CREUTZFELDT-JAKOB-DISEASE - PRESENTATION OF A CASE WITH AN UNUSUALLY LONG CLINICAL COURSE AND A SHORT REVIEW OF THE LITERATURE

The human spongiform encephalopathies are a group of neurodegenerative disorders of unknown origin. They comprise a group of horizontally tr ansmissible and genetically determined diseases. We present here a cas e of Creutzfeldt-Jakob disease with an unusually long clinical course, in which the prion protein was localized immunohistochemically. The g enerally accepted hypothesis on the pathogenesis of these diseases is the so-called 'prion-hypothesis'. The implications of this hypothesis are discussed and a short review of the literature is given.