期刊
JOURNAL OF NEUROLOGY
卷 256, 期 11, 页码 1869-1875出版社
SPRINGER HEIDELBERG
DOI: 10.1007/s00415-009-5212-5
关键词
Kennedy syndrome; X-linked spinal and bulbar muscular atrophy (SBMA); Motor neurone disease; Neuropsychology; Cognition; Executive function; Memory; Attention; Frontal lobe function; Temporal lobe function
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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