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Title of Journal: Acta Neuropathol

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Abbravation: Acta Neuropathologica

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Springer-Verlag

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10.1016/0730-725x(89)90531-6

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1432-0533

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Occurrence of αsynuclein pathology in the cerebel

Authors: Joseph Sebeo Patrick R Hof Daniel P Perl
Publish Date: 2004/03/16
Volume: 107, Issue: 6, Pages: 497-503
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Abstract

Amyotrophic lateral sclerosis/parkinsonismdementia complex ALS/PDC is a progressive neurodegenerative disease affecting the indigenous Chamorro population of Guam Neuropathologically PDC is characterized by neuronal loss in the substantia nigra pars compacta with severe widespread neurofibrillary tangles NFTs similar to those observed in Alzheimer’s disease AD and is thus considered a tauopathy Following reports of αsynuclein pathology in PDC patients of Guam PDC has also been neuropathologically classified as a synucleinopathy Recently the presence of αsynucleinpositive bodies has been reported in the cerebellum of some patients with Parkinson’s disease PD diffuse Lewy body disease DLBD or multiple system atrophy MSA Using immunohistochemical techniques we investigated the deposition of αsynuclein in the cerebellum of Guamanian PDC patients Numerous αsynucleinimmunoreactive spherical structures were found in the molecular layer of the cerebellum of 636 of PDC patients These structures were only seen in patients showing αsynuclein pathology in the amygdala The average density of αsynucleinimmunoreactive structures in the cerebellum of Guamanian PDC patients was almost an order of magnitude higher than in nonGuamanian PD patients and this αsynuclein pathology was much more pronounced in the hemisphere than in the vermis In addition double immunohistochemistry revealed that cerebellar αsynuclein is colocalized with the neuronal marker calbindin and with glialfibrillary acidic protein suggesting the involvement of Purkinje cells and Bergmann glia These findings demonstrate that the αsynuclein pathology in PDC of Guam affects not only the amygdala but also the cerebellum where it appears to involve both Purkinje cells and specialized astrocytes

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