Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081 Ulm, Germany.
J Neurol. 2009 Nov;256(11):1869-75. doi: 10.1007/s00415-009-5212-5. Epub 2009 Jul 2.
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.
一项针对 20 名经基因证实患有脊髓和延髓肌肉萎缩症 (SBMA) 的男性患者的认知功能的横断面神经心理学研究，将其认知表现与 20 名年龄和教育程度匹配的对照组进行了比较。神经心理学评估涵盖了执行功能、记忆和注意力控制。SBMA 患者在言语和非言语流畅性以及概念形成方面存在缺陷。此外，他们在所有被调查的工作记忆、短期和长期记忆领域都表现出明显的记忆缺陷。在注意力控制方面，SBMA 患者在相关子测试中的表现较差，尽管整体性能差异没有达到显著水平。我们的结论是，尽管处于亚临床水平，但 SBMA 患者的额颞叶认知功能受损。因此，SBMA 的功能缺陷不仅局限于运动神经元，还影响运动外网络。
