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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
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
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
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 16, 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...
September 12, 2016: Journal of the Peripheral Nervous System: JPNS
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
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
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...
August 24, 2016: International Immunopharmacology
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
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
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
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
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
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
Jiro Fukae, Jun Tsugawa, Shinji Ouma, Tomoko Umezu, Susumu Kusunoki, Yoshio Tsuboi
There have been reports from several countries that hepatitis E virus (HEV) infection is frequently associated with Guillain-Barré syndrome (GBS). This study aimed to determine the frequency of HEV infection associated with GBS in Japanese patients, and to clarify the clinical characteristics of these patients. Sera obtained from 63 patients with GBS or Miller Fisher syndrome (MFS) and 60 control subjects were examined for both HEV-IgM and HEV-IgG. Of the 63 patients, three were positive for both HEV-IgM and elevated hepatic enzymes: Two had GBS, and one had MFS...
July 7, 2016: Neurological Sciences
Hassaan Tohid
A rare kind of antibody, known as anti-glutamic acid decarboxylase (GAD) autoantibody, is found in some patients. The antibody works against the GAD enzyme, which is essential in the formation of gamma aminobutyric acid (GABA), an inhibitory neurotransmitter found in the brain. Patients found with this antibody present with motor and cognitive problems due to low levels or lack of GABA, because in the absence or low levels of GABA patients exhibit motor and cognitive symptoms. The anti-GAD antibody is found in some neurological syndromes, including stiff-person syndrome, paraneoplastic stiff-person syndrome, Miller Fisher syndrome (MFS), limbic encephalopathy, cerebellar ataxia, eye movement disorders, and epilepsy...
July 2016: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
Scott A McLeod, Wallace Wee, Francois D Jacob, Isabelle Chapados, Francois V Bolduc
Introduction. Acute complete external ophthalmoplegia is a rare finding in clinical practice that is associated with diseases affecting the neuromuscular junction, the oculomotor nerves, or the brainstem. Ophthalmoplegia has been reported with acute ataxia in Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE). Up to 95% of these cases are associated with anti-GQ1b antibodies. Only a small number of cases of anti-GQ1b negative MFS have been documented in pediatric patients. This is the first case reporting a recurrence of ocular symptoms in an anti-GQ1b antibody negative patient with BBE...
2016: Case Reports in Neurological Medicine
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
Francesca Notturno, Norito Kokubun, Yukari Sekiguki, Takahide Nagashima, Angelo De Lauretis, Nobuhiro Yuki, Satoshi Kuwabara, Antonino Uncini
Guillain-Barré syndrome (GBS) is considered a monophasic disorder yet recurrences occur in up to 6% of patients. We retrospectively studied an Italian-Japanese population of 236 GBS and 73 Miller Fisher syndrome (MFS) patients and searched for factors which may be associated with recurrence. A recurrent patient was defined as having at least two episodes that fulfilled the diagnostic criteria for GBS and MFS with an identifiable recovery after each episode and a minimum of 2months between episodes. Preceding Campylobacter jejuni (C...
June 15, 2016: Journal of the Neurological Sciences
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