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

E. Mercuri, S. Messina, R. Battini, A. Berardinelli, P. Boffi, R. Bono, C. Bruno, N. Carboni, C. Cini, F. Colitto, A. D'Amico, C. Minetti, M. Mirabella, T. Mongini, L. Morandi, N. Dlamini, S. Orcesi, M. Pelliccioni, M. Pane, A. PiniA. V. Swan, M. Villanova, G. Vita, M. Main, F. Muntoni, E. Bertini

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)93-98
Number of pages6
JournalNeuromuscular Disorders
Volume16
Issue number2
DOIs
Publication statusPublished - Feb 2006

Keywords

  • Clinical trial
  • Functional abilities
  • Hammersmith functional motor scale
  • Reliability
  • SMA
  • Validation

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Neurology

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