The functional assessment and tests for children patients with spinal muscular atrophy


Authors: I. Švábová 1,2;  J. Válková 1,2;  J. Haberlová 2
Authors‘ workplace: Klinika rehabilitace a tělovýchovného lékařství FN Motol, Praha 1;  Neuromuskulární centrum, Klinika dětské neurologie FN Motol, Praha 2
Published in: Cesk Slov Neurol N 2020; 83/116(Supplementum 2): 24-26
doi: https://doi.org/10.48095/cccsnn2S24

Overview

Introduction to functional tests and scales used by Neuromuscular center in the University Hospital in Motol. These examinations of motor skills for patients with spinal muscular atrophy were developed and are used to evaluate the state of complex motor functions to evaluate the natural course of spinal muscular atrophy. They are especially important for the treatment with Spinraza – determination of default state with the aim to observe the efficiency on motor functions. For now, motor scales mentioned in the text are only a marker of the available treatment, therefore extremely needed.

Keywords:

motor scales – functional assessment – spinal muscular atrophy – Chop Intend: Children´s Hospital of Philadelphia Infant test of Neuromuscular Disorders – Hammersmith Functional Motor Scale Expanded – 6 Minute Walk Test – RULM


Sources

1. CHOP INTEND – Manual of procedures and score sheets. Biogen 2018.

2. Glanzman AM, Mazzone E, Main M et al. The Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disordes (CHOP INTEND): test development and reliability. Neuromuscular Disordes 2010; 20(3): 155–161. doi: 10.1016/ j.nmd.2009.11.014.

3. HFMSE – Manual of procedures and score sheets. Biogen 2018.

4. Ramsey D, Scoto M, Mayhew A et al.Revised Hammersmith Scale for spinal muscular atrophy: a SMA specific clinical outcome assessment tool. PLoS One 2017; 12(2): e0172346. doi: 10.1371/ journal.pone.0172346.

5. RULM – Manual of procedures and score sheets. Biogen 2018.

6. Mazzone ES, Mayhew A, Montes J et al. Revised upper limb modul for spinal muscular atrophy: Development of a new module. Muscle Nerve 2017; 55(6): 869–874. doi: 10.1002/ mus.25430.

7. Young SD, Montes J, Kramer S et al. Six-minute walk test is reliable and valid in spinal muscular atrophy. Muscle Nerve 2016; 54(6): 836–842. doi: 10.1002/ mus.25120.

8. Angerová Y, Švestková O, Sussová J et al. Neurorehabili­tace. Cesk Slov Neurol N 2010; 73/ 106(2): 131–135.

9. Kolář P et al. Rehabilitace v klinické praxi. Praha: Galén 2009.

Labels
Paediatric neurology Neurosurgery Neurology
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