[Creutzfeldt-Jakob disease. Report of a case with an unusually long course and immunohistochemical localization of the prion protein and overview of current information].
The human spongiform encephalopathies are a group of neurodegenerative disorders of unknown origin. They comprise a group of horizontally transmissible and genetically determined diseases. We present here a case of Creutzfeldt-Jakob disease with an unusually long clinical course, in which the prion protein was localized immunohistochemically. The generally 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.
Bogumil
T
T
Institut für Neuropathologie der Universität Göttingen.
Beuche
W
W
Schindler
C
C
Schachenmayr
W
W
Kretzschmar
H A
HA
D001284:Atrophy; D001479:Basal Ganglia / Q000473:pathology; D001921:Brain / Q000473:pathology*; D002540:Cerebral Cortex / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D004252:DNA Mutational Analysis; D005260:Female; D006801:Humans; D008875:Middle Aged; D009410:Nerve Degeneration / Q000235:genetics / Q000502:physiology; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D011328:Prions / Q000032:analysis* / Q000235:genetics
D011328:Prions
Die Creutzfeldt-Jakob-Krankheit. Bericht über einen Fall mit ungewöhnlich langem Verlauf und immunhistochemischer Lokalisierung des Prionproteins sowie Uberblick über den heutigen Wissensstand.