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Miller-Fisher

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https://www.readbyqxmd.com/read/28067631/schwann-cells-are-activated-by-atp-released-from-neurons-in-an-in-vitro-cellular-model-of-miller-fisher-syndrome
#1
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
https://www.readbyqxmd.com/read/28040387/takotsubo-cardiomyopathy-associated-with-miller-fisher-syndrome
#2
Dalvir Gill, Kan Liu
51-year-old female who presented with progressive paresthesia, numbness of the lower extremities, double vision, and trouble walking. Physical exam was remarkable for areflexia, and ptosis. Her initial EKG showed nonspecific ST segment changes and her Troponin T was elevated to 0.41ng/mL which peaked at 0.66ng/mL. Echocardiogram showed a depressed left ventricular ejection fraction to 35% with severely hypokinetic anterior wall and left ventricular apex was severely hypokinetic. EMG nerve conduction study showed severely decreased conduction velocity and prolonged distal latency in all nerves consistent with demyelinating disease...
December 22, 2016: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27974981/an-overlapping-case-of-miller-fisher-syndrome-bickerstaff-s-encephalitis-and-the-asman-variant-of-guillain-barre-syndrome
#3
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
https://www.readbyqxmd.com/read/27939129/guillain-barr%C3%A3-syndrome-is-induced-in-non-obese-diabetic-nod-mice-following-campylobacter-jejuni-infection-and-is-exacerbated-by-antibiotics
#4
J L St Charles, J A Bell, B J Gadsden, A Malik, H Cooke, L K Van de Grift, H Y Kim, E J Smith, L S Mansfield
Campylobacter jejuni is a leading cause of bacterial gastroenteritis linked to several serious autoimmune sequelae such as the peripheral neuropathies Guillain Barré syndrome (GBS) and Miller Fisher syndrome (MFS). We hypothesized that GBS and MFS can result in NOD wild type (WT) mice or their congenic interleukin (IL)-10 or B7-2 knockouts secondary to C. jejuni infection. Mice were gavaged orally with C. jejuni strains HB93-13 and 260.94 from patients with GBS or CF93-6 from a patient with MFS and assessed for clinical neurological signs and phenotypes, anti-ganglioside antibodies, and cellular infiltrates and lesions in gut and peripheral nerve tissues...
December 8, 2016: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27928406/late-presentation-of-ataxia-areflexia-and-electrophysiological-abnormalities-as-part-of-miller-fisher-syndrome-case-report
#5
Deniz Somer, Arzu Yilmaz, Serap Tiras Teber, Fatma Gul Cinar
Miller Fisher syndrome (MFS) is characterised by the triad of ophthalmoplegia, ataxia, and areflexia. A case with external ophthalmoplegia and absence of ataxia and areflexia until the end of second week is presented. Electrophysiological findings became apparent after the third week and showed reduced amplitudes of sensory nerve action potentials and prolonged latencies of F with no evidence of conduction blocks. There was no response to intravenous immunoglobulin, but there was response to corticosteroids...
August 2016: Neuro-ophthalmology
https://www.readbyqxmd.com/read/27888275/acute-disseminated-encephalomyelitis-following-campylobacter-jejuni-gastroenteritis-case-report-and-review-of-the-literature
#6
Simone Marziali, Eliseo Picchi, Francesca Di Giuliano, Simone Altobelli, Giorgia Mataluni, Girolama Marfia, Francesco Garaci, Roberto Floris
We describe a case of a 25-year-old male with a diagnosis of acute disseminated encephalomyelitis (ADEM) following infection with Campylobacter jejuni, which is implicated in various human pathologies regarding the central nervous system (CNS) with acute course like Guillain-Barré syndrome (GBS), Miller-Fisher syndrome (MFS), Bickerstaff's brainstem encephalitis (BEE), acute transverse myelitis (ATM) as well as ADEM. These conditions are caused by cross-reactivity between Campylobacter's epitopes and cells of the CNS that causes an immunomediated inflammatory demyelination of the CNS...
November 25, 2016: Neuroradiology Journal
https://www.readbyqxmd.com/read/27875624/a-case-of-miller-fisher-syndrome-associated-with-preceding-herpes-zoster-ophthalmicus
#7
Angelo Cascio Rizzo, Martina Ulivi, Nicoletta Brunelli, Alessio Pepe, Giovanni Assenza, Lucia Florio, Vincenzo Di Lazzaro
No abstract text is available yet for this article.
November 22, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27737870/miller-fisher-syndrome
#8
Suresh Kumar Gupta, Kunal Kishor Jha, Mhd Diaa Chalati, Losan Tareq Alashi
A man aged 30 years presented to the emergency department (ED) with ataxia, areflexia, facial weakness, ophthalmoplegia, extremity weakness and back pain for 4 days. 4 days prior to attending the ED, the patient had suffered from diarrhoea for 2 weeks. The diagnosis of Miller Fisher syndrome was performed on the dual basis of clinical features in addition to an investigations report. Nerve conduction studies and anti-GQ1b IgG antibody analysis were requested. Once IgA deficiency was ruled out, the patient was started on intravenous immunoglobulin (400 mg/kg/day)...
October 13, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27673265/poster-373-a-immunoglobulin-treatment-of-miller-fisher-variant-of-acute-demyelinating-inflammatory-polyneuropathy-with-underlying-chronic-inflammatory-demyelinating-polyneuropathy-a-case-report
#9
John W Hawkins
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/27618602/long-term-effect-of-rituximab-in-a-case-with-late-onset-rasmussen%C3%A2-s-encephalitis-with-anti-ganglioside-iggq1b-and-anti-gad-antibodies-positivity-case-report
#10
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
https://www.readbyqxmd.com/read/27616001/top-100-cited-articles-on-guillain-barr%C3%A3-syndrome-a-bibliometric-analysis
#11
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
https://www.readbyqxmd.com/read/27609285/miller-fisher-syndrome-presenting-as-palate-paralysis
#12
Mohammad Hassan A Noureldine, Ahmad Sweid, Rechdi Ahdab
We report a 63-year old patient who presented to our care initially with a hypernasal voice followed by ataxia, ptosis, dysphonia, and paresthesias. The patient's history, physical examination, and additional tests led to a Miller Fisher syndrome (MFS) diagnosis. Palatal paralysis as an inaugurating manifestation of MFS is quite rare and requires special attention from neurologists and otolaryngologists. Although it may present as benign as an acute change in voice, early diagnosis and prompt management may prevent further complications...
September 15, 2016: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/27597525/an-animal-model-of-miller-fisher-syndrome-mitochondrial-hydrogen-peroxide-is-produced-by-the-autoimmune-attack-of-nerve-terminals-and-activates-schwann-cells
#13
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
https://www.readbyqxmd.com/read/27567246/polyclonal-igm-and-iga-block-in-vitro-complement-deposition-mediated-by-anti-ganglioside-antibodies-in-autoimmune-neuropathies
#14
Makoto Sudo, Kazuki Miyaji, Peter J Späth, Kana Morita-Matsumoto, Yoshiki Yamaguchi, Nobuhiro Yuki
Intravenous immunoglobulin (IVIG), consisting of IgG, is the first-line treatment for Guillain-Barré syndrome and multifocal motor neuropathy. IgG, but neither IgM nor IgA, has been demonstrated in vitro to inhibit complement deposition mediated by anti-ganglioside autoantibodies in sera from patients with both conditions. The objective of this study is to investigate the in vitro effectiveness of IgM and IgA in inhibiting complement deposition to ganglioside/anti-ganglioside antibody complexes. Serum samples were obtained from patients with multifocal motor neuropathy associated with anti-GM1 IgM antibodies, Guillain-Barré syndrome associated with anti-GM1 IgG antibodies and Miller Fisher syndrome associated with anti-GQ1b IgG antibodies...
November 2016: International Immunopharmacology
https://www.readbyqxmd.com/read/27460528/pediatric-miller-fisher-syndrome-complicating-an-epstein-barr-virus-infection
#15
Céline Communal, Anne Filleron, Sandrine Baron-Joly, Randa Salet, Tu-Anh Tran
BACKGROUND: Miller Fisher syndrome, a variant of Guillain-Barré syndrome, is an acute inflammatory demyelinating polyradiculoneuropathy that may occur weeks after a bacterial or viral infection. Campylobacter jejuni and Haemophilus influenzae are frequently reported etiological agents. PATIENT DESCRIPTION: We describe a boy with Miller Fisher syndrome following Epstein-002DBarr virus primary infectious mononucleosis. He presented with bilateral dysfunction of several cranial nerves and hyporeflexia of the limbs but without ataxia...
October 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/27450064/increased-serum-concentrations-of-transforming-growth-factor-%C3%AE-1-tgf-%C3%AE-1-in-patients-with-guillain-barr%C3%A3-syndrome
#16
Kuo-Hsuan Chang, Rong-Kuo Lyu, Yen-Shi Ro, Yi-Chun Chen, Long-Sun Ro, Hong-Shiu Chang, Ching-Chang Huang, Ming-Feng Liao, Yih-Ru Wu, Hong-Chou Kuo, Chun-Che Chu, Chiung-Mei Chen
BACKGROUND: Guillain-Barré syndrome (GBS) is an acquired demyelinating peripheral neuropathy. It has shown that macrophage activation contribute to the pathogenesis of GBS. Therefore macrophage-mediated factors could be the potential markers for disease diagnosis and status of GBS. METHODS: We measured serum concentrations of 4 macrophage-mediated factors, including interleukin-6 (IL-6), transforming growth factor-β1 (TGF-β1), vascular cell adhesion protein 1 (VCAM-1) and vascular endothelial growth factor (VEGF), in 23 chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 28 GBS, 11 Miller-Fisher syndrome (MFS), 40 multiple sclerosis (MS), and 12 Alzheimer's disease (AD) patients, as well as 15 healthy controls...
October 1, 2016: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/27437164/miller-fisher-syndrome-a-case-report-highlighting-heterogeneity-of-clinical-features-and-focused-differential-diagnosis
#17
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
https://www.readbyqxmd.com/read/27436763/ophthalmoplegic-guillain-barr%C3%A3-syndrome-an-independent-entity-or-a-transitional-spectrum
#18
Jong Kuk Kim, Seuk Kyung Hong, Jong Seok Bae, Byeol-A Yoon, Hwan Tae Park, So Young Huh, Sang-Jin Kim, Jong-Eun Kim, Dae-Seong Kim
Ophthalmoplegia can occur in both Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) with typical limb involvement. However, ophthalmoplegic GBS (OGBS) has been poorly defined. We aimed to characterize OGBS and clarify the pathophysiological implications across the overall GBS spectrum. Twenty GBS and seven MFS patients from three university based teaching hospitals in Korea were enrolled and analyzed. Six GBS patients who were classified as OGBS commonly also had facial diplegia (50%) and bulbar palsy (50%), while only a small portion of non-ophthalmoplegic GBS (NOGBS) patients had facial diplegia (21%)...
October 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/27432103/overlap-of-myasthenia-gravis-and-miller-fisher-syndrome
#19
Yuji Tanaka, Kazuo Satomi
In this case report, we describe a patient with myasthenia gravis (MG) and Miller Fisher syndrome (MFS) overlap. A 69-year-old woman presented with acute bilateral ptosis, ophthalmoplegia, ataxic gait, and areflexia. The MFS diagnosis was confirmed with by a positive anti-GQ1b IgG antibody test result. MG was diagnosed from electrophysiological, edrophonium, and serological test results. Although intravenous immunoglobulin therapy is effective for both diseases, two courses of the therapy did not improve the patient's symptoms...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27406055/-bickerstaff-brainstem-encephalitis-after-upper-respiratory-infection
#20
Linda J Locht, Morten Blaabjerg
Bickerstaff brainstem encephalitis (BE) is a very rare neurological condition with subacute onset of ophthalmoplegia, ataxia and altered sensorium, often postinfectious. The condition is associated with the anti-GQ1b antibody syndrome and is part of the spectrum of diseases including Miller Fisher syndrome and Guillain-Barré syndrome. In this case, we report the history, workup, treatment and follow-up of a 48-year-old woman with probable BE without anti-GQ1b antibodies in relation to the international diagnostic criteria...
July 4, 2016: Ugeskrift for Laeger
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