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01-08-2012 | Original Communication

Is cerebral white matter involvement helpful in the diagnosis of dentatorubral-pallidoluysian atrophy?

Authors: Won Tae Yoon, Jinyoung Youn, Jin Whan Cho

Published in: Journal of Neurology | Issue 8/2012

Abstract

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disease clinically characterized by the presence of cerebellar ataxia in combination with variable neurological symptoms. Cerebral white matter involvement of DRPLA is rare and reported mainly in severe, progressed cases of old-aged or juvenile-onset DRPLA. We describe three cases of genetically confirmed DRPLA that developed changes in cerebral white matter in the early stage of middle-aged patients. Our results of our study indicate that cerebral white matter changes are not rare in DRPLA and might be helpful for differentiation in ataxia patients with brainstem and cerebellum atrophy.

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Title: Is cerebral white matter involvement helpful in the diagnosis of dentatorubral-pallidoluysian atrophy?
Authors: Won Tae Yoon
Jinyoung Youn
Jin Whan Cho
Publication date: 01-08-2012
Publisher: Springer-Verlag
Published in: Journal of Neurology / Issue 8/2012
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-011-6401-6

At a glance: The STEP trials

Springer Medicine

Is cerebral white matter involvement helpful in the diagnosis of dentatorubral-pallidoluysian atrophy?

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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