalus, corpus callosum dysgenesis and frontal hypodensities were the most frequent abnormalities. Presence of ophthalmologic abnormalities was the only clinical association observed, being significantly more frequent among patients with cerebral alterations (63% versus 11%. CONCLUSION: CNS abnormalities are frequent in patients with OAVS, especially in carriers of ophthalmologic alterations. However, the absence of detectable cerebral abnormalities did not exclude the possibility that these subjects will subsequently present neurological symptoms.OBJETIVO: Descrever as alterações do sistema nervoso central (SNC presentes em uma amostra de pacientes com espectro óculo-aurículo-vertebral (EOAV, tentando correlacioná-las com os demais achados clínicos. MÉTODO: Foram avaliados dezessete pacientes com diagnóstico de EOAV. Todos apresentavam avaliação radiológica do SNC, cariótipo por bandas GTG normal e achados clínicos em pelo menos duas das quatro das seguintes áreas: oro-crânio-facial, ocular, auricular e vertebral. RESULTADOS: Alterações do SNC foram verificadas em oito dos dezessete pacientes (47%. Hipoplasia cerebral difusa, dilatação dos ventrículos cerebrais laterais (hidrocefalia assintomática, disgenesia do corpo caloso e hipondesidades frontais foram as anormalidades mais frequentes. A presença de

  18. Vocal cord paralysis caused by stingray.

    Science.gov (United States)

    Kwon, Oh Jin; Park, Jung Je; Kim, Jin Pyeong; Woo, Seung Hoon

    2013-11-01

    Foreign bodies in the oral cavity and pharynx are commonly encountered in the emergency room and outpatient departments, and the most frequently observed of these foreign bodies are fish bones. Among the possible complications resulting from a pharyngeal foreign body, vocal cord fixation is extremely rare, with only three cases previously reported in the English literature. The mechanisms of vocal cord fixation can be classified into mechanical articular fixation, direct injury of the recurrent laryngeal nerve, or recurrent laryngeal nerve paralysis secondary to inflammation. The case discussed here is different from previous cases. We report a rare case of vocal cord paralysis caused by the venom of a stingray tail in the hypopharynx.

  19. Nanomedicine for treating spinal cord injury

    Science.gov (United States)

    Tyler, Jacqueline Y.; Xu, Xiao-Ming; Cheng, Ji-Xin

    2013-09-01

    Spinal cord injury results in significant mortality and morbidity, lifestyle changes, and difficult rehabilitation. Treatment of spinal cord injury is challenging because the spinal cord is both complex to treat acutely and difficult to regenerate. Nanomaterials can be used to provide effective treatments; their unique properties can facilitate drug delivery to the injury site, enact as neuroprotective agents, or provide platforms to stimulate regrowth of damaged tissues. We review recent uses of nanomaterials including nanowires, micelles, nanoparticles, liposomes, and carbon-based nanomaterials for neuroprotection in the acute phase. We also review the design and neural regenerative application of electrospun scaffolds, conduits, and self-assembling peptide scaffolds.

  20. Vocal cord paralysis in a fighter pilot.

    Science.gov (United States)

    Maturo, Stephen; Brennan, Joseph

    2006-01-01

    We present in this case report the return to flying duty of a pilot with vocal cord paralysis secondary to removal of a thymoma. We discuss the importance of glottic function as it pertains to the unique aviation environment. We also discuss the anatomy and physiology of the glottis, the evaluation for vocal cord paralysis, and surgical approaches for paralyzed vocal cords. Although the incidence of recurrent laryngeal nerve paralysis is low in the military aviation community, it is important to recognize that its sequelae can be managed so that the aviator may return to flight duties.

  1. Teaching nonlinear dynamics through elastic cords

    Energy Technology Data Exchange (ETDEWEB)

    Chacon, R; Galan, C A; Sanchez-Bajo, F, E-mail: rchacon@unex.e [Departamento de Fisica Aplicada, Escuela de IngenierIas Industriales, Universidad de Extremadura, Apartado Postal 382, E-06071 Badajoz (Spain)

    2011-01-15

    We experimentally studied the restoring force of a length of stretched elastic cord. A simple analytical expression for the restoring force was found to fit all the experimental results for different elastic materials. Remarkably, this analytical expression depends upon an elastic-cord characteristic parameter which exhibits two limiting values corresponding to two nonlinear springs with different Hooke's elastic constants. Additionally, the simplest model of elastic cord dynamics is capable of exhibiting a great diversity of nonlinear phenomena, including bifurcations and chaos, thus providing a suitable alternative model system for discussing the basic essentials of nonlinear dynamics in the context of intermediate physics courses at university level.

  2. New onset neuromyelitis optica in a young Nigerian woman with possible antiphospholipid syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Komolafe Morenikeji A

    2008-11-01

    Full Text Available Abstract Introduction Devic's neuromyelitis optica is an inflammatory demyelinating disease that targets the optic nerves and spinal cord. It has a worldwide distribution and distinctive features that distinguish it from multiple sclerosis. There has been no previous report of neuromyelitis optica from our practice environment, and we are not aware of any case associated with antiphospholipid syndrome in an African person. Case presentation We report the case of a 28-year-old Nigerian woman who presented with neck pain, paroxysmal tonic spasms, a positive Lhermitte's sign and spastic quadriplegia. She later developed bilateral optic neuritis and had clinical and biochemical features of antiphospholipid syndrome. Her initial magnetic resonance imaging showed a central linear hyperintense focus in the intramedullary portion of C2 to C4. Repeat magnetic resonance imaging after treatment revealed resolution of the signal intensity noticed earlier. Conclusion Neuromyelitis optica should be considered in the differential diagnoses of acute myelopathy in Africans. We also highlight the unusual association with antiphospholipid syndrome. Physicians should screen such patients for autoimmune disorders.

  3. Muscle after spinal cord injury

    DEFF Research Database (Denmark)

    Biering-Sørensen, Bo; Kristensen, Ida Bruun; Kjaer, Michael;

    2009-01-01

    The morphological and contractile changes of muscles below the level of the lesion after spinal cord injury (SCI) are dramatic. In humans with SCI, a fiber-type transformation away from type I begins 4-7 months post-SCI and reaches a new steady state with predominantly fast glycolytic IIX fibers...... years after the injury. There is a progressive drop in the proportion of slow myosin heavy chain (MHC) isoform fibers and a rise in the proportion of fibers that coexpress both the fast and slow MHC isoforms. The oxidative enzymatic activity starts to decline after the first few months post-SCI. Muscles...... from individuals with chronic SCI show less resistance to fatigue, and the speed-related contractile properties change, becoming faster. These findings are also present in animals. Future studies should longitudinally examine changes in muscles from early SCI until steady state is reached in order...

  4. Thermography imaging during static and controlled thermoregulation in complex regional pain syndrome type 1: diagnostic value and involvement of the central sympathetic system

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    Westra Mirjam

    2006-05-01

    Full Text Available Abstract Background Complex Regional Pain Syndrome type 1 (CRPS1 is a clinical diagnosis based on criteria describing symptoms of the disease. The main aim of the present study was to compare the sensitivity and specificity of calculation methods used to assess thermographic images (infrared imaging obtained during temperature provocation. The secondary objective was to obtain information about the involvement of the sympathetic system in CRPS1. Methods We studied 12 patients in whom CRPS1 was diagnosed according to the criteria of Bruehl. High and low whole body cooling and warming induced and reduced sympathetic vasoconstrictor activity. The degree of vasoconstrictor activity in both hands was monitored using a videothermograph. The sensitivity and specificity of the calculation methods used to assess the thermographic images were calculated. Results The temperature difference between the hands in the CRPS patients increases significantly when the sympathetic system is provoked. At both the maximum and minimum vasoconstriction no significant differences were found in fingertip temperatures between both hands. Conclusion The majority of CRPS1 patients do not show maximal obtainable temperature differences between the involved and contralateral extremity at room temperature (static measurement. During cold and warm temperature challenges this temperature difference increases significantly. As a result a higher sensitivity and specificity could be achieved in the diagnosis of CRPS1. These findings suggest that the sympathetic efferent system is involved in CRPS1.

  5. The central role of vascular extracellular matrix and basement membrane remodeling in metabolic syndrome and type 2 diabetes: the matrix preloaded

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    Tyagi Suresh C

    2005-06-01

    Full Text Available Abstract The vascular endothelial basement membrane and extra cellular matrix is a compilation of different macromolecules organized by physical entanglements, opposing ionic charges, chemical covalent bonding, and cross-linking into a biomechanically active polymer. These matrices provide a gel-like form and scaffolding structure with regional tensile strength provided by collagens, elasticity by elastins, adhesiveness by structural glycoproteins, compressibility by proteoglycans – hyaluronans, and communicability by a family of integrins, which exchanges information between cells and between cells and the extracellular matrix of vascular tissues. Each component of the extracellular matrix and specifically the capillary basement membrane possesses unique structural properties and interactions with one another, which determine the separate and combined roles in the multiple diabetic complications or diabetic opathies. Metabolic syndrome, prediabetes, type 2 diabetes mellitus, and their parallel companion (atheroscleropathy are associated with multiple metabolic toxicities and chronic injurious stimuli. The adaptable quality of a matrix or form genetically preloaded with the necessary information to communicate and respond to an ever-changing environment, which supports the interstitium, capillary and arterial vessel wall is individually examined.

  6. Isolation of mesenchymal stem cells from equine umbilical cord blood

    OpenAIRE

    Thomsen Preben D; Heerkens Tammy; Koch Thomas G; Betts Dean H

    2007-01-01

    Background: There are no published studies on stem cells from equine cord blood although commercial storage of equine cord blood for future autologous stem cell transplantations is available. Mesenchymal stem cells (MSC) have been isolated from fresh umbilical cord blood of humans collected non-invasively at the time of birth and from sheep cord blood collected invasively by a surgical intrauterine approach. Mesenchymal stem cells isolation percentage from frozen-thawed human cord blood is lo...

  7. Stem cells in the adult rat spinal cord: plasticity after injury and treadmill training exercise.

    Science.gov (United States)

    Foret, Ariane; Quertainmont, Renaud; Botman, Olivier; Bouhy, Delphine; Amabili, Philippe; Brook, Gary; Schoenen, Jean; Franzen, Rachelle

    2010-02-01

    Ependymal cells located around the central canal of the adult spinal cord are considered as a source of neural stem cells (NSCs) and represent an interesting pool of endogenous stem cells for repair strategies. Physical exercise is known to increase ependymal cell proliferation, while improving functional recovery. In this work, we further characterized those endogenous NSCs within the normal and injured adult rat spinal cord and investigated the effects of treadmill training using immunohistochemical and behavioral studies. In uninjured untrained rats, Sox-2, a NSC marker, was detected in all ependymal cells of the central canal, and also scattered throughout the parenchyma of the spinal cord. Within the lesion, Sox-2 expression increased transiently, while the number of nestin-positive ependymal cells increased with a concomitant enhancement of proliferation, as indicated by the mitotic markers Ki67 and bromo-deoxyuridine. Exercise, which improved functional recovery and autonomous micturition, maintained nestin expression in both injured and uninjured spinal cords, with a positive correlation between locomotor recovery and the number of nestin-positive cells.

  8. Induced pluripotent stem cell-derived neural stem cell therapies for spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Corinne A Lee-Kubli; Paul Lu

    2015-01-01

    The greatest challenge to successful treatment of spinal cord injury is the limited regenerative capacity of the central nervous system and its inability to replace lost neurons and severed axons following injury. Neural stem cell grafts derived from fetal central nervous system tissue or embryonic stem cells have shown therapeutic promise by differentiation into neurons and glia that have the potential to form functional neuronal relays across injured spinal cord segments. However, implementation of fetal-derived or embryonic stem cell-derived neural stem cell ther-apies for patients with spinal cord injury raises ethical concerns. Induced pluripotent stem cells can be generated from adult somatic cells and differentiated into neural stem cells suitable for therapeutic use, thereby providing an ethical source of implantable cells that can be made in an autologous fashion to avoid problems of immune rejection. This review discusses the therapeutic potential of human induced pluripotent stem cell-derived neural stem cell transplantation for treatment of spinal cord injury, as well as addressing potential mechanisms, future perspectives and challenges.

  9. Effect of Adding McKenzie Syndrome, Centralization, Directional Preference, and Psychosocial Classification Variables to a Risk-Adjusted Model Predicting Functional Status Outcomes for Patients With Lumbar Impairments.

    Science.gov (United States)

    Werneke, Mark W; Edmond, Susan; Deutscher, Daniel; Ward, Jason; Grigsby, David; Young, Michelle; McGill, Troy; McClenahan, Brian; Weinberg, Jon; Davidow, Amy L

    2016-09-01

    Study Design Retrospective cohort. Background Patient-classification subgroupings may be important prognostic factors explaining outcomes. Objectives To determine effects of adding classification variables (McKenzie syndrome and pain patterns, including centralization and directional preference; Symptom Checklist Back Pain Prediction Model [SCL BPPM]; and the Fear-Avoidance Beliefs Questionnaire subscales of work and physical activity) to a baseline risk-adjusted model predicting functional status (FS) outcomes. Methods Consecutive patients completed a battery of questionnaires that gathered information on 11 risk-adjustment variables. Physical therapists trained in Mechanical Diagnosis and Therapy methods classified each patient by McKenzie syndromes and pain pattern. Functional status was assessed at discharge by patient-reported outcomes. Only patients with complete data were included. Risk of selection bias was assessed. Prediction of discharge FS was assessed using linear stepwise regression models, allowing 13 variables to enter the model. Significant variables were retained in subsequent models. Model power (R(2)) and beta coefficients for model variables were estimated. Results Two thousand sixty-six patients with lumbar impairments were evaluated. Of those, 994 (48%), 10 (variables to the baseline model did not result in significant increases in R(2). McKenzie syndrome or pain pattern explained 2.8% and 3.0% of the variance, respectively. When pain pattern and SCL BPPM were added simultaneously, overall model R(2) increased to 0.44. Although none of these increases in R(2) were significant, some classification variables were stronger predictors compared with some other variables included in the baseline model. Conclusion The small added prognostic capabilities identified when combining McKenzie or pain-pattern classifications with the SCL BPPM classification did not significantly improve prediction of FS outcomes in this study. Additional research is

  10. Clinical Features of Intradural Extramedullary Spinal Cord Metastases 
in Primary Lung Cancer

    Directory of Open Access Journals (Sweden)

    Yan XU

    2016-08-01

    Full Text Available Background and objective Intradural extramedullary spinal cord metastases in lung cancer is rare, and it leads to severe neurological damage. The aim of this study is to identify the clinical features of intradural extramedullary spinal cord metastases in primary lung cancer patients. Methods The 8 cases of lung cancer with intradural extramedullary metastases, who were hospitalized in Peking Union Medical College Hospital (PUMCH during May 2013 to May 2016, were enrolled in the retrospective study. Medical charts of the 8 patients were reviewed systematically. Results Intradural extramedullary spinal cord metastases was diagnosed in 7 cases with non-small cell lung cancer (NSCLC and 1 case with small cell lung cancer (SCLC. Cauda equina syndrome was the most common clinical manifestation. Malignant cells in cerebrospinal fluid were positive in all the 5 cases (100% who underwent lumbar puncture. Contrast-enhanced magnetic resonance imaging (MRI of spine manifested as diffuse abnormal enhancement of pial lining of spinal cordin 3 cases, intradural extramedullary nodules in 4 cases, and both of them in 1 case. Neurological symptoms were improved or stable in 4 cases who underwent targeted therapy and/or radiotherapy. The median overall survival was 5.8 months. Conclusion Intradural extramedullary spinal cord metastases can be diagnosed with caution according to its neurological symptoms and contrast-enhanced MRI presentation.Targeted therapy and/or radiotherapy may be effective for symptoms control.

  11. CB1 cannabinoid receptor enrichment in the ependymal region of the adult human spinal cord.

    Science.gov (United States)

    Paniagua-Torija, Beatriz; Arevalo-Martin, Angel; Ferrer, Isidro; Molina-Holgado, Eduardo; Garcia-Ovejero, Daniel

    2015-12-04

    Cannabinoids are involved in the regulation of neural stem cell biology and their receptors are expressed in the neurogenic niches of adult rodents. In the spinal cord of rats and mice, neural stem cells can be found in the ependymal region, surrounding the central canal, but there is evidence that this region is largely different in adult humans: lacks a patent canal and presents perivascular pseudorosettes, typically found in low grade ependymomas. Using Laser Capture Microdissection, Taqman gene expression assays and immunohistochemistry, we have studied the expression of endocannabinoid system components (receptors and enzymes) at the human spinal cord ependymal region. We observe that ependymal region is enriched in CB1 cannabinoid receptor, due to high CB1 expression in GFAP+ astrocytic domains. However, in human spinal cord levels that retain central canal patency we found ependymal cells with high CB1 expression, equivalent to the CB1(HIGH) cell subpopulation described in rodents. Our results support the existence of ependymal CB1(HIGH) cells across species, and may encourage further studies on this subpopulation, although only in cases when central canal is patent. In the adult human ependyma, which usually shows central canal absence, CB1 may play a different role by modulating astrocyte functions.

  12. Morphological aspects of buffaloes (Bubalus bubalis umbilical cord Aspectos Morfológicos do cordão umbilical de búfalos (Bubalus bubalis

    Directory of Open Access Journals (Sweden)

    Guilherme J. Ferreira

    2009-10-01

    Full Text Available Buffalo is an important livestock resource, with a great participation in agricultural systems, providing milk, meat, and work power. Umbilical cord is responsible for maternal-fetal nutrients exchange during pregnancy, and its alterations can compromise the fetal development. We investigated ten pregnant uteruses collected from cross-bread buffaloes in different stages of gestation. Pregnancy and fetal age was determined by measuring the apex sacral length and development period was calculated by previously published formula. Umbilical cords were measured for length determination. Umbilical cord vascular net and anastomosis were observed by injection of Neoprene latex. Histological sections of the umbilical cord were studied after stain with HE, picrossirius, toluidine blue, orceine, and PAS reaction. Buffaloes' umbilical cord was formed by two central arteries, an allantois duct and two peripheral veins. The artery wall was composed by large quantity of collagen, elastic fibers, fibroblasts and large number of vasa vasorum. The allantois duct was located between the arteries and presented a great number of small nourishing vessels. Small nourishing vessels should be carefully considered to avoid to be mistaken to the arterials and veins vasa vasorum. Medium length of umbilical cord from buffalos was 11.8cm (minimum of 6.8cm and maximum of 17.4cm.Búfalo é uma importante fonte de recurso nos rebanhos animais, apresentando uma grande participação na agropecuária, provendo leite, carne e força de trabalho. O Cordão umbilical é responsável pela troca de nutrientes materno-fetais durante a gestação, e suas alterações podem comprometer o desenvolvimento fetal. Nós investigamos dez úteros gravídicos de búfalos de raças cruzadas em fases diferentes de gestação. O período de gestação e a idade fetal foram determinados pelo comprimento ápice sacral, aplicando fórmulas previamente estabelecidas. Posteriormente mediu-se o

  13. Loss of microRNA-124 expression in neurons in the peri-lesion area in mice with spinal cord injury

    Institute of Scientific and Technical Information of China (English)

    Yu Zhao; Hui Zhang; Dan Zhang; Cai-yong Yu; Xiang-hui Zhao; Fang-fang Liu; Gan-lan Bian; Gong Ju; Jian Wang

    2015-01-01

    MicroRNA-124 (miR-124) is abundantly expressed in neurons in the mammalian central ner-vous system, and plays critical roles in the regulation of gene expression during embryonic neurogenesis and postnatal neural differentiation. However, the expression proifle of miR-124 after spinal cord injury and the underlying regulatory mechanisms are not well understood. In the present study, we examined the expression of miR-124 in mouse brain and spinal cord after spinal cord injury usingin situ hybridization. Furthermore, the expression of miR-124 was examined with quantitative RT-PCR at 1, 3 and 7 days after spinal cord injury. The miR-124 expression in neurons at the site of injury was evaluated by in situ hybridization combined with NeuN immunohistochemical staining. The miR-124 was mainly expressed in neurons through-out the brain and spinal cord. The expression of miR-124 in neurons significantly decreased within 7 days after spinal cord injury. Some of the neurons in the peri-lesion area were NeuN+/miR-124−. Moreover, the neurons distal to the peri-lesion site were NeuN+/miR-124+. These ifndings indicate that miR-124 expression in neurons is reduced after spinal cord injury, and may relfect the severity of spinal cord injury.

  14. Changes of blood flow, oxygen tension, action potential and vascular permeability induced by arterial ischemia or venous congestion on the spinal cord in canine model.

    Science.gov (United States)

    Kobayashi, Shigeru; Yoshizawa, Hidezo; Shimada, Seiichiro; Guerrero, Alexander Rodríguez; Miyachi, Masaya

    2013-01-01

    It is generally considered that the genesis of myelopathy associated with the degenerative conditions of the spine may result from both mechanical compression and circulatory disturbance. Many references about spinal cord tissue ischemic damage can be found in the literature, but not detailed studies about spinal cord microvasculature damage related to congestion or blood permeability. This study investigates the effect of ischemia and congestion on the spinal cord using an in vivo model. The aorta was clamped as an ischemia model of the spinal cord and the inferior vena cava was clamped as a congestion model at the 6th costal level for 30 min using forceps transpleurally. Measurements of blood flow, partial oxygen pressure, and conduction velocity in the spinal cord were repeated over a period of 1 h after release of clamping. Finally, we examined the status of blood-spinal cord barrier under fluorescence and transmission electron microscope. Immediately after clamping of the inferior vena cava, the central venous pressure increased by about four times. Blood flow, oxygen tension and action potential were more severely affected by the aorta clamping; but this ischemic model did not show any changes of blood permeability in the spinal cord. The intramedullar edema was more easily produced by venous congestion than by arterial ischemia. In conclusions, venous congestion may be a preceding and essential factor of circulatory disturbance in the compressed spinal cord inducing myelopathy.

  15. Ultrastructural features of the differentiating thyroid primordium in the sand lizard (Lacerta agilis L.) from the differentiation of the cellular cords to the formation of the follicular lumen.

    Science.gov (United States)

    Rupik, Weronika; Kowalska, Magdalena; Swadźba, Elwira; Maślak, Robert

    2016-04-01

    The differentiation of the thyroid primordium of lacertilian species is poorly understood. The present study reports on the ultrastructural analysis of the developing thyroid primordium in the sand lizard (Lacerta agilis) during the early stages of differentiation. The early thyroid primordium of sand lizard embryos was composed of cellular cords that contained single cells with a giant lipid droplet, which were eliminated by specific autophagy (lipophagy). The follicular lumens at the periphery of the primordium differentiated even before the division of the cellular cords. When the single cells within the cords started to die through paraptosis, the adjacent cells started to polarise and junctional complexes began to form around them. After polarisation and clearing up after the formation of the lumens, the cellular cords divided into definitive follicles. The cellular cords in the central part of the primordium started to differentiate later than those at the periphery. The cellular cords divided into presumptive follicles first and only later differentiated into definitive follicles. During this process, a population of centrally located cells was removed through apoptosis to form the lumen. Although the follicular lumen in sand lizard embryos is differentiated by cavitation similar to that in the grass snake, there were very important differences during the early stages of the differentiation of the cellular cords and the formation of the thyroid follicles.

  16. The high cost of motherhood: End-lactation syndrome in southern sea otters (Enhydra lutris nereis) on the central California, USA, coast

    Science.gov (United States)

    Chinn, Sarah S; Miller, Melissa A.; Tinker, M. Tim; Staedler, Michelle M.; Batac, Francesca I.; Dodd, Erin M.; Henkel, Laird A.

    2016-01-01

    Sea otters (Enhydra lutris) have exceptionally high energetic requirements, which nearly double during lactation and pup care. Thus, females are extremely vulnerable to caloric insufficiency. Despite a number of compensatory strategies, the metabolic challenge of reproduction culminates in numerous maternal deaths annually. Massive depletion of energy reserves results in a case presentation that we define as end-lactation syndrome (ELS), characterized by moderate to severe emaciation not attributable to a concurrent, independent disease process in females dying during late pup care or postweaning. We compiled detailed data for 108 adult female southern sea otters (Enhydra lutris nereis) examined postmortem that stranded in California, US, 2005–12, and assessed pathology, reproductive status, and the location and timing of stranding. We introduce simple, grossly apparent, standardized physical criteria to assess reproductive stage for female sea otters. We also describe ELS, examine associated risk factors, and highlight female life history strategies that likely optimize reproduction and survival. Our data suggest that females can reset both the timing and energetic demands of reproduction through fetal loss, pup abandonment, or early weaning as part of specific physiologic checkpoints during each reproductive cycle. Females appear to preload nutritionally during delayed implantation and gestation to increase fitness and reproductive success. We found that ELS was a major cause of death, affecting 56% of enrolled adult females. Peak ELS prevalence occurred in late spring, possibly reflecting the population trend toward fall/winter pupping. Increasing age and number of pregnancies were associated with a higher risk of ELS. Although the proportion of ELS females was highest in areas with dense sea otter populations, cases were recovered throughout the range, suggesting that death from ELS is associated with, but not solely caused by, population resource limitation.

  17. Prader-Willi Critical Region, a Non-Translated, Imprinted Central Regulator of Bone Mass: Possible Role in Skeletal Abnormalities in Prader-Willi Syndrome.

    Directory of Open Access Journals (Sweden)

    Ee-Cheng Khor

    Full Text Available Prader-Willi Syndrome (PWS, a maternally imprinted disorder and leading cause of obesity, is characterised by insatiable appetite, poor muscle development, cognitive impairment, endocrine disturbance, short stature and osteoporosis. A number of causative loci have been located within the imprinted Prader-Willi Critical Region (PWCR, including a set of small non-translated nucleolar RNA's (snoRNA. Recently, micro-deletions in humans identified the snoRNA Snord116 as a critical contributor to the development of PWS exhibiting many of the classical symptoms of PWS. Here we show that loss of the PWCR which includes Snord116 in mice leads to a reduced bone mass phenotype, similar to that observed in humans. Consistent with reduced stature in PWS, PWCR KO mice showed delayed skeletal development, with shorter femurs and vertebrae, reduced bone size and mass in both sexes. The reduction in bone mass in PWCR KO mice was associated with deficiencies in cortical bone volume and cortical mineral apposition rate, with no change in cancellous bone. Importantly, while the length difference was corrected in aged mice, consistent with continued growth in rodents, reduced cortical bone formation was still evident, indicating continued osteoblastic suppression by loss of PWCR expression in skeletally mature mice. Interestingly, deletion of this region included deletion of the exclusively brain expressed Snord116 cluster and resulted in an upregulation in expression of both NPY and POMC mRNA in the arcuate nucleus. Importantly, the selective deletion of the PWCR only in NPY expressing neurons replicated the bone phenotype of PWCR KO mice. Taken together, PWCR deletion in mice, and specifically in NPY neurons, recapitulates the short stature and low BMD and aspects of the hormonal imbalance of PWS individuals. Moreover, it demonstrates for the first time, that a region encoding non-translated RNAs, expressed solely within the brain, can regulate bone mass in health

  18. ALDH1A1 Deficiency in Gorlin Syndrome Suggests a Central Role for Retinoic Acid and ATM Deficits in Radiation Carcinogenesis.

    Science.gov (United States)

    Weber, Thomas J; Magnaldo, Thierry; Xiong, Yijia

    2014-09-11

    We hypothesize that aldehyde dehydrogenase 1A1 (ALDH1A1) deficiency will result in impaired ataxia-telangiectasia mutated (ATM) activation in a retinoic acid-sensitive fashion. Data supporting this hypothesis include (1) reduced ATM activation in irradiated primary dermal fibroblasts from ALDH1A1-deficient Gorlin syndrome patients (GDFs), relative to ALDH1A1-positive normal human dermal fibroblasts (NHDFs) and (2) increased ATM activation by X-radiation in GDFs pretreated with retinoic acid, however, the impact of donor variability on ATM activation in fibroblasts was not assessed and is a prudent consideration in future studies. Clonogenic survival of irradiated cells showed differential responses to retinoic acid as a function of treatment time. Long-term (5 Day) retinoic acid treatment functioned as a radiosensitizer and was associated with downregulation of ATM protein levels. Short-term (7 h) retinoic acid treatment showed a trend toward increased survival of irradiated cells and did not downregulate ATM protein levels. Using a newly developed IncubATR technology, which defines changes in bulk chemical bond patterns in live cells, we can discriminate between the NHDF and GDF phenotypes, but treatment of GDFs with retinoic acid does not induce reversion of bulk chemical bond patterns associated with GDFs toward the NHDF phenotype. Collectively, our preliminary investigation of the Gorlin phenotype has identified deficient ALDH1A1 expression associated with deficient ATM activation as a possible susceptibility factor that is consistent with the high incidence of spontaneous and radiation-induced carcinogenesis in these patients. The IncubATR technology exhibits sufficient sensitivity to detect phenotypic differences in live cells that may be relevant to radiation health effects.

  19. Spinal Cord Injury: Hope through Research

    Science.gov (United States)

    ... recent tetraplegia. Much as in the general population, cardiovascular disease (CVD) is a leading cause of death in persons with spinal cord injury. After the injury, the opportunity to actively exercise large muscles affected by paralysis is limited or ...

  20. The shape of telephone cord blisters

    Science.gov (United States)

    Ni, Yong; Yu, Senjiang; Jiang, Hongyuan; He, Linghui

    2017-01-01

    Formation of telephone cord blisters as a result of buckling delamination is widely observed in many compressed film-substrate systems. Here we report a universal morphological feature of such blisters characterized by their sequential sectional profiles exhibiting a butterfly shape using atomic force microscopy. Two kinds of buckle morphologies, light and heavy telephone cord blisters, are observed and differentiated by measurable geometrical parameters. Based on the Föppl-von Kármán plate theory, the observed three-dimensional features of the telephone cord blister are predicted by the proposed approximate analytical model and simulation. The latter further replicates growth and coalescence of the telephone cord into complex buckling delamination patterns observed in the experiment.

  1. Spinal cord injury without radiographic abnormality

    Directory of Open Access Journals (Sweden)

    Singh Anil

    2006-01-01

    Full Text Available Spinal cord injury without radiological abnormality is rare in adults. Below we present a case report of 20 yrs old male with isolated cervical cord injury, without accompanying vertebral dislocation or fracture involving the spinal canal rim. He fell down on plain and smooth ground while carrying 40 kg weight overhead and developed quadriparesis with difficulty in respiration. Plain radiographs of the neck revealed no fractures or dislocations. MRI showed bulky spinal cord and an abnormal hyper intense signal on the T2W image from C2 vertebral body level to C3/4 intervertebral disc level predominantly in the anterior aspect of the cord The patient was managed conservatively with head halter traction and invasive ventilatory support for the initial 7 days period in the ICU. In our patient recovery was good and most of the neurological deficit improved over 4 weeks with conservative management.

  2. Effects of the stimulation of colorectal distention on expression of P2X4 and P2X7 receptors in dorsal commissural nucleus and dorsal horn of sacral segment of spinal cord in rats with irritable bowel syndrome%结肠扩张刺激对肠易激综合征大鼠DCN核团及骶髓后角中P2X4及P2X7受体表达的影响

    Institute of Scientific and Technical Information of China (English)

    朱琳; 章鹏宇; 王景杰; 黄裕新

    2011-01-01

    目的 观察肠易激综合征(irritable bowel syndrome,IBS)致内脏高敏感化大鼠在结肠扩张刺激时大鼠腹直肌肌电变化,并观察骶髓后联合核(dorsal commissural nucleus,DCN)和脊髓后角中P2X4和P2X7受体表达的变化情况,为探讨IBS内脏敏化的神经机制提供理论依据.方法 以旋毛虫感染大鼠建立IBS大鼠模型,并以正常大鼠作为对照,实验共分4组:正常大鼠无刺激组,正常大鼠结肠扩张刺激组,IBS大鼠无刺激组,IBS大鼠结肠扩张刺激组.采用免疫组织荧光化学方法,将P2X4和P2X7受体分别标记,观察其在骶髓后角及DCN核团上的表达变化,并同步测定大鼠腹直肌肌电变化.结果 IBS结肠刺激组大鼠腹直肌肌电变化、大鼠骶髓后角及DCN核团中的P2X4和P2X7受体表达较正常大鼠对照组及IBS未刺激组均显著增强.结论 P2X4 和 P2X7受体可能是IBS致内脏敏感性增高的重要因素.%Objective To explore the expression of P2X4 and P2X7 receptors in dorsal commissural nucleus ( DCN ) and dorsal horn of sacral segment of spinal cord in rats with irritable bowel syndrome( IBS ) induced by the stiruulation of colorectal distention,and to provide a theoretical evidence in prevention and treatment of IBS. Methods The rats were gavaged with the Trichinella spiralis to establish the irritable bowel syndrome model,and then divided into 2 groups :IBS without colon distension group and IBS with colon distension group. The normal rats were chosen as controls. and were also divided into 2 groups according to receiving colon distension or not. The expression of P2X4 and P2X7 receptors was detected in neurons of DCN and dorsal horn of the sacral segment of spinal cord in all rats using the immunofluorescent staining method, and the myoelectric changes of the rectus abdominis were recorded at the same time. Result.s The electro-activity of the rectus abdominis and the expression of P2X4 and P2X7 receptors were significantly en

  3. Acute rehabilitation of spinal cord injury

    OpenAIRE

    KIDRIČ-SIVEC, Urška; SEDEJ, Bogdana; MAROLT, Melita

    2015-01-01

    Traumatic spinal cord injury presents with loss of function of neuromuscular and other systems below the level of injury. Patients may suffer from minor loss of strength to complete quadriplegia with respiratory distress. All the patients with traumatic spinal cord injury who are admitted and treated in University Medical Centre Ljubljana are evaluated after admission and individualized plan of rehabilitation is made. The neurological level of injury is documented with international standa...

  4. Umbilical Cord Segmental Hemorrhage and Fetal Distress

    Science.gov (United States)

    Larciprete, Giovanni; Romanini, Maria Elisabetta; Arduini, Domenico; Cirese, Elio; Slowikowska-Hilczer, Jolanta; Kula, Krzysztof

    2006-01-01

    We describe an unexplained case of umbilical cord segmental hemorrhage linked with meconium-stained amniotic fluid. A severely asphyxiated infant was delivered at term by Caesarean section. There were poor prognostic signs on fetal cardiotocography with rupture of membranes with meconium-stained amniotic fluid. The pathophysiologic mechanism in this case is still unknown, even if we argued a possible role of the umbilical cord shortness. PMID:23674981

  5. Advanced Restoration Therapies in Spinal Cord Injury

    Science.gov (United States)

    2015-07-01

    including but not limited to traumatic brain injury , Alzheimer’s disease, cerebrovascular insults, and leukodystrophy. SECTION 2 – KEYWORDS Spinal...Spinal Cord Injury Annual Report to change our proposed anesthesia method from isofluorane to medetomidine. We have made the appropriate changes and...McKinley, W., and Tulsky, D. (2004). Late neurologic recovery after traumatic spinal cord injury . Arch Phys Med Rehabil 85, 1811-1817. Lorenz, D.J

  6. Tuberculosis of the Spermatic Cord: Case Report

    Directory of Open Access Journals (Sweden)

    Amine Benjelloun

    2014-09-01

    Full Text Available The spermatic cord tuberculoma is uncommon, especially in its lower portion. Most cases were described in Japanese literature. We report a case of tuberculosis of the spermatic cord in a sexually active young man, revealed by a scrotal mass mimicking a tumor of the testicle and discuss the suitable diagnostic and therapeutic procedures, with preservation of the testes and the other sexual organs.

  7. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, ... fibrillin. A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, ...

  8. Metabolic Syndrome

    Science.gov (United States)

    ... hypertension, hypertriglyceridemia, insulin resistance syndrome, low HDL cholesterol, Metabolic Syndrome, overweight, syndrome x, type 2 diabetes Family Health, Kids and Teens, Men, Women January 2005 Copyright © American Academy of Family PhysiciansThis ...

  9. Williams syndrome

    Science.gov (United States)

    Williams-Beuren syndrome ... Williams syndrome is caused by not having a copy of several genes. Parents may not have any family history of the condition. However, people with Williams syndrome have a 50% chance of passing the ...

  10. Neuroimaging for spine and spinal cord surgery

    Energy Technology Data Exchange (ETDEWEB)

    Koyanagi, Izumi [Hokkaido Neurosurgical Memorial Hospital (Japan); Iwasaki, Yoshinobu; Hida, Kazutoshi

    2001-01-01

    Recent advances in neuroimaging of the spine and spinal cord are described based upon our clinical experiences with spinal disorders. Preoperative neuroradiological examinations, including magnetic resonance (MR) imaging and computerized tomography (CT) with three-dimensional reconstruction (3D-CT), were retrospectively analyzed in patients with cervical spondylosis or ossification of the posterior longitudinal ligament (130 cases), spinal trauma (43 cases) and intramedullary spinal cord tumors (92 cases). CT scan and 3D-CT were useful in elucidating the spine pathology associated with degenerative and traumatic spine diseases. Visualization of the deformity of the spine or fracture-dislocation of the spinal column with 3D-CT helped to determine the correct surgical treatment. MR imaging was most important in the diagnosis of both spine and spinal cord abnormalities. The axial MR images of the spinal cord were essential in understanding the laterality of the spinal cord compression in spinal column disorders and in determining surgical approaches to the intramedullary lesions. Although non-invasive diagnostic modalities such as MR imaging and CT scans are adequate for deciding which surgical treatment to use in the majority of spine and spinal cord disorders, conventional myelography is still needed in the diagnosis of nerve root compression in some cases of cervical spondylosis. (author)

  11. Cytokines in Sjogren's syndrome

    NARCIS (Netherlands)

    N. Roescher; P.P. Tak; G.G. Illei

    2009-01-01

    Cytokines play a central role in the regulation of immunity and are often found to be deregulated in autoimmune diseases. Sjogren's syndrome is a chronic autoimmune disease characterized by inflammation and loss of secretory function of the salivary and lachrymal glands. This review highlights the c

  12. Reversible cerebral vasoconstriction syndrome

    Directory of Open Access Journals (Sweden)

    Saini Monica

    2009-01-01

    Full Text Available Reversible cerebral vasoconstriction syndromes (RCVS are a group of disorders that have in common an acute presentation with headache, reversible vasoconstriction of cerebral arteries, with or without neurological signs and symptoms. In contrast to primary central nervous system vasculitis, they have a relatively benign course. We describe here a patient who was diagnosed with RCVS.

  13. Vasoactive intestinal polypeptide (VIP) immunoreactivity in the ependymal cells of the rat spinal cord.

    Science.gov (United States)

    Chung, K; Lee, W T

    1988-12-19

    Vasoactive intestinal polypeptide (VIP) was demonstrated immunohistochemically in the entire ependymal and subependymal cells in all levels (cervical: C, thoracic: T, lumbar: L and sacral: S) of normal adult rat spinal cord. The VIP-immunoreactive basal processes from the apical ependymal cells coursed dorsally or ventrally along the median plane and reached the pia mater of the dorsal and ventral median septa. Many VIP-immunoreactive basal processes terminated on the blood vessels in the neuropil around the central canal. A few microvilli of the ependymal cells that project into the central canal also demonstrated intense VIP immunoreactivity. These observations suggest that ependymal cells may be involved in the modulation of VIP levels in the cerebrospinal fluid and regulation of vascular tone of the blood vessels in the spinal cord.

  14. Identification of Multiple Subsets of Ventral Interneurons and Differential Distribution along the Rostrocaudal Axis of the Developing Spinal Cord

    NARCIS (Netherlands)

    C. Francius (Cédric); A Harris (Anna); V. Rucchin (Vincent); T.J. Hendricks (Timothy); F.J. Stam (Floor); M. Barber (Melissa); D. Kurek (Dorota); F.G. Grosveld (Frank); A. Pierani (Alessandra); J. Goulding (John); F. Clotman (Frédéric)

    2013-01-01

    textabstractThe spinal cord contains neuronal circuits termed Central Pattern Generators (CPGs) that coordinate rhythmic motor activities. CPG circuits consist of motor neurons and multiple interneuron cell types, many of which are derived from four distinct cardinal classes of ventral interneurons,

  15. S100 protein content of umbilical cord blood in healthy newborns in relation to mode of delivery

    NARCIS (Netherlands)

    Wirds, J W; Duyn, A E J; Geraerts, S D; Preijer, E; Van Diemen-Steenvoorde, J A A M; Van Leeuwen, J H Schagen; Haas, F J L M; Gerritsen, W B M; De Boer, A; Leusink, J A

    2003-01-01

    BACKGROUND: Early detection and quantification of brain damage in neonatal asphyxia is important. In adults, S100 protein in blood is associated with damage to the central nervous system. OBJECTIVE: To determine whether S100 protein can be detected in arterial and venous cord blood of healthy newbor

  16. The Acquired Immunodeficiency Syndrome on the Firstly Symptom with the Central Nervous System%以中枢神经系统症状为首发表现的艾滋病临床分析

    Institute of Scientific and Technical Information of China (English)

    陶鹏飞; 雷素云; 杨欣平; 黄石珍; 聂皎

    2011-01-01

    目的 探讨以中枢神经系统症状为首发表现的获得性免疫缺陷综合征(AIDS)的临床特征及预后,提高临床医师对这部分患者的诊断识别能力,防止临床漏诊和误诊.方法 回顾性分析2009 ~2010年云南省传染病专科医院感染科收治的500例艾滋病患者的临床资料,其中80例患者以中枢神经系统表现为首发症状.结果 80例患者中AIDS痴呆综合征5例,结核性脑膜炎15例(包括结核性脑膜脑炎),隐球菌脑膜炎30例,弓形虫脑病20例,脑梗死4例,巨细胞病毒神经根炎3例,进行性多灶性白质脑病3例.所有病例出现中枢神经系统表现后行人类免疫缺陷病毒抗体检测阳性,诊断为艾滋病.结论 以中枢神经系统症状为首发症状的AIDS患者病情复杂,预后差,病死率高,早期诊断及治疗可以使患者病死率下降.%Objective To investigate the clinical feature and prognosis on the firstly symptom with the central nervous svstem( CNS )in the acquired immunodeficiency syndrome( AIDS )that boosted the partly diagnosis ability for AIDS patients , to prevent the clinical misdiagnosis and mistreatment. Methods 80 examples were retrospectively analyzed with the CNS symptom among 500 treated AIDS patients in 2009 to 2010,from Infectious Disease Hospital for Yunnan Province. Results In 80 patients relevant AIDS chronic brain syndrome ,including 5 cases of AIDS dementia syndrome , 15 cases of tuherculous meningeal encephalitis , 30 cases of neoformans,20 cases of toxoplasma encephalopathy,4 cases of cerebral infarction,3 cases of CMV nerve root inflammation , 3 cases of progressive multifocal white matter encephalopathy. All cases showing central nervous system symptoms test positive for HIV antibodies and diagnosis of AIDS. Conclusion The results implied that AIDS patients with the first symptom of CNS showed fast development,poor prognosis and high death rate. Early diagnosis and treatment can make a decline of death rate.

  17. Genetics Home Reference: Perry syndrome

    Science.gov (United States)

    ... 2008 Aug 23. Citation on PubMed or Free article on PubMed Central Wider C, Wszolek ZK. Rapidly progressive familial parkinsonism with central hypoventilation, depression and weight loss (Perry syndrome)--a literature review. Parkinsonism Relat Disord. 2008;14(1):1-7. ...

  18. The treatment of neurodegenerative disorders using umbilical cord blood and menstrual blood-derived stem cells.

    Science.gov (United States)

    Sanberg, Paul R; Eve, David J; Willing, Alison E; Garbuzova-Davis, Svitlana; Tan, Jun; Sanberg, Cyndy D; Allickson, Julie G; Cruz, L Eduardo; Borlongan, Cesar V

    2011-01-01

    Stem cell transplantation is a potentially important means of treatment for a number of disorders. Two different stem cell populations of interest are mononuclear umbilical cord blood cells and menstrual blood-derived stem cells. These cells are relatively easy to obtain, appear to be pluripotent, and are immunologically immature. These cells, particularly umbilical cord blood cells, have been studied as either single or multiple injections in a number of animal models of neurodegenerative disorders with some degree of success, including stroke, Alzheimer's disease, amyotrophic lateral sclerosis, and Sanfilippo syndrome type B. Evidence of anti-inflammatory effects and secretion of specific cytokines and growth factors that promote cell survival, rather than cell replacement, have been detected in both transplanted cells.

  19. The Acrocallosal Syndrome in A Neonate With Further Widening of Phenotypic Expression

    Directory of Open Access Journals (Sweden)

    Ravish SINGHAL*

    2014-04-01

    Full Text Available How to Cite This Article: Singhal R, Pandit S, Saini A, Singh P, Dhawan N. The Acrocallosal Syndrome in A Neonate With Further Widening of Phenotypic Expression. Iran J Child Neurol. 2014 Spring;8(2:60-64. The presentation of the typical characteristics of the acrocallosal syndrome (ACLS are hypoplasia/agenesis of corpus callosum, moderate to severe mental retardation, characteristic craniofacial abnormalities, distinctive digitalmalformation, and growth retardation in a neonate.An Indian neonate presented on day 1 of life (youngest in the literature to be reported with combination of abnormalities consistent with the acrocallosal syndrome and some additional findings. The baby, born to non-consanguineous, healthy parents, presented with macrocephaly, prominent forehead, hypertelorism, polydactyly of the hands and feet, duplication of hallux, hypotonia, recurrent cyanotic episodes, rib anomalies, dextro-positioning of heart, and delayed fallof umbilical cord.As the mode of inheritance of ACLS is autosomal recessive, the risk of recurrence is 25%. Genetic counselling is of prime importance, Polydactyly, and central nervous system malformations can be detected by ultrasonography in the second trimester, but due to variability of presentation, prenatal diagnosis may not always be possible.References1. Schinzel A. Postaxial polydactyly, hallux duplication, absence of corpus callosum, macencephaly and severemental retardation: a new syndrome? Helv Paediatr Acta 1979:34:141-146.2. Schinzel A. The acrocallosal syndrome: expansion of the phenotypic spectrum. Clin Dysmorphol 1994;3:31-34.3. Schinzel A, Schmid W. Hallux duplication, postaxial Polydactyly, absence of corpus callosum, severe mental retardation and additional anomalies in two unrelated patients: a new syndrome. Am J Med Genet 1980;6:241-249.4. Moeschler JB, Pober BR, Holmes LB, Graham JM Jr. Acrocallosal syndrome: new findings. Am J Med Genet 1989;32:195-199.5. Schinzel A

  20. Structural and functional reorganization of propriospinal connections promotes functional recovery after spinal cord injury

    Directory of Open Access Journals (Sweden)

    Linard Filli

    2015-01-01

    Full Text Available Axonal regeneration and fiber regrowth is limited in the adult central nervous system, but research over the last decades has revealed a high intrinsic capacity of brain and spinal cord circuits to adapt and reorganize after smaller injuries or denervation. Short-distance fiber growth and synaptic rewiring was found in cortex, brain stem and spinal cord and could be associated with restoration of sensorimotor functions that were impaired by the injury. Such processes of structural plasticity were initially observed in the corticospinal system following spinal cord injury or stroke, but recent studies showed an equally high potential for structural and functional reorganization in reticulospinal, rubrospinal or propriospinal projections. Here we review the lesion-induced plastic changes in the propriospinal pathways, and we argue that they represent a key mechanism triggering sensorimotor recovery upon incomplete spinal cord injury. The formation or strengthening of spinal detour pathways bypassing supraspinal commands around the lesion site to the denervated spinal cord were identified as prominent neural substrate inducing substantial motor recovery in different species from mice to primates. Indications for the existence of propriospinal bypasses were also found in humans after cortical stroke. It is mandatory for current research to dissect the biological mechanisms underlying spinal circuit remodeling and to investigate how these processes can be stimulated in an optimal way by therapeutic interventions (e.g., fiber-growth enhancing interventions, rehabilitation. This knowledge will clear the way for the development of novel strategies targeting the remarkable plastic potential of propriospinal circuits to maximize functional recovery after spinal cord injury.