Single-fiber electromyography shows terminal axon dysfunction in Miller Fisher syndrome: a case report.
We studied a patient with ophthalmoparesis and pupillary areflexia 2 weeks after a viral syndrome. Miller Fisher syndrome was suspected but GQ1b antibodies were not detected. To define neuromuscular involvement we performed electrodiagnostic studies. Single-fiber electromyography (SFEMG) in the extensor digitorum communis (EDC) showed abnormal jitter and axonal blocking, suggesting terminal axon dysfunction. Subsequent GQ1b antibody titers were elevated to borderline levels. Clinical symptoms gradually resolved. SFEMG may help characterize neuropathies associated with antibodies to neuronal ganglioside and identify involvement of the terminal axon and neuromuscular junction.
Department of Neurology, Mt. Sinai School of Medicine, One Gustave L. Levy Place, Box 1052, New York, New York 10029, USA. dale.lange@mssm.edu
Dale J
DJ
Lange
Tracy
T
DeAngelis
Mark A
MA
Sivak
D001369:Axons / Q000502:physiology*; D004576:Electromyography; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D008297:Male; D008875:Middle Aged; D019846:Miller Fisher Syndrome / Q000503:physiopathology*; D018485:Muscle Fibers, Skeletal / Q000502:physiology*; D009431:Neural Conduction / Q000502:physiology; D009460:Neurologic Examination; D009469:Neuromuscular Junction / Q000502:physiology; D015835:Ocular Motility Disorders / Q000503:physiopathology; D004856:Postural Balance / Q000502:physiology; D012027:Reflex, Vestibulo-Ocular / Q000502:physiology; D009435:Synaptic Transmission
D005732:Gangliosides; C039129:GQ1b ganglioside