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

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

doi:10.1002/mus.26419

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

Maria Carmela Pera
, Giorgia Coratti
, Elena S Mazzone
, Jacqueline Montes
, Mariacristina Scoto
, Roberto De Sanctis
, Marion Main
, Anna Mayhew
, Robert Muni Lofra
, Sally Dunaway Young
, Allan M Glanzman
, Tina Duong
, Amy Pasternak
, Danielle Ramsey
, Basil Darras
, John W Day
, Richard S Finkel
, Maria Pia Sormani
, Francesca Bovis
, Darryl C De Vivo

Volker Straub, Francesco Muntoni, Marika Pane, Eugenio Mercuri, iSMAC Consortium Group

Research output: Contribution to journal › Article › peer-review

104 Citations (Scopus)

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.

Original language	English
Pages (from-to)	426-430
Number of pages	5
Journal	Muscle & Nerve
Volume	59
Issue number	4
Early online date	7 Feb 2019
DOIs	https://doi.org/10.1002/mus.26419
Publication status	Published - 1 Apr 2019

Keywords

Functional assessment
Longitudinal Data
Neuromuscular disorders
Outcome measures
Spinal Muscular Atrophy
Upper limb

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Pera, M. C., Coratti, G., Mazzone, E. S., Montes, J., Scoto, M., De Sanctis, R., Main, M., Mayhew, A., Muni Lofra, R., Dunaway Young, S., Glanzman, A. M., Duong, T., Pasternak, A., Ramsey, D., Darras, B., Day, J. W., Finkel, R. S., Sormani, M. P., Bovis, F., ... iSMAC Consortium Group (2019). Revised upper limb module for spinal muscular atrophy: 12 month changes. Muscle & 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. ",

keywords = "Functional assessment, Longitudinal Data, Neuromuscular disorders, Outcome measures, Spinal Muscular Atrophy, Upper limb",

author = "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 Sormani, \{Maria Pia\} and Francesca Bovis and \{De Vivo\}, \{Darryl C\} and Volker Straub and Francesco Muntoni and Marika Pane and Eugenio Mercuri and \{iSMAC Consortium Group\}",

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doi = "10.1002/mus.26419",

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Pera, MC, Coratti, G, Mazzone, ES, Montes, J, Scoto, M, De Sanctis, R, Main, M, Mayhew, A, Muni Lofra, R, Dunaway Young, S, Glanzman, AM, Duong, T, Pasternak, A, Ramsey, D, Darras, B, Day, JW, Finkel, RS, Sormani, MP, Bovis, F, De Vivo, DC, Straub, V, Muntoni, F, Pane, M, Mercuri, E & iSMAC Consortium Group 2019, 'Revised upper limb module for spinal muscular atrophy: 12 month changes', Muscle & Nerve, vol. 59, no. 4, pp. 426-430. https://doi.org/10.1002/mus.26419

TY - JOUR

T1 - Revised upper limb module for spinal muscular atrophy: 12 month changes

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 - Sormani, Maria Pia

AU - Bovis, Francesca

AU - De Vivo, Darryl C

AU - Straub, Volker

AU - Muntoni, Francesco

AU - Pane, Marika

AU - Mercuri, Eugenio

AU - iSMAC Consortium Group

PY - 2019/4/1

Y1 - 2019/4/1

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.

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.

KW - Functional assessment

KW - Longitudinal Data

KW - Neuromuscular disorders

KW - Outcome measures

KW - Spinal Muscular Atrophy

KW - Upper limb

UR - https://www.scopus.com/pages/publications/85061291239

U2 - 10.1002/mus.26419

DO - 10.1002/mus.26419

M3 - Article

C2 - 30677148

SN - 1097-4598

VL - 59

SP - 426

EP - 430

JO - Muscle & Nerve

JF - Muscle & Nerve

IS - 4

ER -

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

Abstract

Keywords

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