Occurrence of adrenocortical insufficiency in adrenomyeloneuropathy

Abstract

Adrenoleukodystrophy and its clinical variant adrenomyeloneuropathy (AMN) are disorders due to peroxisomal degradation of very-long-chain fatty acids (VLCFA). Both forms are characterized by CNS involvement and variable involvement of endocrine functions, especially the adrenal cortex. [1] It has been a matter of debate whether adrenocortical insufficiency (Addison's disease) may precede or follow the onset of neurologic symptoms. [2,3] We evaluated the onset of symptoms of adrenocortical insufficiency and of neurologic symptoms using a questionnaire supported by the German ``Informationsnetzwerk Leukodystrophie, Bonn.'' Items of adrenocortical insufficiency were fatigue, hyperpigmentation, nausea, and vomiting and items of neurologic involvement were muscle weakness, spasticity, and sensory disturbances. The questionnaire was returned by 41 male patients, ranging in age from 18 to 59 years …