Reliability of the Hammersmith functional motor scale for spinal muscular atrophy in a multicentric study.
The aim of this study was to validate the Hammersmith functional motor scale for children with spinal muscular atrophy in a large cohort of 90 non-ambulant children with spinal muscular atrophy type 2 or 3. All had a baseline assessment (T0) and were reassessed either at 3 months (T1) (n = 66) or at 6 months (T2) (n = 24). Inter-observer reliability, tested on 13 children among 3 examiners, was > 95%. Of the 66 children examined after 3 months 4 had adverse effects in between assessments and were excluded from the analysis. Forty-two (68%) of the remaining 62 reassessed had no variation in scores between T0 and T1 and 13 (21%) were within +/- 1 point. 9 (37.5%) of the 24 children reassessed after 6 months had no variation in scores between T0 and T2 and another 9 (37.5%) had variations within +/- 1 point. Our study confirms previous observations of the reliability of the scale and helps to establish a baseline for assessing changes of functional ability over 3 and 6 month intervals. This information can be valuable in view of therapeutic trials.
Mercuri
E
E
Department of Paediatric Neurology, Catholic University, Largo Gemelli, 00168 Rome, Italy. e.mercuri@imperial.ac.uk
Messina
S
S
Battini
R
R
Berardinelli
A
A
Boffi
P
P
Bono
R
R
Bruno
C
C
Carboni
N
N
Cini
C
C
Colitto
F
F
D'Amico
A
A
Minetti
C
C
Mirabella
M
M
Mongini
T
T
Morandi
L
L
Dlamini
N
N
Orcesi
S
S
Pelliccioni
M
M
Pane
M
M
Pini
A
A
Swan
A V
AV
Villanova
M
M
Vita
G
G
Main
M
M
Muntoni
F
F
Bertini
E
E
D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003627:Data Interpretation, Statistical; D005260:Female; D006801:Humans; D007558:Italy; D008297:Male; D009046:Motor Neurons / Q000502:physiology*; D015588:Observer Variation; D011446:Prospective Studies; D011597:Psychomotor Performance; D012044:Regression Analysis; D015203:Reproducibility of Results; D014897:Spinal Muscular Atrophies of Childhood / Q000503:physiopathology*; D013997:Time Factors; D006113:United Kingdom