Revised upper limb module for spinal muscular atrophy: 12 month changes

iSMAC Consortium Group

doi:10.1002/mus.26419

Revised upper limb module for spinal muscular atrophy: 12 month changes

iSMAC Consortium Group

Research output: Contribution to journal › Article

Abstract

INTRODUCTION: The aim of the study was to assess 12 month changes in upper limb function in patients affected by spinal muscular atrophy type 2 and 3.

METHODS: Longitudinal 12 month data was collected in 114 patients, 60 type 2 and 54 type 3, using the Revised Upper Limb Module.

RESULTS: The 12 month changes ranged between -7 and 9 (mean: -0.41; SD: 2.93). The mean changes were not significantly different between the three spinal muscular atrophy groups (-0.45 in type 2, -0.23 in non-ambulant type 3 and -0.34 in ambulant type 3, p = 0.96) and the relationship between 12 month change and age classes was not significantly different among the three types of SMA patients.

DISCUSSION: Our results confirm that the Module explores a wide range of functional abilities and can be used in ambulant and non-ambulant patients of different ages in conjunction with other functional scales. Muscle Nerve 59:426-430, 2019.

Original language	English
Pages (from-to)	426-430
Number of pages	5
Journal	Muscle and Nerve
Volume	59
Issue number	4
DOIs	https://doi.org/10.1002/mus.26419
Publication status	Published - Apr 2019

Keywords

Adolescent
Adult
Algorithms
Child
Child, Preschool
Disease Progression
Female
Health Status
Humans
Longitudinal Studies
Male
Middle Aged
Muscular Atrophy, Spinal/pathology
Reference Standards
Spinal Muscular Atrophies of Childhood/pathology
Upper Extremity/pathology
Walking
Young Adult

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10.1002/mus.26419

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iSMAC Consortium Group (2019). Revised upper limb module for spinal muscular atrophy: 12 month changes. Muscle and Nerve, 59(4), 426-430. https://doi.org/10.1002/mus.26419

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title = "Revised upper limb module for spinal muscular atrophy: 12 month changes",

abstract = "INTRODUCTION: The aim of the study was to assess 12 month changes in upper limb function in patients affected by spinal muscular atrophy type 2 and 3.METHODS: Longitudinal 12 month data was collected in 114 patients, 60 type 2 and 54 type 3, using the Revised Upper Limb Module.RESULTS: The 12 month changes ranged between -7 and 9 (mean: -0.41; SD: 2.93). The mean changes were not significantly different between the three spinal muscular atrophy groups (-0.45 in type 2, -0.23 in non-ambulant type 3 and -0.34 in ambulant type 3, p = 0.96) and the relationship between 12 month change and age classes was not significantly different among the three types of SMA patients.DISCUSSION: Our results confirm that the Module explores a wide range of functional abilities and can be used in ambulant and non-ambulant patients of different ages in conjunction with other functional scales. Muscle Nerve 59:426-430, 2019.",

keywords = "Adolescent, Adult, Algorithms, Child, Child, Preschool, Disease Progression, Female, Health Status, Humans, Longitudinal Studies, Male, Middle Aged, Muscular Atrophy, Spinal/pathology, Reference Standards, Spinal Muscular Atrophies of Childhood/pathology, Upper Extremity/pathology, Walking, Young Adult",

author = "{iSMAC Consortium Group} and Pera, {Maria Carmela} and Giorgia Coratti and Mazzone, {Elena S} and Jacqueline Montes and Mariacristina Scoto and {De Sanctis}, Roberto and Marion Main and Anna Mayhew and {Muni Lofra}, Robert and {Dunaway Young}, Sally and Glanzman, {Allan M} and Tina Duong and Amy Pasternak and Danielle Ramsey and Basil Darras and Day, {John W} and Finkel, {Richard S} and {De Vivo}, {Darryl C} and Sormani, {Maria Pia} and Francesca Bovis and Volker Straub and Francesco Muntoni and Marika Pane and Eugenio Mercuri",

note = "{\textcopyright} 2019 Wiley Periodicals, Inc.",

year = "2019",

month = apr,

doi = "10.1002/mus.26419",

language = "English",

volume = "59",

pages = "426--430",

journal = "Muscle and Nerve",

issn = "0148-639X",

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TY - JOUR

T1 - Revised upper limb module for spinal muscular atrophy

T2 - 12 month changes

AU - iSMAC Consortium Group

AU - Pera, Maria Carmela

AU - Coratti, Giorgia

AU - Mazzone, Elena S

AU - Montes, Jacqueline

AU - Scoto, Mariacristina

AU - De Sanctis, Roberto

AU - Main, Marion

AU - Mayhew, Anna

AU - Muni Lofra, Robert

AU - Dunaway Young, Sally

AU - Glanzman, Allan M

AU - Duong, Tina

AU - Pasternak, Amy

AU - Ramsey, Danielle

AU - Darras, Basil

AU - Day, John W

AU - Finkel, Richard S

AU - De Vivo, Darryl C

AU - Sormani, Maria Pia

AU - Bovis, Francesca

AU - Straub, Volker

AU - Muntoni, Francesco

AU - Pane, Marika

AU - Mercuri, Eugenio

PY - 2019/4

Y1 - 2019/4

N2 - INTRODUCTION: The aim of the study was to assess 12 month changes in upper limb function in patients affected by spinal muscular atrophy type 2 and 3.METHODS: Longitudinal 12 month data was collected in 114 patients, 60 type 2 and 54 type 3, using the Revised Upper Limb Module.RESULTS: The 12 month changes ranged between -7 and 9 (mean: -0.41; SD: 2.93). The mean changes were not significantly different between the three spinal muscular atrophy groups (-0.45 in type 2, -0.23 in non-ambulant type 3 and -0.34 in ambulant type 3, p = 0.96) and the relationship between 12 month change and age classes was not significantly different among the three types of SMA patients.DISCUSSION: Our results confirm that the Module explores a wide range of functional abilities and can be used in ambulant and non-ambulant patients of different ages in conjunction with other functional scales. Muscle Nerve 59:426-430, 2019.

AB - INTRODUCTION: The aim of the study was to assess 12 month changes in upper limb function in patients affected by spinal muscular atrophy type 2 and 3.METHODS: Longitudinal 12 month data was collected in 114 patients, 60 type 2 and 54 type 3, using the Revised Upper Limb Module.RESULTS: The 12 month changes ranged between -7 and 9 (mean: -0.41; SD: 2.93). The mean changes were not significantly different between the three spinal muscular atrophy groups (-0.45 in type 2, -0.23 in non-ambulant type 3 and -0.34 in ambulant type 3, p = 0.96) and the relationship between 12 month change and age classes was not significantly different among the three types of SMA patients.DISCUSSION: Our results confirm that the Module explores a wide range of functional abilities and can be used in ambulant and non-ambulant patients of different ages in conjunction with other functional scales. Muscle Nerve 59:426-430, 2019.

KW - Adolescent

KW - Adult

KW - Algorithms

KW - Child

KW - Child, Preschool

KW - Disease Progression

KW - Female

KW - Health Status

KW - Humans

KW - Longitudinal Studies

KW - Male

KW - Middle Aged

KW - Muscular Atrophy, Spinal/pathology

KW - Reference Standards

KW - Spinal Muscular Atrophies of Childhood/pathology

KW - Upper Extremity/pathology

KW - Walking

KW - Young Adult

U2 - 10.1002/mus.26419

DO - 10.1002/mus.26419

M3 - Article

C2 - 30677148

VL - 59

SP - 426

EP - 430

JO - Muscle and Nerve

JF - Muscle and Nerve

SN - 0148-639X

IS - 4

ER -