gq1b-ganglioside and Reflex--Abnormal

Miller Fisher's syndrome is a rare variant of Guillain-Barré's syndrome characterized by the acute development of ataxia, ophthalmoparesis, and areflexia. Most patients have a measureable antibody in serum directed against the GQ1b ganglioside. This antibody is also present in the serum of patients with other forms of Guillain-Barré's syndrome who have prominent ataxia or ophthalmoplegia as part of their clinical presentation. Miller Fisher's syndrome generally is self-limited and has an excellent prognosis.

Topics: Ataxia; Autoantibodies; Gangliosides; Humans; Miller Fisher Syndrome; Ophthalmoplegia; Reflex, Abnormal; Treatment Outcome

This review primarily concerns two issues. First, in myasthenia gravis, what mechanisms are likely to underlie the low threshold for extraocular muscle involvement in this disease? Second, what is the likely importance of anti-GQ1b ganglioside antibodies in the diagnosis and causation of Miller-Fisher syndrome?

Topics: Autoantibodies; Diagnosis, Differential; Gangliosides; Humans; Myasthenia Gravis; Oculomotor Muscles; Ophthalmoplegia; Polyradiculoneuropathy; Receptors, Cholinergic; Reflex, Abnormal

Miller Fisher syndrome is defined by a triad of symptoms, namely areflexia, ataxia, and ophthalmoparesis. The ophthalmoparesis is mostly severe, undulating weakness of eye movements with ptosis and increased fatigability resembling a neuromuscular transmission disorder. We present a 52-year-old man with severe Miller Fisher syndrome with a high level of anti-GQ1b antibodies and a presynaptic type of neuromuscular transmission disorder. The diagnosis was confirmed by stimulated single-fiber electromyography with the use of a concentric needle electrode and various stimulation rates.

Topics: Ataxia; Autoantibodies; Diplopia; Electromyography; Evoked Potentials, Somatosensory; Gangliosides; Humans; Male; Middle Aged; Miller Fisher Syndrome; Neuromuscular Junction Diseases; Ophthalmoplegia; Reflex, Abnormal

Whether Bickerstaff's brainstem encephalitis (BBE) is a distinct disease or a subtype of Fisher syndrome (FS) is unclear as there have been no clinical studies with sufficiently large numbers of patients with FS or BBE. Our aim was to clarify the nosological relationship. Medical records of patients suffering acute ophthalmoplegia and ataxia within four weeks of onset were reviewed. BBE was the diagnosis for patients with impaired consciousness, FS for those with clear consciousness and areflexia. Clinical features, neuroimages, and laboratory findings were analyzed. Patients were grouped as having BBE (n = 53), FS (n = 466), or as unclassified (n = 62). The BBE and FS groups had similar features; positive serum anti-GQ1b IgG antibody (68 % versus 83 %), antecedent Campylobacter jejuni infection (23 % versus 21 %), CSF albuminocytological dissociation (46 % versus 76 %), brain MRI abnormality (11 % versus 2 %), and abnormal EEG findings (57 % versus 25 %). BBE (n = 4) and FS (n = 28) subgroups underwent detailed electrophysiological testing. Both groups frequently showed absent soleus H-reflexes, but normal sensory nerve conduction (75 % versus 74 %) and a 1-Hz power spectrum peak on postural body sway analysis (67 % versus 72 %). Common autoantibodies, antecedent infections, and MRI and neurophysiological results found in this large study offer conclusive evidence that Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum with variable CNS and PNS involvement.

Topics: Adolescent; Adult; Age Distribution; Aged; Aged, 80 and over; Autoantibodies; Brain Stem; Campylobacter Infections; Causality; Child; Child, Preschool; Electroencephalography; Encephalitis; Female; Gangliosides; Humans; Infant; Infant, Newborn; Japan; Magnetic Resonance Imaging; Male; Middle Aged; Miller Fisher Syndrome; Peripheral Nerves; Reflex, Abnormal; Sex Distribution; Terminology as Topic

Bickerstaff brainstem encephalitis is characterized by the occurrence of ataxia, ophthalmoplegia, motor weakness with areflexia and central nervous system symptoms, with drowsiness, pyramidal syndrome and sensorial symptoms. Diagnosis is based on MR findings and GQ1b antibodies. Treatment is not well known.. We report a patient aged 39 years native of Laos who presented weakness, loss of reflexes, and drowsiness. Brain MR showed hyperintense signals in the brain stem. GQ1b antibodies were positive. The course was characterized by decrease of the weakness, normalization of MR and negativity of GQ1b antibodies.. This observation underlines common features of Bickerstaff brainstem encephalitis, Miller Fisher syndrome and Guillain Barre syndrome. A favorable course and GQ1b antibodies are shared by these syndromes.

Topics: Adrenal Cortex Hormones; Autoantibodies; Autoantigens; Blindness; Brain Stem; Coma; Combined Modality Therapy; Demyelinating Autoimmune Diseases, CNS; Electroencephalography; Encephalitis; Female; Gangliosides; Humans; Magnetic Resonance Imaging; Middle Aged; Optic Atrophy; Plasmapheresis; Quadriplegia; Reflex, Abnormal; Syndrome

The Miller Fisher syndrome, Guillain-Barre syndrome and Bickerstaff's brainstem encephalitis are related conditions in which anti-GQ1b antibody positivity occur in varied frequencies. This report demonstrates the presence of corticobulbar and corticospinal dysfunction in BBE, by means of a novel transcranial magnetic stimulation technique. It further supports the presence of protean manifestations in anti-GQ1b IgG antibody-positive spectrum of disorders.

Topics: Adult; Autoantibodies; Brain Stem; Dysarthria; Encephalitis; Evoked Potentials, Motor; Gangliosides; Guillain-Barre Syndrome; Humans; Hypoglossal Nerve; Hypoglossal Nerve Diseases; Male; Motor Neurons; Neural Conduction; Neurologic Examination; Pyramidal Tracts; Reaction Time; Reflex, Abnormal; Remission, Spontaneous; Syndrome; Tongue; Transcranial Magnetic Stimulation

Topics: Autoantibodies; Blepharoptosis; Diagnosis, Differential; Electromyography; Gangliosides; Humans; Immunoglobulins, Intravenous; Male; Middle Aged; Miller Fisher Syndrome; Motor Neurons; Neuromuscular Junction; Neuromuscular Junction Diseases; Oculomotor Muscles; Ophthalmoplegia; Peripheral Nerves; Predictive Value of Tests; Reflex, Abnormal

The authors reviewed clinical profiles and laboratory findings for 100 cases of abducens nerve paresis without impairment of the other cranial nerves, limb weakness, and ataxia throughout the clinical course. Review of the medical records of 9300 patients referred to our neuoroimmunological laboratory for serum anti-ganglioside antibody testing. Information was obtained from each primary physician on symptoms of preceding infection; initial symptoms; neurological signs during the illness; the clinical course; treatment provided; and outcome. Isolated abducens nerve paresis was present in 100 patients and bilateral paresis in 29. Tentative diagnoses made by the primary physicians on request of anti-ganglioside antibody testing were abducens nerve palsy (n = 68), Fisher syndrome (n = 14), acute ophthalmoparesis without ataxia (n = 14). Symptoms of infection anteceded in 63. Tendon reflexes were absent or decreased in 27. Distal paresthesias were experienced by seven. Serum anti-GQ1b antibody was positive in 25. These findings suggest that some cases of isolated abducens nerve palsy can be categorized as a regional variant of Guillain-Barré syndrome or mild form of Fisher syndrome.

Topics: Abducens Nerve; Abducens Nerve Diseases; Adolescent; Adult; Aged; Aged, 80 and over; Autoantibodies; Child; Child, Preschool; Diagnosis, Differential; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Infant; Infections; Male; Middle Aged; Miller Fisher Syndrome; Plasmapheresis; Predictive Value of Tests; Reflex, Abnormal; Steroids

Anti-GQ1b IgG frequently is present in sera of patients with Miller Fisher syndrome (MFS), Guillain-Barré syndrome (GBS) with ophthalmoplegia, Bickerstaff's brainstem encephalitis (BBE), and acute ophthalmoparesis (AO) in the acute phase. Why various clinical signs develop under these conditions, however, has yet to be clarified. We investigated the fine specificity of anti-GQ1b IgG and its clinical correlation in sera from 82 patients: 56 with MFS, 11 with GBS, 13 with BBE, and 2 with AO. Anti-GQ1b IgG antibodies were absorbed by GT1a in 80 (98%) of the 82 sera, by GD1b in 11 (13%), and by the other b-series gangliosides GD3, GD2, or GT1b in 24 (29%). The most frequent pattern of fine specificity was the cross-reaction with GT1a alone, seen in 56 (68%) samples. Of the 11 patients with anti-GQ1b IgG, cross-reacting with GD1b, 6 (55%) had impaired deep sense, and the association was significant (p=0.02). This is the first study to show that the fine specificity of anti-GQ1b IgG is heterogeneous and that the difference is correlated with the presence of a particular clinical sign.

Topics: Antibody Specificity; Autoantibodies; Carbohydrate Sequence; Gangliosides; Humans; Miller Fisher Syndrome; Molecular Sequence Data; Ophthalmoplegia; Reflex, Abnormal; Somatosensory Disorders

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

Circulating IgG antibodies to carbohydrate determinants on GQ1b ganglioside are found in the acute phase sera of patients with Miller Fisher syndrome, a variant of Guillain-Barré syndrome. Here we report that the IgG subclass distribution of the anti-GQ1b antibodies is mainly restricted to IgG1 and IgG3 antibodies, subclasses typically associated with a T cell-dependent immune response to protein antigens. This is highly unusual in that IgG responses to carbohydrate determinants are typically of the IgG2 subclass. Anti-GQ1b antibodies also have a limited ability to bind GQ1b in a membrane-like environment, particularly at body temperature. These data suggest that the antigen initiating the immune response in MFS is not likely GQ1b but an unidentified cross-reactive glycoprotein antigen(s). Similar results were obtained for anti-GM1 IgG antibodies in Guillain-Barré syndrome.

Topics: Antibodies; Antibody Formation; Ataxia; Body Temperature; Drug Carriers; Gangliosides; Glycolipids; Humans; Immunoglobulin G; Immunoglobulins; Liposomes; Nerve Growth Factors; Oligoclonal Bands; Ophthalmoplegia; Reflex, Abnormal; Syndrome

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

We studied serum anti-glycolipid antibodies by enzyme-linked immunosorbent assay and thin-layer chromatography-enzyme immunoassay in six consecutive patients with typical Miller Fisher syndrome. In all six, increased activity of IgG antibody against ganglioside GQ1b was present in the early phase and reduced with time, whereas such activity was not detected in normal control subjects and disease control subjects including those with Guillain-Barré syndrome. Anti-GQ1b IgG antibody is a new possible diagnostic marker of Miller Fisher syndrome and could well be related to the disease process itself.

Topics: Adult; Aged; Antibody Specificity; Ataxia; Autoantibodies; Autoimmune Diseases; Biomarkers; Female; Gangliosides; Glycolipids; Humans; Immunoglobulin G; Male; Middle Aged; Multiple Sclerosis; Ophthalmoplegia; Polyradiculoneuropathy; Reflex, Abnormal; Syndrome