Journal Article

DZNE-2020-03376

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Progressive cognitive dysfunction in spinocerebellar ataxia type 3.

Röske, S. (First author)DZNE* ; Filla, I. ; Heim, S. ; Amunts, K. ; Helmstaedter, C. ; Wüllner, U. ; Wagner, M. ; Klockgether, T.DZNE* ; Minnerop, M.

2013
Wiley New York, NY

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Please use a persistent id in citations: doi:10.1002/mds.25512

Abstract: Although it is well established that there is cognitive dysfunction in spinocerebellar ataxia type 3 (SCA3), it is unknown whether cognition deteriorates with disease progression. We therefore prospectively studied cognitive function in patients with SCA3.Eleven patients with SCA3 were assessed using an extensive neuropsychological test battery and retested after 3.5 ± 0.4 years.In addition to ataxia and motor control, verbal learning and verbal and figural memory deteriorated significantly during the follow-up period. An increase in depressive symptoms was not observed.The observation that memory and learning abilities deteriorated with disease progression suggests that cognitive dysfunction is an integral part of SCA3. Because the applied tests for memory function did not require motor responses, cognitive decline cannot be attributed to progressive cerebellar ataxia. The deterioration of verbal and figural memory can be explained either by extracerebellar pathology or by disruption of cerebellar-cerebral circuitries.

Keyword(s): Adult (MeSH) ; Analysis of Variance (MeSH) ; Cognition Disorders: etiology (MeSH) ; Cognition Disorders: psychology (MeSH) ; Depression: etiology (MeSH) ; Depression: psychology (MeSH) ; Disease Progression (MeSH) ; Female (MeSH) ; Follow-Up Studies (MeSH) ; Humans (MeSH) ; Machado-Joseph Disease: complications (MeSH) ; Machado-Joseph Disease: genetics (MeSH) ; Machado-Joseph Disease: psychology (MeSH) ; Male (MeSH) ; Memory: physiology (MeSH) ; Middle Aged (MeSH) ; Neurologic Examination (MeSH) ; Neuropsychological Tests (MeSH) ; Prospective Studies (MeSH) ; Psychiatric Status Rating Scales (MeSH) ; Reaction Time: physiology (MeSH) ; Verbal Behavior: physiology (MeSH)

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Contributing Institute(s):

1. Neuropsychology (AG Wagner)
2. Patient Studies (Bonn) (Patient Studies (Bonn))

Research Program(s):

1. 344 - Clinical and Health Care Research (POF3-344) (POF3-344)

Appears in the scientific report 2013

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Database coverage:
; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Current Contents - Life Sciences ; IF >= 5 ; JCR ; NCBI Molecular Biology Database ; Nationallizenz ; SCOPUS ; Science Citation Index ; Science Citation Index Expanded ; Web of Science Core Collection

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