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Longitudinal conduction studies in hereditary motor and sensory neuropathy type 1

✍ Scribed by Dr. E. Pierre Roy III; Ludwig Gutmann; Jack E. Riggs

Publisher: John Wiley and Sons
Year: 1989
Tongue: English
Weight: 315 KB
Volume: 12
Category: Article
ISSN: 0148-639X
DOI: 10.1002/mus.880120110

No coin nor oath required. For personal study only.

✦ Synopsis

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 arnplitude reduction, reflecting progressive axonal loss, correlated with clinical deterioration.

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