Psychiatric presentation of Jakob-Creutzfeldt disease

A case of presenile dementia, which on a post-mortem examination proved to be due to Jakob-Creutzfeldt Disease, is described. The most dramatic early clinical feature was that of hysterical aphonia, temporarily removable by hypnotic suggestion. Anxiety symptoms and irritability also occurred early on, followed by a transient hallucinatory psychosis. The hysterical features persisted throughout. In view of the lack of dementing features or neurological signs, a psychogenic diagnosis was repeatedly made by several neurologists. This case illustrates the fact that primary hysterical neurotic features almost never occur for the first time in mid-life.