title: Reliability of the Hammersmith functional motor scale for spinal muscular atrophy in a multicentric study.
abstract: 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.
authors.affiliation: Department of Paediatric Neurology, Catholic University, Largo Gemelli, 00168 Rome, Italy. e.mercuri@imperial.ac.uk
authors.forename: E
authors.initials: E
authors.lastname: Mercuri
authors.forename: S
authors.initials: S
authors.lastname: Messina
authors.forename: R
authors.initials: R
authors.lastname: Battini
authors.forename: A
authors.initials: A
authors.lastname: Berardinelli
authors.forename: P
authors.initials: P
authors.lastname: Boffi
authors.forename: R
authors.initials: R
authors.lastname: Bono
authors.forename: C
authors.initials: C
authors.lastname: Bruno
authors.forename: N
authors.initials: N
authors.lastname: Carboni
authors.forename: C
authors.initials: C
authors.lastname: Cini
authors.forename: F
authors.initials: F
authors.lastname: Colitto
authors.forename: A
authors.initials: A
authors.lastname: D'Amico
authors.forename: C
authors.initials: C
authors.lastname: Minetti
authors.forename: M
authors.initials: M
authors.lastname: Mirabella
authors.forename: T
authors.initials: T
authors.lastname: Mongini
authors.forename: L
authors.initials: L
authors.lastname: Morandi
authors.forename: N
authors.initials: N
authors.lastname: Dlamini
authors.forename: S
authors.initials: S
authors.lastname: Orcesi
authors.forename: M
authors.initials: M
authors.lastname: Pelliccioni
authors.forename: M
authors.initials: M
authors.lastname: Pane
authors.forename: A
authors.initials: A
authors.lastname: Pini
authors.forename: A V
authors.initials: AV
authors.lastname: Swan
authors.forename: M
authors.initials: M
authors.lastname: Villanova
authors.forename: G
authors.initials: G
authors.lastname: Vita
authors.forename: M
authors.initials: M
authors.lastname: Main
authors.forename: F
authors.initials: F
authors.lastname: Muntoni
authors.forename: E
authors.initials: E
authors.lastname: Bertini
mesh_terms: 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