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-Jak...

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Published in:Journal of neurology, neurosurgery and psychiatry neurosurgery and psychiatry, 1994-10, Vol.57 (10), p.1249-1251
Main Authors: Jendroska, K, Hoffmann, O, Schelosky, L, Lees, A J, Poewe, W, Daniel, S E
Format: Article
Language:English
Subjects:
Biological and medical sciences
Brain - pathology
Brain Diseases - pathology
Creutzfeldt-Jakob Syndrome - pathology
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Dementia - pathology
Humans
Immunohistochemistry
Medical sciences
Nerve Degeneration
Neurology
Parkinson Disease - pathology
Prions - analysis
Supranuclear Palsy, Progressive - pathology
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Summary: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.
ISSN:0022-3050
1468-330X
DOI:10.1136/jnnp.57.10.1249