Absence of disease related prion protein in neurodegenerative disorders presenting with Parkinson's syndrome

J Neurol Neurosurg Psychiatry. 1994 Oct;57(10):1249-51. doi: 10.1136/jnnp.57.10.1249.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Brain / pathology*
  • Brain Diseases / pathology
  • Creutzfeldt-Jakob Syndrome / pathology
  • Dementia / pathology
  • Humans
  • Immunohistochemistry
  • Nerve Degeneration
  • Parkinson Disease / pathology*
  • Prions / analysis*
  • Supranuclear Palsy, Progressive / pathology

Substances

  • Prions