T. Bogumil et al., CREUTZFELDT-JAKOB-DISEASE - PRESENTATION OF A CASE WITH AN UNUSUALLY LONG CLINICAL COURSE AND A SHORT REVIEW OF THE LITERATURE, Nervenarzt, 65(12), 1994, pp. 865-873
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.