gq1b-ganglioside and Cerebellar-Ataxia

Serum anti-GQ1b IgG antibody is specifically detected in almost all the patients with Fisher syndrome and those with Guillain-Barré syndrome with ophthalmoplegia in the acute phase. An anti-GQ1b mouse monoclonal antibody immunostained the paranodal regions of the extramedullary portion of the human oculomotor, trochlear and abducens nerves. Such immunostaining was not observed in the other cranial and peripheral nerves. Anti-GQ1b IgG antibody may be involved in the pathogenetic mechanism of ophthalmoplegia in Fisher syndrome and Guillain-Barré syndrome by binding to the paranodal regions of the oculomotor, trochlear and abducens nerves. Investigation of serum anti-GQ1b antibody is considered to be a useful diagnostic marker in Fisher syndrome and Guillain-Barré syndrome with ophthalmoplegia.

Topics: Autoantibodies; Carbohydrate Sequence; Cerebellar Ataxia; Gangliosides; Humans; Molecular Sequence Data; Ophthalmoplegia; Polyradiculoneuropathy

Clinical and immunological evaluation of 'incomplete' Bickerstaff brainstem encephalitis (BBE).. We studied two patients with postinfectious brainstem syndromes who presented at National University Hospital Singapore. Laboratory work-up included measurement of antiganglioside antibodies.. Both patients displayed hypersomnolence and cerebellar-like ataxia in the absence of external ophthalmoplegia and carried high serum titres of IgG anti-GQ1b antibodies, strongly indicative of BBE.. Ophthalmoplegia can be absent or incomplete in BBE, and the absence of this clinical feature should not exclude BBE from the clinicians' differential. Such cases of incomplete BBE could be defined as 'ataxic hypersomnolence without ophthalmoplegia'.

Topics: Adult; Autoantibodies; Brain Stem; Cerebellar Ataxia; Disorders of Excessive Somnolence; Encephalitis; Female; Follow-Up Studies; G(M1) Ganglioside; Gangliosides; Humans; Immunization, Passive; Middle Aged; Neurologic Examination; Ophthalmoplegia

Topics: Adult; Autoantibodies; Brain Stem; Cerebellar Ataxia; Disorders of Excessive Somnolence; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Intracellular Signaling Peptides and Proteins; Mental Status Schedule; Neurologic Examination; Neuropeptides; Ophthalmoplegia; Orexins

We report a successful treatment by plasma exchange(PE) in the case of Miller Fisher syndrome.. A 71-year-old woman rapidly developed diplopia and unsteady gait, and was admitted to Minami-matsuyama Hospital on day 3 of her illness. Ophthalmological and neurological examination on admission revealed bilateral complete external ophthalmoplegia with moderate mydriasis, cerebellar ataxia, and weakness of biceps reflexs, but her consciousness was intact. Protein concentration in the cerebrospinal-fluid was slightly increased and serum anti-GQlb IgG antibody was positive. With the diagnosis of Miller Fisher syndrome, PE was carried out on days 12, 14, and 16. After PE, ophthalmoplegia and cerebellar ataxia improved markedly. The ocular symptoms resolved after a month.. The findings indicate that PE is a beneficial treatment in Miller Fisher syndrome during the acute phase of the disease.

Topics: Aged; Autoantibodies; Cerebellar Ataxia; Female; Gangliosides; Humans; Miller Fisher Syndrome; Ophthalmoplegia; Plasma Exchange

We report a 30-year-old woman who presented symptoms of oropharyngeal palsy and glove-stocking type sensory disturbance followed by acute cerebellar ataxia, the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), gastroenteric symptoms, urinary disturbance and orthostatic hypotension. She did not have any preceding infection. She was diagnosed as having Guillain-Barré syndrome with autonomic failure. Autonomic failure such as sinus tachycardia and nocturnal ventricular arrhythmia in addition to motor and sensory dysfunction was palliated by immunoadsorption. During the course of her illness, there were elevations of antiganglioside antibodies to GT1a and GQ1b in the IgG subclass, and to GD1b and GQ1b in the IgM subclass. The elevation of anti-GD1b antibody and anti-GQ1b antibody may be pathologically related to autonomic failure, cerebellar ataxia and SIADH.

Topics: Adult; Autoantibodies; Autonomic Nervous System Diseases; Cerebellar Ataxia; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Inappropriate ADH Syndrome

Anti-GQ1b antibody seems to be a pathogenetic factor in the development of Fisher syndrome (FS). Although several patients received immunoadsorption therapy (IAT), whether it can remove the autoantibody has not yet been clarified. We treated two patients with FS by IAT using tryptophane column (TR-C) and phenylalanine column (PH-C) (TR-C; 9 times altogether in 2 patients, PH-C: twice altogether in 2 patients), and compared the removal ability of IgG anti-GQ1b antibody and immunoglobulin between TR-C and PH-C. TR-C removed the IgG anti-GQ1b antibodies, IgG, IgA and IgM more than PH-C did. TR-C removed the IgG anti-GQ1b antibody more selectively than non-specific immunoglobulin. In practicing IAT on FS, the use of TR-C rather than PH-C is recommended in view of the removal ability of the autoantibody.

Topics: Adult; Autoantibodies; Cerebellar Ataxia; Female; Gangliosides; Humans; Immunoglobulin G; Immunosorbent Techniques; Ophthalmoplegia; Phenylalanine; Polyradiculoneuropathy; Reflex, Abnormal; Syndrome; Tryptophan

In the present report, an unusual case of recurrent Miller Fisher syndrome is described. The patient presented within ten years three similar episodes of ophthalmoplegia, ataxia and areflexia associated with oropharyngeal weakness and signs of mild distal sensory neuropathy. An elevated titer of anti-GQ1b ganglioside antibodies correlated well with the clinical symptoms and signs. The pathogenic role of these antibodies in Miller Fisher syndrome is discussed.

Topics: Autoantibodies; Cerebellar Ataxia; Follow-Up Studies; Gangliosides; Humans; Male; Middle Aged; Nerve Growth Factors; Neurologic Examination; Ophthalmoplegia; Polyneuropathies; Recurrence; Reflex, Abnormal

We isolated Campylobacter jejuni from 2 patients with Fisher's syndrome subsequent to enteritis. Crude lipopolysaccharide fractions were extracted from the bacteria and separated by thin-layer chromatography. Monoclonal antibodies to GQ1b ganglioside (GMR13 and 7F5) reacted with both lipopolysaccharide fractions, indicating that the lipopolysaccharides bear the GQ1b epitope. This is the first report of molecular mimicry between neural tissue components and the antecedent infectious agents of Fisher's syndrome.

Topics: Antibodies, Monoclonal; Campylobacter Infections; Campylobacter jejuni; Cerebellar Ataxia; Chromatography, Thin Layer; Epitopes; Gangliosides; Humans; Lipopolysaccharides; Male; Molecular Mimicry; Ophthalmoplegia; Syndrome

A 15-year-old man developed diplopia, ataxic gait and bulbar palsy. Two days after the onset of neurological symptoms, neurological examination revealed external ophthalmoplegia, cerebellar ataxia, and areflexia. Muscle weakness in the areas innervated by cranial nerves and in the four limbs, and glove and stocking type sensory impairment were also observed. On the 13th hospital day, CSF protein was elevated with normal cellularity. Serum IgM anti-GQ1b antibody was increased, which decreased concurrently with the clinical improvement. Recent studies have revealed the frequent presence of serum anti-GQ1b antibody in Fisher's syndrome. Therefore, this patient showed Fisher's syndrome at the beginning, then evolved to Guillain-Barré syndrome associated with anti-GQ1b antibody, which would support close association between Fisher's syndrome and Guillain-Barré syndrome.

Topics: Adolescent; Autoantibodies; Cerebellar Ataxia; Gangliosides; Humans; Immunoglobulin M; Male; Ophthalmoplegia; Polyradiculoneuropathy; Syndrome

Topics: Animals; Autoantibodies; Cells, Cultured; Cerebellar Ataxia; Ganglia, Spinal; Gangliosides; Humans; Neurites; Neurons; Neurotoxins; Ophthalmoplegia; Plasmapheresis; Rats; Rats, Wistar; Reflex, Abnormal; Syndrome