A prion-linked psychiatric disorder

Abstract

The finding that new variant Creutzfeldt-Jakob disease, the recently discovered prion disease thought to be associated with ingestion of bovine offal¹, has a prolonged course and can present with psychiatric symptoms² raised the possibility that hereditary forms of prion disease may also be associated with serious psychiatric disorders. We have sequenced the open reading frame of the prion protein (PrP) gene isolated from 10 patients with a strong family history of psychiatric illness. In one patient we discovered a previously undescribed sequence alteration, N171S, which raises the possibility of a significant extension in the pathologies associated with inherited prion diseases.