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진행성 인지장애를 첫 증상으로 보인 치아적핵창백핵루이체위축증 1예Dentatorubropallidoluysian Atrophy Presenting Progressive Cognitive Dysfunction as an Initial Symptom

Other Titles
Dentatorubropallidoluysian Atrophy Presenting Progressive Cognitive Dysfunction as an Initial Symptom
Authors
박종규고지애박정호이태경성기범박선아
Issue Date
2010
Publisher
대한치매학회
Keywords
Key Words: DRPLA; Cognitive symptoms; Atrophin-1
Citation
Dementia and Neurocognitive Disorders(대한치매학회지), v.9, no.2, pp 64 - 68
Pages
5
Journal Title
Dementia and Neurocognitive Disorders(대한치매학회지)
Volume
9
Number
2
Start Page
64
End Page
68
URI
https://scholarworks.bwise.kr/sch/handle/2021.sw.sch/18353
ISSN
1738-1495
2384-0757
Abstract
A 50-yr-old woman presented with progressive cognitive impairment and later developed abnormal movements. Brain magnetic resonance imaging (MRI) showed diffuse atrophy of the cerebellum and brainstem and severe high signal intensities in the periventricular white matter on T2-weighted images. The genetic analysis confirmed the diagnosis of dentatorubropallidoluysian atrophy (DRPLA). DRPLA should be considered in a patient with family history who showed cognitive impairment alone for a substantial period and severe white matter involvement with diffuse atrophy on MRI.
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