The peroneal muscular atrophy syndrome. Clinical genetic, electrophysiological and nerve biopsy studies. Part 3. Clinical, electrophysiological and pathological correlations.
This report analyses correlations between clinical, electrophysiological and pathological data derived from a series of families with peroneal muscular atrophy. It was found that the observed differences between families in median nerve forearm motor conduction velocity were unlikely to be due to differences in the age or severity of the cases. Similarly it it was unlikely that the pathological differences between cases were due to age or severity of the case. The conduction velocity in the Hypertrophic Neuropathy Group tended to increase slightly with age, while that in other cases tended to fall slightly. The conduction velocity and total myelinated fibre counts were inversely related to the degree of segmental demyelination. The Hypertrophic Neuropathy Group and the Intermediate Group of cases were found to behave differently in a number of correlative analyses, thus supporting the suggestion that they represent different disease entities.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D001369:Axons / Q000473:pathology; D002648:Child; D003711:Demyelinating Diseases / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008475:Median Nerve / Q000473:pathology / Q000503:physiopathology; D008875:Middle Aged; D009133:Muscular Atrophy / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D009186:Myelin Sheath / Q000473:pathology; D009431:Neural Conduction; D013577:Syndrome