Article Text


Pathology of peroneal muscular atrophy (Charcot-Marie-Tooth disease)
  1. J. Trevor Hughes,
  2. Betty Brownell
  1. Department of Neuropathology, Radcliffe Infirmary, Oxford
  2. The Burden Neuropathological Laboratory, Frenchay Hospital, Bristol


    Four cases of peroneal muscular atrophy (Charcot-Marie-Tooth disease) are described in which observations were made from the necropsy examination of the central nervous system, the peripheral nervous system, and the skeletal muscles. The pattern of nerve degeneration in these various locations is described and the conclusion is drawn that the basis of the disease is a very chronic progressive neuropathy. To the pathological descriptions of previous observers is added the finding of fibrosis affecting the muscle spindles which was present in all four of the cases. The literature of the case reports with necropsy examination is reviewed.

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