TY - JOUR A1 - Schmidt, Hendrikje A1 - Felisatti, Arianna A1 - Aster, Michael von A1 - Wilbert, Jürgen A1 - Moers, Arpad von A1 - Fischer, Martin H. T1 - Neuromuscular diseases affect number representation and processing T2 - Frontiers in psychology / Frontiers Research Foundation N2 - Spinal muscular atrophy (SMA) and Duchenne muscular dystrophy (DMD) both are rare genetic neuromuscular diseases with progressive loss of motor ability. The neuromotor developmental course of those diseases is well documented. In contrast, there is only little evidence about characteristics of general and specific cognitive development. In both conditions the final motor outcome is characterized by an inability to move autonomously: children with SMA never accomplish independent motoric exploration of their environment, while children with DMD do but later lose this ability again. These profound differences in developmental pathways might affect cognitive development of SMA vs. DMD children, as cognition is shaped by individual motor experiences. DMD patients show impaired executive functions, working memory, and verbal IQ, whereas only motor ability seems to be impaired in SMA. Advanced cognitive capacity in SMA may serve as a compensatory mechanism for achieving in education, career progression, and social satisfaction. This study aimed to relate differences in basic numerical concepts and arithmetic achievement in SMA and DMD patients to differences in their motor development and resulting sensorimotor and environmental experiences. Horizontal and vertical spatial-numerical associations were explored in SMA/DMD children ranging between 6 and 12 years through the random number generation task. Furthermore, arithmetic skills as well as general cognitive ability were assessed. Groups differed in spatial number processing as well as in arithmetic and domain-general cognitive functions. Children with SMA showed no horizontal and even reversed vertical spatial-numerical associations. Children with DMD on the other hand revealed patterns in spatial numerical associations comparable to healthy developing children. From the embodied Cognition perspective, early sensorimotor experience does play a role in development of mental number representations. However, it remains open whether and how this becomes relevant for the acquisition of higher order cognitive and arithmetic skills. KW - spatial-numerical associations KW - numerical processing KW - mathematics KW - child development KW - embodied cognition KW - neuromuscular disease KW - spinal muscular atrophy KW - Duchenne muscular dystrophy Y1 - 2021 UR - https://publishup.uni-potsdam.de/frontdoor/index/index/docId/52230 SN - 1664-1078 VL - 12 PB - Frontiers Research Foundation CY - Lausanne ER -