Absence of disease related prion protein in neurodegenerative disorders presenting with Parkinson's syndrome.
Movement disorders presenting with parkinsonism may share histopathological features with Creutzfeldt-Jakob disease, a spongiform encephalopathy caused by the accumulation of pathological prion protein in brain. To investigate a possible aetiological link between these conditions and Creutzfeldt-Jakob disease, histoblot immunostaining for pathological prion protein was carried out in 90 cases including idiopathic Parkinson's disease, multiple system atrophy, diffuse Lewy body disease, Steele-Richardson-Olszewski syndrome, corticobasal degeneration, and Pick's disease. Pathological prion protein was identified in four controls with Creutzfeldt-Jakob disease but not in any of the other diseases examined. The findings suggest that an aetiological role for prions in these movement disorders is unlikely. Histoblotting provides a useful method for screening large areas of tissue for the presence of pathological prion protein and may be helpful in the differential diagnosis of difficult cases.
Jendroska
K
K
Department of Neurology, Universitätsklinikum Rudolf Virchow, Berlin, Germany.
Hoffmann
O
O
Schelosky
L
L
Lees
A J
AJ
Poewe
W
W
Daniel
S E
SE
D001921:Brain / Q000473:pathology*; D001927:Brain Diseases / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology; D003704:Dementia / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D009410:Nerve Degeneration; D010300:Parkinson Disease / Q000473:pathology*; D011328:Prions / Q000032:analysis*; D013494:Supranuclear Palsy, Progressive / Q000473:pathology
D011328:Prions