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01.11.2009 | Original Paper

Frontotemporal cognitive function in X-linked spinal and bulbar muscular atrophy (SBMA): a controlled neuropsychological study of 20 patients

verfasst von: Georg Rüdiger Soukup, Anne-Dorte Sperfeld, Ingo Uttner, Jochen Karitzky, Albert Christian Ludolph, Jan Kassubek, Herbert Schreiber

Erschienen in: Journal of Neurology | Ausgabe 11/2009

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Abstract

A cross-sectional neuropsychological study of cognitive functions in 20 male patients with genetically proven spinal and bulbar muscular atrophy (SBMA) was performed, with a comparison of their cognitive performance with that of 20 age- and education-matched control subjects. Neuropsychological assessment covered executive functioning, memory, and attentional control. The SBMA patients revealed deficits in verbal and non-verbal fluency as well as concept formation. Additionally, they showed significant memory deficits in all of the investigated domains of working memory, short-term and long-term memory. With respect to attentional control, the SBMA patients underperformed in relevant subtests, although performance differences did not reach significance overall. We conclude that fronto-temporal cognitive functions are impaired in SMBA, although at a subclinical level. Thus, functional deficits in SBMA are not confined to motor neurons but also affect extramotor networks.

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Titel: Frontotemporal cognitive function in X-linked spinal and bulbar muscular atrophy (SBMA): a controlled neuropsychological study of 20 patients
verfasst von: Georg Rüdiger Soukup
Anne-Dorte Sperfeld
Ingo Uttner
Jochen Karitzky
Albert Christian Ludolph
Jan Kassubek
Herbert Schreiber
Publikationsdatum: 01.11.2009
Verlag: Springer-Verlag
Erschienen in: Journal of Neurology / Ausgabe 11/2009
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-009-5212-5

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