Peroneal muscular atrophy with pyramidal tract features (hereditary motor and sensory neuropathy type V): a clinical, neurophysiological, and pathological study of a large kindred.
A large family with autosomal dominant inheritance of peroneal muscular atrophy, associated with extensor plantar responses in some cases, has been studied. Onset was usually in the first two decades and spasticity was not a feature. Nerve conduction studies in 21 cases and light and electron microscope findings on six sural nerve biopsies were similar to those in hereditary motor and sensory neuropathy type II.
Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, University of Sydney, Australia.
J A
JA
Frith
J G
JG
McLeod
G A
GA
Nicholson
F
F
Yang
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D006801:Humans; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000503:physiopathology*; D009133:Muscular Atrophy / Q000235:genetics* / Q000503:physiopathology*; D009431:Neural Conduction; D010375:Pedigree; D013497:Sural Nerve / Q000503:physiopathology; D014459:Ulnar Nerve / Q000503:physiopathology