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Katherine B Santosa, Alexandra M Keane, Albina Jablonka-Shariff, Bianca Vannucci, Alison K Snyder-Warwick
The terminal Schwann cell (tSC), a type of nonmyelinating Schwann cell, is a significant yet relatively understudied component of the neuromuscular junction. In addition to reviewing the role tSCs play on formation, maintenance, and remodeling of the synapse, we review studies that implicate tSCs in neuromuscular diseases including spinal muscular atrophy, Miller-Fisher syndrome, and amyotrophic lateral sclerosis, among others. We also discuss the importance of these cells on degeneration and regeneration after nerve injury...
March 13, 2018: Journal of Neuroscience Research
Anas Alrohimi, Rajive Jassal
Headache is an uncommon symptom in Guillain-Barré syndrome (GBS). We review four clinical settings related to GBS in which headache may be present. We focus on pathophysiological explanations, alerting the clinician to further potential investigations and treatment. Most reports of headache in GBS occur in the context of the posterior reversible encephalopathy syndrome, an increasingly recognized dysautonomia-related GBS complication. Less frequent is headache in the setting of increased intracranial pressure and papilledema (secondary intracranial hypertension), Miller Fisher syndrome, and cerebral venous sinus thrombosis...
March 2018: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
Tatsuya Ueno, Ryoya Kimura, Tomoya Kon, Rie Haga, Haruo Nishijima, Jin-Ichi Nunomura, Masahiko Tomiyama
Miller Fisher syndrome (MFS) can be difficult to diagnose, particularly in mild cases where some of the standard triad of symptoms (external ophthalmoplegia, ataxia, and loss of deep tendon reflex) are absent. We herein report a case of the incomplete form of MFS diagnosed in a 54-year-old Japanese man who presented only with ataxia symptoms and was positive for the anti-GQ1b antibody. However, the patient also suffered from dysgeusia, a significant impairment of taste perception. We propose that dysgeusia in acute-onset ataxia cases may constitute an important clinical feature to aid in the diagnosis of the incomplete form of MFS...
February 28, 2018: Internal Medicine
Chaoyang Jing, Zhuo Wang, Chaojia Chu, Ming Dong, Weihong Lin
RATIONALE: Bickerstaff brainstem encephalitis (BBE) and Miller-Fisher syndrome (MFS) might be a pedigree disease. Herein, we report a rare case that fits the diagnoses of both MFS and BBE. PATIENT CONCERNS: A 48-year-old woman was hospitalized due to blurred vision and unsteady gait lasting for 9 days, and numbness of the limbs lasting for 6 days. Physical examination showed restricted eye movement without nystagmus, bilateral flattening of forehead and nasolabial folds, and positive eyelash sign...
March 2018: Medicine (Baltimore)
Mitch J Hargis, Melissa Howdeshell
No abstract text is available yet for this article.
March 2018: Journal of Clinical Neuromuscular Disease
Sadanandavalli Retnaswami Chandra, Venkata Raviteja Karru, M A Mukheem Mudabbir, Subashree Ramakrishnan, Anitha Mahadevan
Introduction: Immune-mediated peripheral neuropathy is the term applied to a spectrum of peripheral nerve disorders where immune dysregulation plays a role. Therefore, they are treatable. We analyzed the cases seen in the past 3 years by us and evaluated the clinical, laboratory, and outcome parameters in these patients. Patients and Methods: Consecutive patients seen by the authors and diagnosed as immune-mediated neuropathy were analyzed for etiology, pathology, and outcome assessed...
January 2018: Journal of Neurosciences in Rural Practice
Junliang Yuan, Juan Zhang, Bingwei Zhang, Wenli Hu
OBJECTIVE: To evaluate all the coincidence cases of Guillain-Barre syndrome (GBS) and myasthenia gravis (MG). METHODS: We performed web-based research of the overlapping incidence of GBS and MG in studies occurring from 1982 to 2016 and restricted to the English language. RESULTS: Among 15 cases, an elevated CSF protein level without pleocytosis was found in 10 cases (66.7%); reduced nerve conduction was found in 13 cases (86.6%); a positive repetitive nerve stimulation test occurred in 11 cases (73...
January 2018: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
Jingzhe Han, Ye Ji, Duanhua Cao, Zhilei Kang, Jianguo Zhu
RATIONALE: There were no reports of Miller Fisher syndrome (MFS) with acute angle-closure glaucoma as the first manifestation. PATIENT CONCERNS: A 78-year-old female patient was admitted to our hospital with pain in her left eye, blurred vision along with nausea, and vomiting for 2 days. It was extremely rare that ophthalmoplegia, loss of tendon reflexes, and ataxia, did not occur in the early stages of MFS, and initial presentation was only dilated pupis and an increase in intraocular pressure...
December 2017: Medicine (Baltimore)
Shuping Liu, Zheman Xiao, Min Lou, Fang Ji, Bei Shao, Hongyuan Dai, Can Luo, Bo Hu, Ruiling Zhou, Zhangyu Zou, Jing Li, Xiaoyi Li, Jun Xu, Fan Hu, Chao Qin, Lian Wang, Tao Liu, Runtao Bai, Yangmei Chen, Haiyan Lv, Ruxu Zhang, Xiaoming Wang, Yunfu Wang, Shanling Ren, Xiaoming He, Zhenwei Jiang, Huiwen Wu, Donghui Yu, Wenqiong Yang, Wenjing Luo, Daokai Gong, Bin Chen, Yin Liu, Jiajia Yao, Yujie Yang, Jingxia Guan, Mingzhen Zhu, Xiujuan Fu, Genshan Gao, Hong Zhang, Man Ding, Shanghua Fan, Qian Cao, Jingyi Lu, Zuneng Lu
OBJECTIVES: The clinical and epidemiological profiles of Guillain-Barré syndrome (GBS) in southern China have yet to be fully recognised. We aimed to investigate the subtypes of GBS in southern China, compare the clinical features of demyelinating form with that of axonal form and test whether preceding infections and age have influence on the clinical phenotype, disease course and severity of GBS. METHODS: Medical records of patients with a diagnosis of GBS admitted to 31 tertiary hospitals, located in 14 provinces in southern China, from 1 January 2013 to 30 September 2016, were collected and retrospectively reviewed...
January 11, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
Mustafa Aldag, Sebnem Albeyoglu, Ufuk Ciloglu, Hakan Kutlu, Levent Ceylan
Miller-Fisher syndrome (MFS) is an uncommon neurological disorder that is considered a variant of the Guillain-Barre syndrome (GBS). It is clinically defined by a triad of symptoms, namely ataxia, areflexia and ophthalmoplegia. These acute inflammatory polyradiculopathic syndromes can be triggered by viral infections, major surgery, pregnancy or vaccination. While the overall incidence of GBS is 1.2-2.3 per 100 000 per year, MFS is a relatively rare disorder. Only six cases of GBS after cardiac surgery have been reported, and to our knowledge, we describe the first case of MFS after coronary artery bypass surgery...
November 23, 2017: Cardiovascular Journal of Africa
Eelco F M Wijdicks
Neurologic examination of the comatose patient has gradually matured. Less than 50 years ago, neurological examination in coma became a regular part of textbooks with separate chapters devoted to the topic but many were deficient in detail. In 1969, C.M. Fisher published an extraordinary 56-page paper on the examination of the comatose patient. The paper-one of Fisher's gems-is not well known and infrequently cited. The many new observations collected in this comprehensive paper are reviewed in this vignette, which highlights not only how these contributions shaped our thinking on coma but also questioned shaky concepts...
December 29, 2017: Neurocritical Care
X Y Yang, T L Han, W H Zhang, S Zhang, F Fang
Objective: To investigate the clinical manifestations, laboratory findings, treatment and outcome of anti-GQ1b antibody syndrome. Method: The clinical manifestations, laboratory examination, diagnosis, treatment and prognosis of (4 patients 4 male patients, from 4 to 12 years) with anti-GQ1b syndrome in Beijing Children's Hospital affiliated to Capital Medical University from 2015 to 2016 were retrospectively analyzed. Result: All 4 children presented with ataxia. Case 1 showed impaired speech, ptosis and weakness of arms; case 2 and 3 had external ophthalmoplegia, weakness of limbs; case 4 presented hypersomnia, irritability and hallucinations...
December 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Tiago Fernando Souza de Araújo, Marcos Lange, Viviane H Zétola, Ayrton Massaro, Hélio A G Teive
Charles Miller Fisher is considered the father of modern vascular neurology and one of the giants of neurology in the 20th century. This historical review emphasizes Prof. Fisher's magnificent contribution to vascular neurology and celebrates the 65th anniversary of the publication of his groundbreaking study, "Transient Monocular Blindness Associated with Hemiplegia."
October 2017: Arquivos de Neuro-psiquiatria
Qiaoxia Hu, Hongfu Li, Jun Tian, Baorong Zhang
The study aimed to determine the incidence and the onset time of bulbar paralysis (BP) associated with Miller-Fisher syndrome (MFS) and its overlaps, to better understand the clinical characteristics among patients with MFS and its overlaps. Medical records from 48 patients with MFS and its overlaps were divided into two groups based on the presence (MFS-BP+) or absence (MFS-BP-) of BP. Their clinical features, laboratory and electrophysiological findings, neuroimaging data, and treatment plan were analyzed and compared between two groups...
November 9, 2017: Neurological Sciences
Zsolt Illes, Morten Blaabjerg
The classic immunologic alteration of the cerebrospinal fluid (CSF) in Guillain-Barré syndrome (GBS), albuminocytologic dissociation, has been known since the original paper by Guillain, Barré, and Strohl. Albuminocytologic dissociation has been also described in other forms of the GBS spectrum, such as axonal motor or motor-sensory forms (AMAN, AMSAN), the anti-GQ1b spectrum of Miller Fisher syndrome, and Bickerstaff brainstem encephalitis. Cytokines, chemokines, antibodies, complement components, and molecules with a putative neuroprotective role or indicating axonal damage have also been examined using different methods...
2017: Handbook of Clinical Neurology
Nobuko Shiraiwa, Mitsumasa Umesawa, Sachiko Hoshino, Tsuyoshi Enomoto, Susumu Kusunoki, Akira Tamaoka, Norio Ohkoshi
No abstract text is available yet for this article.
August 29, 2017: Neurology International
Eric D Goldstein, Rocco Cannistraro, Stephen English, Rocio Vazquez Do Campo, Benjamin Eidelman
Limb-shaking transient ischemic attacks (LSTIAs) are a phenomenon that occurs due to transient hypoperfusion to a cerebral motor territory with a chronically outstripped autoregulatory vascular reserve. First described in 1962 by Miller Fisher, the pathogenesis and the global understanding of this presentation have undergone a significant advancement throughout the years. Typically, patients will present with this syndrome of transient hypoperfusion in the context of extracranial carotid intrinsic vessel stenosis or by intracranial vascular stenosis to select motor pathways...
October 25, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Yoshitsugu Nakamura, Hideto Nakajima, Takafumi Hosokawa, Kazushi Yamane, Shimon Ishida, Fumiharu Kimura
We herein report the case of a 53-year-old man with cerebellar ataxia with anti-glutamic acid decarboxylase antibody (GAD-Ab) who mimicked Miller Fisher syndrome (MFS). He developed ophthalmoplegia, diplopia, and gait ataxia for one week. The serum and CSF GAD-Ab titers were greatly increased, and the GAD-Ab index suggesting intrathecal antibody synthesis was elevated, while GQ1b-Ab was negative. After steroid pulse therapy and following prednisolone, his symptoms dramatically improved over the course of 11 months with the simultaneous decline of GAD-Ab titers...
November 1, 2017: Internal Medicine
Christine Verboon, Heleen van Berghem, Pieter A van Doorn, Liselotte Ruts, Bart C Jacobs
BACKGROUND: Patients with Miller Fisher syndrome (MFS) may have a relatively mild clinical course or progress to Guillain-Barré syndrome with limb weakness (MFS-GBS overlap syndrome). Other variants in this spectrum are GBS with ophthalmoparesis and Bickerstaff's Brainstem encephalitis (BBE). AIM: To compare the clinical course of MFS and overlap syndromes and to identify predictors of disease progression. METHODS: In a prospective study of 170 patients with GBS and variant forms, 37 (22%) had a MFS, MFS-GBS overlap syndrome, ophthalmoplegic GBS or BBE...
October 24, 2017: Journal of the Peripheral Nervous System: JPNS
Jayantee Kalita, Mritunjai Kumar, Usha K Misra
INTRODUCTION: There have been few reports on subtypes of Guillain-Barré syndrome (GBS) in children. We compared clinical and laboratory findings of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). METHODS: One hundred forty children with GBS were included. Based on nerve conduction study (NCS) findings, patients were subclassified as AIDP, AMAN, acute motor sensory axonal neuropathy (AMSAN), and equivocal...
October 20, 2017: Muscle & Nerve
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