Journal of child neurology · 2011
Validation of the Expanded Hammersmith Functional Motor Scale in spinal muscular atrophy type II and III.
СтатьяПоддержка NIH: внешняя программаПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Glanzman Allan M, O'Hagen Jessica M, McDermott Michael P, Martens William B, Flickinger Jean, Riley Susan, Quigley Janet, Montes Jacqueline, Dunaway Sally, Deng Liyong, Chung Wendy K, Tawil Rabi, Darras Basil T, De Vivo Darryl C, Kaufmann Petra, Finkel Richard S, Pediatric Neuromuscular Clinical Research Network for Spinal Muscular Atrophy (PNCR), Muscle Study Group (MSG)
Аннотация
The relationships between the Expanded Hammersmith Functional Motor Scale (HFMSE) and genotype and motor and respiratory outcomes were examined in patients with spinal muscular atrophy types II and III (n = 70). The correlation between the HFMSE and Gross Motor Function Measure was r = 0.98. Correlations between HFMSE and forced vital capacity (percentage of predicted normal) (n = 56) and a functional rating (n = 57) were r = 0.87 and r = 0.92, respectively. Correlations with strength were as follows: knee extension, r = 0.74 (n = 60); elbow flexion, r = 0.77 (n = 61); and knee flexion, r = 0.74 (n = 58). The HFMSE differentiated patients by SMN2 copy number (P = .0007); bi-level positive airway pressure use, <8 versus ≥8 hours/day (P < .0001); ambulatory status (P < .0001); and spinal muscular atrophy type (P < .0001). The HFMSE demonstrates significant associations with established measures of function, strength, and genotype, and discriminates patients based on function, diagnostic category, and bi-level positive airway pressure need. Time of administration averaged 12 minutes. The HFMSE is a valid, time-efficient outcome measure for clinical trials in spinal muscular atrophy types II and III.