Longitudinal conduction studies in hereditary motor and sensory neuropathy type 1.
Motor conduction studies were performed serially in 10 patients, ages 10-62 years, with clinical and electrophysiological criteria of hereditary motor and sensory neuropathy type 1 (HMSN-1) over periods of 11-19 years. Median nerve conduction velocity (MNCV) and distal motor latency showed no significant change on serial studies. Mean median compound muscle action potential (CMAP) amplitude values, however, decreased 66% in 8 patients. Observed clinical progression in HMSN-1, over prolonged periods of time, was not associated with MNCV slowing. However, CMAP amplitude reduction, reflecting progressive axonal loss, correlated with clinical deterioration.
Roy
E P
EP
Department of Neurology, West Virginia University School of Medicine, Morgantown.
Gutmann
L
L
Riggs
J E
JE
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D004594:Electrophysiology; D005260:Female; D015417:Hereditary Sensory and Motor Neuropathy / Q000503:physiopathology*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008475:Median Nerve / Q000502:physiology*; D008875:Middle Aged; D009431:Neural Conduction