Congenital hypo- and hypermyelination neuropathy. Two cases.

Acta Neuropathol

Department of Neurology, University Hospital, Limoges, France.

Published: December 1987

Two young patients were referred recently to the authors for investigation of a peroneal atrophy syndrome. Since the first symptoms were observed in infancy, a congenital hypomyelination neuropathy was suspected, and superficial peroneal nerve biopsies were taken. Signs of severe and widespread demyelination/remyelination were observed. These features appeared morphologically similar to those observed in the globular or tomaculous neuropathies. The mechanism of the hypermyelination is discussed.

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http://dx.doi.org/10.1007/BF00692853DOI Listing

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