Cognitive Performance of Patients with Adult 5q-Spinal Muscular Atrophy and with Amyotrophic Lateral Sclerosis

• Published in: Brain sciences Vol. 11; no. 1; p. 8
• Main Authors: 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
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 23.12.2020; MDPI
• Subjects: Adults; Age; Amyotrophic lateral sclerosis; amyotrophic lateral sclerosis (ALS); Atrophy; Cognition & reasoning; Cognitive ability; cognitive function; Dementia; Edinburgh Cognitive and Behavioural ALS Screen (ECAS); Education; Etiology; Executive function; German vocabulary test (Wortschatztest, WST); Intelligence tests; motor neuron disease; Motor neuron diseases; Motor neurone disease; Mutation; Patients; Spinal muscular atrophy; spinal muscular atrophy (SMA); cognitive function; German vocabulary test (Wortschatztest, WST); motor neuron disease; amyotrophic lateral sclerosis (ALS); Edinburgh Cognitive and Behavioural ALS Screen (ECAS); spinal muscular atrophy (SMA)
• ISSN: 2076-3425; 2076-3425
• 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.