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

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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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Authors and Affiliations

  1. Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany

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

  2. Kreiskrankenhaus Biberach, Ziegelhausstraße 50, 88400, Biberach, Germany

    Georg Rüdiger Soukup

  3. Department of Psychiatry, University of Ulm, Leimgrubenweg 12, 89075, Ulm, Germany

    Jochen Karitzky

  4. Neuropoint Patient Academy, Pfauengasse 8, 89075, Ulm, Germany

    Herbert Schreiber

Authors
  1. Georg Rüdiger Soukup
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  2. Anne-Dorte Sperfeld
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  3. Ingo Uttner
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  4. Jochen Karitzky
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  5. Albert Christian Ludolph
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  6. Jan Kassubek
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  7. Herbert Schreiber
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Correspondence to Herbert Schreiber.

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J. Kassubek and H. Schreiber contributed equally (shared senior authorship).

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Soukup, G.R., Sperfeld, AD., Uttner, I. et al. Frontotemporal cognitive function in X-linked spinal and bulbar muscular atrophy (SBMA): a controlled neuropsychological study of 20 patients. J Neurol 256, 1869–1875 (2009). https://doi.org/10.1007/s00415-009-5212-5

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  • DOI: https://doi.org/10.1007/s00415-009-5212-5

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