title: Electrophysiological study of four patients with Fisher syndrome: a mechanism of areflexia.
abstract: Electrophysiological studies were performed on four patients with the Fisher syndrome (two 4-year-old boys, a 5-year-old boy and a 9-year-old girl). Motor nerve conduction velocity (MNCV), nerve action potential, F wave, H reflex and T reflex were measured at the stage when areflexia was present but ataxia and ophthalmoplegia had recovered. MNCV and SNCV by compound mixed nerve potential were normal in all our patients. The amplitude of the muscle responses (M-responses) and compound mixed nerve action potentials were also within normal limits. The latencies of the F waves and F wave conduction velocities were normal. However, H-reflexes and T-reflexes were absent in all four cases. Our results suggested that areflexia in the Fisher syndrome is attributed to the desynchronization of the impulses or the partial conduction block of GIa fibers.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Fukuoka University, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Mitsudome
authors.forename: H
authors.initials: H
authors.lastname: Ogata
authors.forename: M
authors.initials: M
authors.lastname: Ohfu
authors.forename: K
authors.initials: K
authors.lastname: Irie
authors.forename: H
authors.initials: H
authors.lastname: Hirano
mesh_terms: D001259:Ataxia / Q000134:cerebrospinal fluid / Q000503:physiopathology*; D002556:Cerebrospinal Fluid Proteins / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006181:H-Reflex / Q000502:physiology; D006801:Humans; D008297:Male; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction; D009475:Neurons, Afferent / Q000502:physiology; D009886:Ophthalmoplegia / Q000134:cerebrospinal fluid / Q000503:physiopathology*; D012018:Reflex / Q000502:physiology*; D013577:Syndrome
chemical_list: D002556:Cerebrospinal Fluid Proteins