Motor function measures in adult paients with spinal muscular atrophy


Authors: D. Botiková D 1,2;  T. Horák 2;  S. Voháňka 2
Authors‘ workplace: Rehabilitační oddělení, FN Brno 1;  ERN-EURO NMD Centrum, Neurologická klinika LF MU a FN Brno 2
Published in: Cesk Slov Neurol N 2020; 83/116(Supplementum 2): 27-30
doi: https://doi.org/10.48095/cccsnn20202S27

Overview

Along with the advance in wholesome/ overall care and its availability to patients with spinal muscular atrophy, longevity in this population has increased. This, however, brings up new questions and challenges to professionals in the neuromuscular field. Nowadays, with the development of therapeutic strategies modifying the course of the disease, and with availability of other symptomatic interventions, the need of outcome measures, which can follow the natural trajectory of the disease, and the effect of the therapy is emphasised. To observe the whole spectrum of motor functions, a very sensitive, reliable and clinically meaningful tool is necessary. However, it might be challenging to find suitable assessment methods which can capture complaints of very weak patients and, at the same time, meaningful aspects of much stronger individuals. That’s why the overall assessment should consist of tools measuring the muscle strength, range of motion in joints, relevant motor function scales and timed assessments reflecting patients’ activities of daily life. 

Keywords:

spinal muscular atrophy – functional assesment – motor scales


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Paediatric neurology Neurosurgery Neurology
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