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Neuromuscul Disord. 2014 Apr;24(4):347-52. doi: 10.1016/j.nmd.2014.01.003. Epub 2014 Jan 16.

Hammersmith Functional Motor Scale and Motor Function Measure-20 in non ambulant SMA patients.

Author information

1
Department of Paediatric Neurology, Catholic University, Rome, Italy.
2
Dubowitz Neuromuscular Centre, Institute of Child Health, University College, London, UK.
3
Child Neurology, University Hospitals Leuven, Leuven, Belgium.
4
Department of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
5
Service of Physical Medicine and Rehabilitation, University Hospital Sant Joan de Deu, Barcelona, Spain.
6
University Medical Centre, Freiburg, Germany.
7
Child and Adolescence Neuropsychiatry Unit, Department of Neuroscience, and Department of Laboratory Medicine, Unit of Molecular Medicine, Bambino Gesù Hospital, Rome, Italy.
8
Institute of Genetic Medicine, Newcastle Upon Tyne, UK.
9
Institute of Myology, Groupe hospitalier La Pitié Salpétrière, Paris, France.
10
Department of Paediatric Neurology, Catholic University, Rome, Italy. Electronic address: mercuri@rm.unicatt.it.

Abstract

The aim of this prospective longitudinal multi centric study was to evaluate the correlation between the Hammersmith Functional Motor Scale and the 20 item version of the Motor Function Measure in non ambulant SMA children and adults at baseline and over a 12 month period. Seventy-four non-ambulant patients performed both measures at baseline and 49 also had an assessment 12 month later. At baseline the scores ranged between 0 and 40 on the Hammersmith Motor function Scale and between 3 and 45 on the Motor Function Measure 20. The correlation between the two scales was 0.733. The 12 month changes ranged between -11 and 4 for the Hammersmith and between -11 and 7 for the Motor Function Measure 20. The correlation between changes was 0.48. Our results suggest that both scales provide useful information although they appeared to work differently at the two extremes of the spectrum of abilities. The Hammersmith Motor Function Scale appeared to be more suitable in strong non ambulant patients, while the Motor Function Measures appeared to be more sensitive to capture activities and possible changes in the very weak patients, including more items capturing axial and upper limb activities. The choice of these measures in clinical trials should therefore depend on inclusion criteria and magnitude of expected changes.

KEYWORDS:

Outcome measures; Spinal muscular atrophy

PMID:
24491485
DOI:
10.1016/j.nmd.2014.01.003
[Indexed for MEDLINE]
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