Adrenomyeloneuropathy is a rare disease in which neurological deficit is associated with Addison's disease. Patients may have both central and peripheral demyelination. The clinical picture may be one of a spastic paraparesis associated with a peripheral neuropathy, and diagnosis may be made by analysis of long-chain fatty acids in cultured skin fibroblasts. In contrast to adrenoleucodystrophy which begins in childhood, adrenomyeloneuropathy starts in adulthood.
These conditions are inherited as sex-linked recessive states, and Addison's disease may precede the neurological deficit in both disorders. When adrenomyeloneuropathy develops, it may produce subtle changes such as lack of concentration, behavioural disturbance, or a compulsive disorder, before more obvious focal signs are found.
