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Congenital hypo- and hypermyelination neuropathy

Two cases

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Summary

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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Vallat, J.M., Gil, R., Leboutet, M.J. et al. Congenital hypo- and hypermyelination neuropathy. Acta Neuropathol 74, 197–201 (1987). https://doi.org/10.1007/BF00692853

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  • DOI: https://doi.org/10.1007/BF00692853

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