Neurogenic muscular atrophy and low density of large myelinated fibres of sural nerve in chorea-acanthocytosis.

Article, Other literature type English OPEN
Ohnishi, A ; Sato, Y ; Nagara, H ; Sakai, T ; Iwashita, H ; Kuroiwa, Y ; Nakamura, T ; Shida, K (1981)
  • Publisher: BMJ Publishing Group
  • Journal: Journal of Neurology, Neurosurgery, and Psychiatry, volume 44, issue 7, pages 645-648 (issn: 0022-3050)
  • Subject: Research Article | chorea-acanthocytosis | Neurology | 神経学
    mesheuropmc: nervous system diseases

In three cases of chorea-acanthocytosis (acanthocytosis and neurological disease, or familial degeneration of the basal ganglia with acanthocytosis), biopsies of short peroneal muscles and sural nerves were studied histologically. The muscles showed groups of atrophic fibres with clumping of sarcolemmal nuclei in all cases. It was concluded that neurogenic muscular atrophy should be included as one of the main pathological findings in chorea-acanthocytosis. The sural nerves showed a small number of large myelinated fibres in two cases. This finding remains to be confirmed in other cases.
Share - Bookmark