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dc.date.accessioned2014-01-08T16:12:16Z
dc.date.available2014-01-08T16:12:16Z
dc.date.created2013-12-09T18:49:20Z
dc.date.issued2013
dc.identifier.citationWedding, Iselin Marie Koht, Jeanette Dietrichs, Espen Landrø, Nils Inge Tallaksen, Chantal . Cognition is only minimally impaired in Spinocerebellar ataxia type 14 (SCA 14): a neuropsychological study of ten Norwegian subjects compared to intrafamilial controls and population norm. BMC Neurology. 2013
dc.identifier.urihttp://hdl.handle.net/10852/37954
dc.description.abstractBackground: There is an increasing awareness of the role of the cerebellum not only in motor, but also in cognitive and emotional functions. Spinocerebellar ataxia type 14 (SCA14) is an autosomal dominant hereditary ataxia characterized by a relatively pure cerebellar phenotype. Cognitive impairment has been reported in studies with phenotype descriptions of SCA14, but previous studies have been small without control groups, and no homogeneous and systematic test panel has been used. The objective of this study was to thoroughly characterize the neuropsychological profile in ten Norwegian SCA14 subjects compared to unaffected family members and population norm data. Methods: Ten SCA14 subjects and ten intrafamilial unaffected age- and education-matched controls from two Norwegian families were included. The unaffected intrafamilial controls included six first degree relatives, two second degree relatives, and two spouses. General intellectual ability, memory, visuoperceptive skills, psychomotor speed, executive functions, depression and anxiety were examined using internationally standardized tests, with minimal need for manual response to avoid motor bias. Results: No significant cognitive deficit was found in SCA14 subjects compared to intrafamilial controls. Verbal IQ, verbal executive function and psychomotor speed tended to be reduced in affected subjects, but previously reported non-verbal executive dysfunction was not confirmed in this study. Conclusion: Only subtle cognitive impairment was found in SCA14 affected subjects. The current findings do not confirm earlier reports of cognitive dysfunction in SCA14, but does shows a mild impairment in specific verbal executive functions. Genotypic differences may partly account for this discrepancy, and further studies on larger materials are needed to verify the findings.
dc.languageEN
dc.publisherBioMed Central
dc.relation.ispartofWedding, Iselin Marie (2016) Hereditary spinocerebellar degenerative disorders in Norway. Molecular and clinical studies of ataxia subtypes in a Norwegian patient population. Doctoral thesis. http://urn.nb.no/URN:NBN:no-53846
dc.relation.urihttp://urn.nb.no/URN:NBN:no-53846
dc.rightsAttribution 2.0 Generic
dc.rights.urihttp://creativecommons.org/licenses/by/2.0/
dc.titleCognition is only minimally impaired in Spinocerebellar ataxia type 14 (SCA 14): a neuropsychological study of ten Norwegian subjects compared to intrafamilial controls and population norm
dc.typeJournal article
dc.creator.authorWedding, Iselin Marie
dc.creator.authorKoht, Jeanette
dc.creator.authorDietrichs, Espen
dc.creator.authorLandrø, Nils Inge
dc.creator.authorTallaksen, Chantal
cristin.unitcode185,53,12,17
cristin.unitnameNevrologisk avdeling
cristin.ispublishedtrue
cristin.fulltextoriginal
cristin.qualitycode1
dc.identifier.cristin1074465
dc.identifier.bibliographiccitationinfo:ofi/fmt:kev:mtx:ctx&ctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=BMC Neurology&rft.volume=&rft.spage=&rft.date=2013
dc.identifier.jtitleBMC Neurology
dc.identifier.volume13
dc.identifier.pagecount13
dc.identifier.doihttp://dx.doi.org/10.1186/1471-2377-13-186
dc.identifier.urnURN:NBN:no-40243
dc.type.documentTidsskriftartikkel
dc.type.peerreviewedPeer reviewed
dc.source.issn1471-2377
dc.identifier.fulltextFulltext https://www.duo.uio.no/bitstream/handle/10852/37954/1/publisertartikkelSCA14kognisjon.pdf
dc.type.versionPublishedVersion
cristin.articleid186


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