The nigrostriatal pathway in Creutzfeldt-Jakob disease.

Anne Vital, Pierre-Olivier Fernagut, Marie-Hélène Canron, Julien Joux, Erwan Bezard, Marie-Laure Martin-Negrier, Claude Vital, François Tison
J Neuropathol Exp Neurol. 2009-07-01; 68(7): 809-815
DOI: 10.1097/nen.0b013e3181abdae8

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1. J Neuropathol Exp Neurol. 2009 Jul;68(7):809-15. doi:

The nigrostriatal pathway in Creutzfeldt-Jakob disease.

Vital A(1), Fernagut PO, Canron MH, Joux J, Bezard E, Martin-Negrier ML, Vital C,
Tison F.

Author information:
(1)Université Victor Segalen-Bordeaux 2, Centre National de la Recherche
Scientifique, Bordeaux Institute of Neuroscience, Bordeaux University Hospital,
Bordeaux, France.

Parkinsonism, chorea, and dystonia are well-known clinical manifestations of
Creutzfeldt-Jakob disease (CJD), but lesions of the nigrostriatal pathway have
never been thoroughly studied. We performed a detailed neuropathologic study of
the nigrostriatal pathway in 15 sporadic CJD and 2 variant CJD cases that
included clinical correlations and assessment of neuron subtype loss,
distribution of prion protein, alpha-synuclein, ubiquitin, and 14-3-3
aggregation. We found evidence of nigrostriatal pathway damage in these CJD
cases. Dopaminergic neurons and striatal outflow neurons were markedly affected
in sporadic CJD, whereas cholinergic interneurons were spared. In cases of CJD
with chorea or myoclonus, there was less presynaptic dopaminergic loss than in
cases of CJD with parkinsonism. The 2 variant CJD cases with parkinsonism or
chorea showed severe cholinergic interneuron loss in the caudate and putamen, a
pattern that differed from that found in sporadic CJD. alpha-Synuclein,
ubiquitin, and 14-3-3 aggregation coexisted with prion protein aggregation,
thereby generating mixed pathological features. These findings suggest a possible
pathophysiological overlap of abnormal protein aggregation in CJD and Parkinson

DOI: 10.1097/NEN.0b013e3181abdae8
PMID: 19535991 [Indexed for MEDLINE]

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