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Miller fisher variant

Nooshin Salehi, Eric D Choi, Roger C Garrison
BACKGROUND Miller Fisher Syndrome is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia, and is considered to be a variant of Guillain-Barre Syndrome. Miller Fisher Syndrome is observed in approximately 1-5% of all Guillain-Barre cases in Western countries. Patients with Miller Fisher Syndrome usually have good recovery without residual deficits. Venous thromboembolism is a common complication of Guillain-Barre Syndrome and has also been reported in Miller Fisher Syndrome, but it has generally been reported in the presence of at least one prothrombotic risk factor such as immobility...
January 16, 2017: American Journal of Case Reports
Umberto Rodella, Samuele Negro, Michele Scorzeto, Elisanna Bergamin, Kees Jalink, Cesare Montecucco, Nobuhiro Yuki, Michela Rigoni
The neuromuscular junction is exposed to different types of insults including mechanical traumas, toxins or autoimmune antibodies and, accordingly, has retained through evolution a remarkable ability to regenerate. Regeneration is driven by multiple signals that are exchanged among the cellular components of the junction. These signals are largely unknown.Miller Fisher syndrome is a variant of Guillain-Barré syndrome caused by autoimmune antibodies specific for epitopes of peripheral axon terminals. Using an animal model of Miller Fisher syndrome, we recently reported that a monoclonal anti-polysialoganglioside GQ1b antibody plus complement damages nerve terminals with production of mitochondrial hydrogen peroxide, that activates Schwann cells...
January 6, 2017: Disease Models & Mechanisms
E J Pegg, S K Chhetri, U G Lekwuwa, T Majeed
A 56-year-old man presented with a 3-day history of progressive tingling of the hands, unsteadiness, and diplopia. He was initially diagnosed clinically with Miller Fisher Syndrome (MFS) but later developed limb weakness consistent with Guillain-Barre Syndrome (GBS) and subsequently reduced consciousness consistent with Bickerstaff's brainstem encephalitis (BBE). Neurophysiology revealed an axonal motor and sensory neuropathy, in keeping with the Acute Motor and Sensory Axonal Neuropathy (AMSAN) variant of GBS...
2016: Case Reports in Neurological Medicine
Zachary A Miller, Virginia E Sturm, Gamze Balci Camsari, Anna Karydas, Jennifer S Yokoyama, Lea T Grinberg, Adam L Boxer, Howard J Rosen, Katherine P Rankin, Maria Luisa Gorno-Tempini, Giovanni Coppola, Daniel H Geschwind, Rosa Rademakers, William W Seeley, Neill R Graff-Radford, Bruce L Miller
OBJECTIVE: To determine the prevalence of autoimmune disease in symptomatic C9ORF72 (C9) mutation carriers and frontotemporal dementia with motor neuron disease (FTD/MND) cohorts. METHODS: In this case-control study, we reviewed the clinical histories of 66 patients with FTD/MND and 57 symptomatic C9 carriers (24 overlapping cases), a total of 99 charts, for history of autoimmune disease. The prevalence of autoimmune disease in C9 and FTD/MND cohorts was determined by χ(2) and Fisher exact comparisons between the combined C9 and FTD/MND group with normal control, Alzheimer disease, and progressive supranuclear palsy cohorts, as well as comparisons within C9 and FTD/MND cohorts...
December 2016: Neurology® Neuroimmunology & Neuroinflammation
John W Hawkins
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Gabriela Timarova, Iveta Lisa, Peter Kukumberg
Rasmussen's encephalitis is a rare autoimmune encephalitis usually involving one brain hemisphere, presenting with refractory epileptic seizures, and neurological and cognitive decline. Only 10% of cases start later in adolescence/adulthood. The only effective treatment for refractory seizures in childhood is hemispherectomy. For late-onset cases with mild neurological deficit the hemispherectomy is usually postponed because of its severe consequences. Immunotherapy shows some temporal effect for seizure control and slowing the brain atrophy, mainly in late onset Rasmussen's encephalitis...
July 2016: Neuro Endocrinology Letters
Jee-Eun Kim, Jong Kuk Kim, Kang Min Park, Yerim Kim, Dae Young Yoon, Jong Seok Bae
Since the first description of Guillain-Barré syndrome (GBS) 100 years ago, the concept of this syndrome has changed remarkably. The purpose of our study was to identify and characterize the most-cited articles that have contributed to advancing the understanding of GBS. Based on the database of Journal Citation Reports, we selected 554 journals that were considered as potential sources of reports on studies related to clinical neurology and general medicine. The Web of Science search tools were used to identify the most-cited articles relevant to GBS or other variants in the selected journals...
December 2016: Journal of the Peripheral Nervous System: JPNS
Umberto Rodella, Michele Scorzeto, Elisa Duregotti, Samuele Negro, Bryan C Dickinson, Christopher J Chang, Nobuhiro Yuki, Michela Rigoni, Cesare Montecucco
The neuromuscular junction is a tripartite synapse composed of the presynaptic nerve terminal, the muscle and perisynaptic Schwann cells. Its functionality is essential for the execution of body movements and is compromised in a number of disorders, including Miller Fisher syndrome, a variant of Guillain-Barré syndrome: this autoimmune peripheral neuropathy is triggered by autoantibodies specific for the polysialogangliosides GQ1b and GT1a present in motor axon terminals, including those innervating ocular muscles, and in sensory neurons...
December 2016: Neurobiology of Disease
Ilya V Yepishin, Randall Z Allison, David A Kaminskas, Natalia M Zagorski, Kore K Liow
Miller Fisher Syndrome (MFS) is a rare variant of Guillain-Barré Syndrome (GBS) that has a geographically variable incidence. It is largely a clinical diagnosis based on the cardinal clinical features of ataxia, areflexia, and opthalmoplegia, however, other neurological signs and symptoms may also be present. Serological confirmation with the anti-GQ1b antibody is available and allows for greater diagnostic certainty in the face of confounding symptoms. A self-limiting course is typical of MFS. The following case report is that of a patient who presented with generalized weakness, somatic pain, inability to walk, and diplopia following an upper respiratory illness...
July 2016: Hawai'i Journal of Medicine & Public Health: a Journal of Asia Pacific Medicine & Public Health
Ioannis E Dagklis, Sotirios Papagiannopoulos, Varvara Theodoridou, Dimitrios Kazis, Ourania Argyropoulou, Sevasti Bostantjopoulou
Miller-Fisher syndrome (MFS) is considered as a variant of the Guillain-Barre syndrome (GBS) and its characteristic clinical features are ophthalmoplegia, ataxia, and areflexia. Typically, it is associated with anti-GQ1b antibodies; however, a significant percentage (>10%) of these patients are seronegative. Here, we report a 67-year-old female patient who presented with the typical clinical features of MFS. Workup revealed antibodies against glutamic acid decarboxylase (GAD) in relatively high titers while GQ1b antibodies were negative...
2016: Case Reports in Neurological Medicine
Rhona McGonigal, Madeleine E Cunningham, Denggao Yao, Jennifer A Barrie, Sethu Sankaranarayanan, Simon N Fewou, Koichi Furukawa, Ted A Yednock, Hugh J Willison
INTRODUCTION: Guillain-Barré syndrome (GBS) is an autoimmune disease that results in acute paralysis through inflammatory attack on peripheral nerves, and currently has limited, non-specific treatment options. The pathogenesis of the acute motor axonal neuropathy (AMAN) variant is mediated by complement-fixing anti-ganglioside antibodies that directly bind and injure the axon at sites of vulnerability such as nodes of Ranvier and nerve terminals. Consequently, the complement cascade is an attractive target to reduce disease severity...
March 2, 2016: Acta Neuropathologica Communications
Kai-Zheong Lim, Nirosen Vijiaratnam
No abstract text is available yet for this article.
May 2016: Muscle & Nerve
François Guisset, Christine Ferreiro, Serge Voets, Julie Sellier, France Debaugnies, Francis Corazza, Nicolas Deconinck, Cynthia Prigogine
BACKGROUND: Miller Fisher syndrome (MFS) is an acute polyradiculoneuritis regarded as an uncommon clinical variant of Guillain-Barré syndrome (GBS). MFS is characterized by the acute onset of the clinical triad of ophthalmoplegia, cereballar ataxia and areflexia. Atypical forms of MFS presenting as isolated ophthalmoplegia without ataxia have been rarely described, mostly in adults. PATIENTS: We present two cases of acute isolated bilateral ophthalmoplegia in childhood, both occurring shortly after Campylobacter jejuni enteritis...
May 2016: European Journal of Paediatric Neurology: EJPN
Bing Liao, Carlos Kamiya-Matsuoka, Xiang Fang, Robert G Smith, Elena Shanina
Miller Fisher syndrome (MFS), variant of acute inflammatory demyelinating polyradiculoneuropathy, is recognized as clinical triad (ophthalmoplegia-ataxia-areflexia). Ganglioside antibodies play an important role in the pathogenesis of acute polyradiculoneuropathy including MFS. To our knowledge, there has been no report of MFS presenting with atypical alternating ptosis or with concurrent elevation of serum GD1 and asialo-GM1 antibody titers, and negative titers of GQ1b antibody such as our patient. Our objective is to report MFS with unusual symptoms and to share our diagnostic approach...
December 2016: International Journal of Neuroscience
Gokulan Ratnarajan, Alexander Thompson, Caroline Dodridge, Allyson Parry, John Elston
No abstract text is available yet for this article.
November 2015: JAMA Neurology
Daniel De la O-Peña, Martín Robles-Figueroa, Quetzalcóatl Chávez-Peña, Martín Bedolla-Barajas
BACKGROUND: Guillain-Barré syndrome (GBS) has a cosmopolitan distribution. Its course is usually mild and tends to limit itself, but in severe cases it can cause death. The aim of this article is to describe the characteristics of a group of adults with GBS diagnosed and treated at a university hospital. METHODS: All cases of GBS that occurred between January 1, 2005 to December 31, 2009 were analyzed. The clinical records were reviewed through the implementation of a structured survey that included the following sections: patient identification, clinical data, history of infection, season of occurrence, rates of electrophysiological variants, and lethality...
November 2015: Revista Médica del Instituto Mexicano del Seguro Social
Garima Gupta, Antonio Liu
Miller Fisher syndrome is a variant of Guillain-Barre syndrome characterized by the classic triad of ophthalmoplegia, ataxia, and areflexia. Pupillary involvement is common in MFS and has been reported in 35-42% of MFS patients. Although case reports have discussed isolated ophthalmoplegia as a presentation of MFS, anisocoria and rapid fluctuation of pupillary diameter have not been reported in anti-GQ1b antibody positive individuals. Here we describe an individual who presented with diplopia and was found to have progressive internal and external ophthalmoplegia with frequent fluctuations in pupillary diameter and anisocoria...
2015: Case Reports in Neurological Medicine
Helen Chioma Okoh, Sandeep Singh Lubana, Spencer Langevin, Susan Sanelli-Russo, Adriana Abrudescu
Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate. Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease. We are reporting a rare case of Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with remarkable and complete recovery after the second 5-day course of IVIG...
2015: Case Reports in Rheumatology
Yuriy Zeylikman, Vishal Shah, Umang Shah, Thomas R Mirsen, Joseph V Campellone
We describe a patient with acute progressive weakness and areflexia. Both botulism and Miller-Fisher variant of Guillain-Barré syndrome were initial diagnostic considerations, and she was treated with intravenous immunoglobulin and botulinum antitoxin. A mouse bioassay was positive for botulinum toxin A, although her clinical course, electrodiagnostic studies, and cerebrospinal fluid findings supported Miller-Fisher syndrome. This patient's atypical features offer points of discussion regarding the evaluation of patients with acute neuromuscular weakness and emphasize the limitations of the botulism bioassay...
September 2015: Journal of Clinical Neuromuscular Disease
Francesco Pellegrini, Giovanni Prosdocimo, Jason J S Barton
A 56-year-old man with diabetes presented with acute diplopia and signs of bilateral complete abduction deficits. Diffuse areflexia was his only other sign at presentation. Within a few days, he developed complete ophthalmoplegia and ataxia, consistent with a clinical diagnosis of Miller-Fisher syndrome, and repeated history revealed a possible gastroenteritis 3 weeks prior. This case illustrates an "ophthalmoplegia without ataxia" variant of this classic autoimmune condition, which should be considered in patients presenting with bilateral VI nerve palsies...
March 2016: Survey of Ophthalmology
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