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CRD42020140343). Two independent reviewers searched the PubMed\/MEDLINE, LILACS, Embase, and Scopus databases. Inclusion criteria were cross\u2010sectional or longitudinal studies or randomized controlled trials that used scales or questionnaires to assess upper\u2010limb function in paediatric patients with NMDs. The COSMIN Risk of Bias checklist and criteria for good measurement properties were applied to assess the methodological quality of the instruments.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 34 articles and 12 instruments were included. The Brooke Upper Extremity (<jats:italic>n<\/jats:italic>=16) and Performance of Upper Limb (PUL) (<jats:italic>n<\/jats:italic>=12) instruments were the most used tools. The PUL and Duchenne muscular dystrophy (DMD) Upper Limb patient\u2010reported outcome measures (PROMs) tested more measurement properties and provided higher methodological quality scores for patients with DMD. Likewise, the Revised Upper Limb Module (RULM) was the most suitable instrument for patients with spinal muscular atrophy. No instrument has been devised to assess upper\u2010limb function in patients with Charcot\u2013Marie\u2013Tooth disease and no other disease\u2010specific instruments were found.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>The PUL, DMD Upper Limb PROM, and RULM are the most suitable instruments to assess upper\u2010limb function in the two most prevalent paediatric NMDs. The identified gaps and methodological flaws of the available instruments indicate a need to develop high\u2010quality instruments to assess other types of paediatric NMDs.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p>\n<jats:boxed-text content-type=\"box\" position=\"anchor\"><jats:caption><jats:title>What this paper adds<\/jats:title><\/jats:caption><jats:p>\n<jats:list list-type=\"bullet\">\n\n<jats:list-item><jats:p>The most suitable observer\u2013rater instrument to assess upper\u2010limb function in Duchenne muscular dystrophy (DMD) is the Performance of Upper Limb.<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>The most suitable observer\u2013rater instrument to assess upper\u2010limb function in spinal muscular atrophy is the Revised Upper Limb Module.<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>The DMD Upper Limb patient\u2010reported outcome measure is recommended to assess the upper\u2010limb performance of patients with DMD.<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>Literature gaps and methodological flaws indicate the need to develop high\u2010quality instruments to assess other types of paediatric neuromuscular disease.<\/jats:p><\/jats:list-item>\n<\/jats:list>\n<\/jats:p><\/jats:boxed-text>\n<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.14887","type":"journal-article","created":{"date-parts":[[2021,4,9]],"date-time":"2021-04-09T06:43:40Z","timestamp":1617950620000},"page":"1030-1037","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Instruments to assess upper\u2010limb function in children and adolescents with neuromuscular diseases: a systematic review"],"prefix":"10.1111","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4981-3868","authenticated-orcid":false,"given":"Gabriela Barroso De Queiroz","family":"Davoli","sequence":"first","affiliation":[{"name":"Department of Health Science Ribeir\u00e3o 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