Adrenocortical insufficiency and diffuse cerebral sclerosis. Problems of presentation and diagnosis

Three cases of adrenocortical insufficiency with diffuse cerebral sclerosis are described. One, which was confirmed by brain biopsy, had a family history of the combined disorder affecting members of a previous generation; this has not been previously reported; however pathological confirmation of the diagnosis in the earlier cases was not available. Two cases presented as acute neurosurgical emergencies and attention is drawn to this mode of presentation. Problems of aetiology, presentation and diagnosis are discussed and the finding of normal basal steroid levels is stressed.