Abstract
Motor conduction velocity was examined in twenty five cases of various neurological dege nerative diseases, and peripheral nerve involve ment in these cases was discussed by comparing motor conduction velocitiy with clinical features and electromyographic findings.
1. Significant reduction in motor conduction velocity was noted in two cases of metachro matic leucodystrophy, a case with globoid cell leucodystrophy and one with Friedreich's ataxia.
2. Electromyography showed finding sugges tive of neurogenic process in Sandhoff's disease, metachromatic leucodystrophy, globoid cell leucodystrophy, Friedreich's ataxia, Fabry's disease and infantile neuroaxonal dystrophy.
3. Cerebrospinal fluid examination revealed albuminocytologic dissociation in two cases of metachromatic leucodystrophy and a case with globoid cell leucodystrophy.
4. It was concluded that there exist peripheral neuropathies in metachromatic leucodystrophy, globoid cell leucodystrophy, Friedreich's ataxia, infantile neuroaxonal dystrophy and Fabry's disease.
Further investigation seems to be necessary on peripheral nerve invlovement of other lipidoses and ataxia telangiectasia.
