Adrenomyeloneuropathy: report of a family and electron microscopical findings in peripheral nerve

Abstract

A family with adrenomyeloneuropathy (AMN) is reported, whose female carriers showed severe neurological and biochemical abnormalities. Cytoplasmic lamellar inclusions were found in endoneurial cells of the biopsied sural nerve of a male patient. In spite of the peripheral nervous system involvement in patients with AMN, there have been few reports of these inclusions in the peripheral nerves of such cases.