Adrenocortical atrophy and diffuse cerebral sclerosis (Addison-Schilder's disease)

Abstract

Clinical histopathologic observations and studies of steroid excretion are described ina 5-year-old Negro male in whom classical Addison's disease preceded the development of diffuse cerebral sclerosis (Schilder's disease). Although the first reported in this country, the patient described here is the eleventh in whom the coexistence of these diseases has been documented. The frequency with which the concurrence of adrenocortical atrophy and Schilder'sdisease has been described, its appearance as a familial illness, and other characteristic clinical features suggest that the association represents a distinct clinical syndrome.