Assessing upper limb function in nonambulant SMA patients: Development of a new module

Elena Mazzone; Flaviana Bianco; Diego Martinelli; Allan M. Glanzman; Sonia Messina; Roberto De Sanctis; Marion Main; Michelle Eagle; Julaine Florence; Kristin Krosschell; Gessica Vasco; Marco Pelliccioni; Marilena Lombardo; Marika Pane; Richard Finkel; Francesco Muntoni; Enrico Bertini; Eugenio Mercuri

doi:10.1016/j.nmd.2011.02.014

Assessing upper limb function in nonambulant SMA patients: Development of a new module

Elena Mazzone, Flaviana Bianco, Diego Martinelli, Allan M. Glanzman, Sonia Messina, Roberto De Sanctis, Marion Main, Michelle Eagle, Julaine Florence, Kristin Krosschell, Gessica Vasco, Marco Pelliccioni, Marilena Lombardo, Marika Pane, Richard Finkel, Francesco Muntoni, Enrico Bertini, Eugenio Mercuri^*

^*Corresponding author for this work

Physical Therapy and Human Movement Sciences

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

79 Scopus citations

Abstract

We report the development of a module specifically designed for assessing upper limb function in nonambulant SMA patients, including young children and those with severe contractures. The application of the module to a preschool cohort of 40 children (age 30-48 months) showed that all the items could be completed by 30 months.The module was also used in 45 nonambulant SMA patients (age 30 months to 27 years). Their scores were more variable than in the preschool cohort, ranging from 0 to 18. The magnitude of scores was not related to age (r= -0.19).The upper limb scores had a good correlation with the Hammersmith Functional Motor Scale, r= 0.75, but the upper limb function did not always strictly follow the overall gross motor function. These findings suggest that even some of the very weak nonambulant children possess upper limb skills that can be measured.

Original language	English (US)
Pages (from-to)	406-412
Number of pages	7
Journal	Neuromuscular Disorders
Volume	21
Issue number	6
DOIs	https://doi.org/10.1016/j.nmd.2011.02.014
State	Published - Jun 2011

Keywords

Limb
Outcome measures
Sma
Upper

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Neurology
Clinical Neurology
Genetics(clinical)

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10.1016/j.nmd.2011.02.014

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Mazzone, E., Bianco, F., Martinelli, D., Glanzman, A. M., Messina, S., Sanctis, R. D., Main, M., Eagle, M., Florence, J., Krosschell, K., Vasco, G., Pelliccioni, M., Lombardo, M., Pane, M., Finkel, R., Muntoni, F., Bertini, E., & Mercuri, E. (2011). Assessing upper limb function in nonambulant SMA patients: Development of a new module. Neuromuscular Disorders, 21(6), 406-412. https://doi.org/10.1016/j.nmd.2011.02.014

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title = "Assessing upper limb function in nonambulant SMA patients: Development of a new module",

abstract = "We report the development of a module specifically designed for assessing upper limb function in nonambulant SMA patients, including young children and those with severe contractures. The application of the module to a preschool cohort of 40 children (age 30-48 months) showed that all the items could be completed by 30 months.The module was also used in 45 nonambulant SMA patients (age 30 months to 27 years). Their scores were more variable than in the preschool cohort, ranging from 0 to 18. The magnitude of scores was not related to age (r= -0.19).The upper limb scores had a good correlation with the Hammersmith Functional Motor Scale, r= 0.75, but the upper limb function did not always strictly follow the overall gross motor function. These findings suggest that even some of the very weak nonambulant children possess upper limb skills that can be measured.",

keywords = "Limb, Outcome measures, Sma, Upper",

author = "Elena Mazzone and Flaviana Bianco and Diego Martinelli and Glanzman, {Allan M.} and Sonia Messina and Sanctis, {Roberto De} and Marion Main and Michelle Eagle and Julaine Florence and Kristin Krosschell and Gessica Vasco and Marco Pelliccioni and Marilena Lombardo and Marika Pane and Richard Finkel and Francesco Muntoni and Enrico Bertini and Eugenio Mercuri",

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Mazzone, E, Bianco, F, Martinelli, D, Glanzman, AM, Messina, S, Sanctis, RD, Main, M, Eagle, M, Florence, J, Krosschell, K, Vasco, G, Pelliccioni, M, Lombardo, M, Pane, M, Finkel, R, Muntoni, F, Bertini, E & Mercuri, E 2011, 'Assessing upper limb function in nonambulant SMA patients: Development of a new module', Neuromuscular Disorders, vol. 21, no. 6, pp. 406-412. https://doi.org/10.1016/j.nmd.2011.02.014

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AU - Messina, Sonia

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AU - Main, Marion

AU - Eagle, Michelle

AU - Florence, Julaine

AU - Krosschell, Kristin

AU - Vasco, Gessica

AU - Pelliccioni, Marco

AU - Lombardo, Marilena

AU - Pane, Marika

AU - Finkel, Richard

AU - Muntoni, Francesco

AU - Bertini, Enrico

AU - Mercuri, Eugenio

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Assessing upper limb function in nonambulant SMA patients: Development of a new module

Abstract

Keywords

ASJC Scopus subject areas

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