Reliability of the Hammersmith functional motor scale for spinal muscular atrophy in a multicentric study

被引:60
作者
Mercuri, E
Messina, S
Battini, R
Berardinelli, A
Boffi, P
Bono, R
Bruno, C
Carboni, N
Cini, C
Colitto, F
D'Amico, A
Minetti, C
Mirabella, M
Mongini, T
Morandi, L
Dlamini, N
Orcesi, S
Pelliccioni, M
Pane, M
Pini, A
Swan, AV
Villanova, M
Vita, G
Main, A
Muntoni, F
Bertini, E
机构
[1] Univ Sacred Heart, Dept Paediat Neurol, I-00168 Rome, Italy
[2] Univ London Imperial Coll Sci Technol & Med, Hammersmith Hosp, Dubowitz Neuromuscular Ctr, London, England
[3] Univ Messina, Dept Neurosci Psychiat & Anaesthesiol, I-98100 Messina, Italy
[4] IRCCS Stella Maris, Dept Dev Neurosci, Pisa, Italy
[5] Univ Pavia, IRCCS, C Mondino Fdn, I-27100 Pavia, Italy
[6] Univ Turin, Dept Child Neuropsychiat, I-10124 Turin, Italy
[7] Natl Neurol Inst C Besta, Div Dev Neurol, Milan, Italy
[8] G Gaslini Inst Children, Neuromuscular Dis Unit, Genoa, Italy
[9] Bambino Gesu Pediat Hosp, Dept Lab Med, Mol Med Unit, Rome, Italy
[10] Catholic Univ Rome, Inst Neurol, Rome, Italy
[11] Univ Turin, SG Battista Hosp, Neuromuscular Ctr, I-10124 Turin, Italy
[12] Natl Neurol Inst C Besta, Div Neuromuscular Dis, Milan, Italy
[13] UILDM, Rome, Italy
[14] Maggiore Hosp, Child Neurol & Psychiat Unit, Bologna, Italy
[15] Guys Kings & St Thomas Sch Med, Dept Child Neurosci, London, England
[16] Nigrisoli Hosp, Bologna, Italy
关键词
SMA; functional abilities; validation; reliability; Hammersmith functional motor scale; clinical trial;
D O I
10.1016/j.nmd.2005.11.010
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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. (C) 2005 Elsevier B.V. All rights reserved.
引用
收藏
页码:93 / 98
页数:6
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