Improvement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series

Although nusinersen has been demonstrated to improve motor function in patients with spinal muscular atrophy (SMA), no studies have investigated its effect on fine manual dexterity. The present study aimed to investigate the ability of nusinersen to improve fine manual dexterity in patients with SMA...

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Autores principales:	Minsu Gu, Hyun-Ho Kong
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	spinal muscular atrophy (SMA) nusinersen fine manual dexterity Pediatrics RJ1-570
Acceso en línea:	https://doaj.org/article/71bca707eb66421d943f28725339943a
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spelling	oai:doaj.org-article:71bca707eb66421d943f28725339943a2021-11-25T17:14:46ZImprovement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series10.3390/children81110392227-9067https://doaj.org/article/71bca707eb66421d943f28725339943a2021-11-01T00:00:00Zhttps://www.mdpi.com/2227-9067/8/11/1039https://doaj.org/toc/2227-9067Although nusinersen has been demonstrated to improve motor function in patients with spinal muscular atrophy (SMA), no studies have investigated its effect on fine manual dexterity. The present study aimed to investigate the ability of nusinersen to improve fine manual dexterity in patients with SMA type 2. A total of five patients with SMA type 2 were included. The Hammersmith Functional Motor Scale (expanded version) (HFMSE) and Purdue Pegboard (PP) tests were used to evaluate gross motor function and fine manual dexterity, respectively, until 18 months after nusinersen administration. HFMSE scores improved by 3–10 points (+13–53%) in all patients following nusinersen administration. PP scores also improved in all patients, from 4 to 9 points (+80–225%) in the preferred hand and from 3 to 7 points (+60–500%) in the non-preferred hand. These results suggest that nusinersen treatment improved both gross motor function and fine manual dexterity in children with SMA type 2. Addition of the PP test may aid in evaluating the fine manual dexterity essential for activities of daily living in these patients.Minsu GuHyun-Ho KongMDPI AGarticlespinal muscular atrophy (SMA)nusinersenfine manual dexterityPediatricsRJ1-570ENChildren, Vol 8, Iss 1039, p 1039 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	spinal muscular atrophy (SMA) nusinersen fine manual dexterity Pediatrics RJ1-570
spellingShingle	spinal muscular atrophy (SMA) nusinersen fine manual dexterity Pediatrics RJ1-570 Minsu Gu Hyun-Ho Kong Improvement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series
description	Although nusinersen has been demonstrated to improve motor function in patients with spinal muscular atrophy (SMA), no studies have investigated its effect on fine manual dexterity. The present study aimed to investigate the ability of nusinersen to improve fine manual dexterity in patients with SMA type 2. A total of five patients with SMA type 2 were included. The Hammersmith Functional Motor Scale (expanded version) (HFMSE) and Purdue Pegboard (PP) tests were used to evaluate gross motor function and fine manual dexterity, respectively, until 18 months after nusinersen administration. HFMSE scores improved by 3–10 points (+13–53%) in all patients following nusinersen administration. PP scores also improved in all patients, from 4 to 9 points (+80–225%) in the preferred hand and from 3 to 7 points (+60–500%) in the non-preferred hand. These results suggest that nusinersen treatment improved both gross motor function and fine manual dexterity in children with SMA type 2. Addition of the PP test may aid in evaluating the fine manual dexterity essential for activities of daily living in these patients.
format	article
author	Minsu Gu Hyun-Ho Kong
author_facet	Minsu Gu Hyun-Ho Kong
author_sort	Minsu Gu
title	Improvement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series
title_short	Improvement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series
title_full	Improvement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series
title_fullStr	Improvement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series
title_full_unstemmed	Improvement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series
title_sort	improvement in fine manual dexterity in children with spinal muscular atrophy type 2 after nusinersen injection: a case series
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/71bca707eb66421d943f28725339943a
work_keys_str_mv	AT minsugu improvementinfinemanualdexterityinchildrenwithspinalmuscularatrophytype2afternusinerseninjectionacaseseries AT hyunhokong improvementinfinemanualdexterityinchildrenwithspinalmuscularatrophytype2afternusinerseninjectionacaseseries
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Improvement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series

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