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

Alma Osmanovic; Gary Wieselmann; Lucas Mix; Hannah Alexandra Siegler; Mareike Kumpe; Gresa Ranxha; Claudia D Wurster; Alexander Steinke; Albert C Ludolph; Bruno Kopp; Dorothée Lulé; Susanne Petri; Olivia Schreiber-Katz

doi:10.3390/brainsci11010008

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

Brain Sci. 2020 Dec 23;11(1):8. doi: 10.3390/brainsci11010008.

Affiliations

¹ Department of Neurology, Hannover Medical School, 30625 Hannover, Germany.
² Department of Neurology, Neuropsychology, University of Ulm, 89081 Ulm, Germany.
³ Department of Neurology, University of Ulm, 89081 Ulm, Germany.
⁴ Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE), 89081 Ulm, Germany.

Abstract

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.

Keywords: Edinburgh Cognitive and Behavioural ALS Screen (ECAS); German vocabulary test (Wortschatztest, WST); amyotrophic lateral sclerosis (ALS); cognitive function; motor neuron disease; spinal muscular atrophy (SMA).