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July 1953

PROGRESSIVE NEUROPATHIC (PERONEAL) MUSCULAR ATROPHY (CHARCOT-MARIE-TOOTH DISEASE): Histological Findings in Muscle Biopsy Specimens in Fourteen Cases, with Notes on Clinical Diagnosis and Familial Occurrence

Author Affiliations


From the Neurological University Clinic, Rikshospitalet (Series 2, No. 200) and the Anatomical Institute, University of Oslo.

AMA Arch NeurPsych. 1953;70(1):1-29. doi:10.1001/archneurpsyc.1953.02320310007001

THERE seems to be a general feeling among neurologists that one is justified in considering as a disease entity the condition variously called "peroneal muscular atrophy of the Charcot-Marie-Tooth type," "progressive neuritic muscular atrophy," or "spinal neuritic form of progressive muscular atrophy." The weight of available evidence concerning its etiology, most recently reviewed by England and Denny-Brown,1 is in favor of an affection of the spinal cord and of peripheral nerves being the primary process, the muscular changes being secondary; but few autopsy reports are found in the literature. However, biopsy of affected muscles may give information of interest, since it is now well established that the histological changes in the muscles in progressive muscular dystrophies differ in certain respects from those occurring in diseases involving primarily the peripheral motor neurons, such as amytrophic lateral sclerosis, progressive spinal muscular atrophy, syringomyelia, and poliomyelitis.2 Brodal and Refsum3 described

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