Adrenomyeloneuropathy and Adrenoleukodystrophy in Two Brothers

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Abstract
A 19 yr old patient with spastic paraparesis and peripheral neuropathy without clinical or biochemical signs of adrenal insufficiency is presented. The diagnosis of adrenomyeloneuropathy was supported by the typical ultrastructural of peripheral nerve and endomysial nerve bundles and a positive family history, e.g., his younger brother died at age 12 from a demyelinating disease of the CNS. Cutaneous nerve bundles and the postmortem brain demonstrated features specific for adrenoleukodystrophy. The various features of both disorders are discussed. Their occurrence in the same sibship pleads strongly for a very close relationship.

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