Osmanovic Alma, Wieselmann Gary, Mix Lucas, Siegler Hannah Alexandra, Kumpe Mareike, Ranxha Gresa, Wurster Claudia D, Steinke Alexander, Ludolph Albert C, Kopp Bruno, Lulé Dorothée, Petri Susanne, Schreiber-Katz Olivia
Department of Neurology, Hannover Medical School, 30625 Hannover, Germany.
Department of Neurology, Neuropsychology, University of Ulm, 89081 Ulm, Germany.
Brain Sci. 2020 Dec 23;11(1):8. doi: 10.3390/brainsci11010008.
Motor neuron diseases, such as spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS), share several clinical similarities while differing substantially in etiology, disease onset and progression. Cognitive dysfunction, a clinically relevant non-motor feature in a substantial proportion of ALS patients, has been less frequently investigated in SMA. In this prospective multicenter cross-sectional study, cognitive function was assessed by the Edinburgh Cognitive (and Behavioural) ALS Screen (ECAS) and a German vocabulary test (Wortschatztest, WST) in 34 adult patients with SMA types 2-4 and in 34 patients with ALS. Demographic and clinical parameters were assessed to identify factors that potentially influence cognitive function. While SMA and ALS patients were comparable in the vocabulary test, on average, SMA patients performed better than ALS patients in the cognitive domains of memory, language and executive function. Better cognitive abilities in SMA patients seemed to be related to the early onset, rather than the extent or the duration, of their physical handicap. Future studies should focus on disease-specific cognitive functions in SMA.
运动神经元疾病，如脊髓性肌萎缩症（SMA）和肌萎缩侧索硬化症（ALS），有一些临床相似之处，但在病因、疾病发作和进展方面有很大差异。认知功能障碍是相当一部分ALS患者临床上相关的非运动特征，在SMA中较少受到研究。在这项前瞻性多中心横断面研究中，通过爱丁堡认知（和行为）ALS筛查（ECAS）和德语词汇测试（Wortschatztest，WST）对34例2-4型成年SMA患者和34例ALS患者的认知功能进行了评估。评估了人口统计学和临床参数，以确定可能影响认知功能的因素。虽然SMA和ALS患者在词汇测试中具有可比性，但平均而言，SMA患者在记忆、语言和执行功能的认知领域表现优于ALS患者。SMA患者较好的认知能力似乎与其身体残疾的早发有关，而非其程度或持续时间。未来的研究应关注SMA中特定疾病的认知功能。
