Hammersmith Functional Motor Scale and Motor Function Measure-20 in non ambulant SMA patients.
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.
Department of Paediatric Neurology, Catholic University, Rome, Italy.
E
E
Mazzone
Department of Paediatric Neurology, Catholic University, Rome, Italy.
R
R
De Sanctis
Department of Paediatric Neurology, Catholic University, Rome, Italy.
L
L
Fanelli
Department of Paediatric Neurology, Catholic University, Rome, Italy.
F
F
Bianco
Dubowitz Neuromuscular Centre, Institute of Child Health, University College, London, UK.
M
M
Main
Child Neurology, University Hospitals Leuven, Leuven, Belgium.
M
M
van den Hauwe
Dubowitz Neuromuscular Centre, Institute of Child Health, University College, London, UK.
M
M
Ash
Department of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
R
R
de Vries
Service of Physical Medicine and Rehabilitation, University Hospital Sant Joan de Deu, Barcelona, Spain.
J
J
Fagoaga Mata
University Medical Centre, Freiburg, Germany.
K
K
Schaefer
Child and Adolescence Neuropsychiatry Unit, Department of Neuroscience, and Department of Laboratory Medicine, Unit of Molecular Medicine, Bambino Gesù Hospital, Rome, Italy.
A
A
D'Amico
Child and Adolescence Neuropsychiatry Unit, Department of Neuroscience, and Department of Laboratory Medicine, Unit of Molecular Medicine, Bambino Gesù Hospital, Rome, Italy.
G
G
Colia
Department of Paediatric Neurology, Catholic University, Rome, Italy.
C
C
Palermo
Dubowitz Neuromuscular Centre, Institute of Child Health, University College, London, UK.
M
M
Scoto
Institute of Genetic Medicine, Newcastle Upon Tyne, UK.
A
A
Mayhew
Institute of Genetic Medicine, Newcastle Upon Tyne, UK.
M
M
Eagle
Institute of Myology, Groupe hospitalier La Pitié Salpétrière, Paris, France.
L
L
Servais
Service of Physical Medicine and Rehabilitation, University Hospital Sant Joan de Deu, Barcelona, Spain.
M
M
Vigo
Service of Physical Medicine and Rehabilitation, University Hospital Sant Joan de Deu, Barcelona, Spain.
A
A
Febrer
University Medical Centre, Freiburg, Germany.
R
R
Korinthenberg
Department of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
M
M
Jeukens
Department of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
M
M
de Viesser
Institute of Myology, Groupe hospitalier La Pitié Salpétrière, Paris, France.
A
A
Totoescu
Institute of Myology, Groupe hospitalier La Pitié Salpétrière, Paris, France.
T
T
Voit
Institute of Genetic Medicine, Newcastle Upon Tyne, UK.
K
K
Bushby
Dubowitz Neuromuscular Centre, Institute of Child Health, University College, London, UK.
F
F
Muntoni
Child Neurology, University Hospitals Leuven, Leuven, Belgium.
N
N
Goemans
Child and Adolescence Neuropsychiatry Unit, Department of Neuroscience, and Department of Laboratory Medicine, Unit of Molecular Medicine, Bambino Gesù Hospital, Rome, Italy.
E
E
Bertini
Department of Paediatric Neurology, Catholic University, Rome, Italy.
M
M
Pane
Department of Paediatric Neurology, Catholic University, Rome, Italy. Electronic address: mercuri@rm.unicatt.it.
E
E
Mercuri
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004185:Disability Evaluation; D005060:Europe; D005500:Follow-Up Studies; D006801:Humans; D008137:Longitudinal Studies; D009043:Motor Activity; D009134:Muscular Atrophy, Spinal / Q000175:diagnosis* / Q000503:physiopathology; D011446:Prospective Studies; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D055815:Young Adult
Outcome measures; Spinal muscular atrophy