gq1b-ganglioside and Optic-Neuritis

To establish the sensitivity and specificity of serum and CSF antibodies targeting the gangliosides GQ1b (GQ1bAb) in isolated ophthalmologic syndromes, such as acute ophthalmoplegia (AO) and optic neuritis (ON), caused by disorders other than Miller-Fisher syndrome (MFS).. We measured serum and CSF GQ1bAb in patients with MFS and with AO or ON caused by other disorders than MFS.. Twenty-one patients with AO (21 serum, 9 CSF), 13 with ON (13 serum, 13 CSF), and 12 with MFS (12 serum, 10 CSF) were included in the study. There were no significant differences in age, sex, and CSF findings between the AO and MFS groups. Elevated serum GQ1b titers occurred in 11 of 12 patients with MFS but in only 1 of the 34 patients without MFS. Sensitivity was 92% (95% confidence interval [CI] 62%-100%) and specificity 97% (95% CI 85%-100%). In CSF, GQ1bAb were identified in 2 of 10 patients with MFS but in none with other disorders. Sensitivity was 20% (95% CI 2%-56%) and specificity 100% (95% CI 85%-100%).. Increased serum GQ1bAb are highly specific for MFS. Measurement of GQ1bAb in CSF does not improve diagnosis.. This study provides Class III evidence that serum GQ1bAb accurately distinguish MFS from other disorders (sensitivity 92%, 95% CI 62%-100%; specificity 97%, 95% CI 85%-100%).

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Biomarkers; Diagnosis, Differential; Enzyme-Linked Immunosorbent Assay; Female; Gangliosides; Humans; Immunoglobulin G; Immunoglobulin M; Immunotherapy; Male; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia; Optic Neuritis; Retrospective Studies; Sensitivity and Specificity; Young Adult

Topics: Adult; Autoantibodies; Brain; Gangliosides; Humans; Magnetic Resonance Imaging; Male; Optic Neuritis

A 31-year-old man had optic neuritis 2 weeks after a diarrheal illness, followed by several deficits including palatal dysarthria, diplopia, ataxia, sensory dysfunction, and mild dysautonomia. Brain MRI and CSF were normal. Nerve conduction studies were initially normal and subsequently showed mild reduction in sensory amplitudes. Anti-GQ1b IgG titer was positive. Deficits resolved after treatment with IVIg. This clinical constellation represents an overlap between Miller Fisher syndrome (MFS) and the pharyngeal-cervical-brachial (PCB) variant of Guillain-Barre syndrome (GBS), along with the infrequently reported central feature of optic neuritis. Campylobacter jejuni enteritis may have triggered the syndrome by molecular mimicry. GQ1b antibodies are associated with MFS, GBS, Bickerstaff brainstem encephalitis and PCB; they form an overlapping spectrum of features, hence the anti-GQ1b syndrome.

Topics: Abducens Nerve Diseases; Adult; Diarrhea; Dysarthria; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Magnetic Resonance Imaging; Male; Neurologic Examination; Optic Neuritis; Palate; Speech Disorders

Topics: Adult; Antibodies; Female; G(M1) Ganglioside; Gangliosides; Humans; Miller Fisher Syndrome; Optic Neuritis; Reaction Time; Recurrence