Compound muscle action potential and motor function in children with spinal muscular atrophy.
Muscle Nerve
; 42(5): 703-8, 2010 Nov.
Article
en En
| MEDLINE
| ID: mdl-20737553
ABSTRACT
Reliable outcome measures that reflect the underlying disease process and correlate with motor function in children with SMA are needed for clinical trials. Maximum ulnar compound muscle action potential (CMAP) data were collected at two visits over a 4-6-week period in children with SMA types II and III, 2-17 years of age, at four academic centers. Primary functional outcome measures included the Modified Hammersmith Functional Motor Scale (MHFMS) and MHFMS-Extend. CMAP negative peak amplitude and area showed excellent discrimination between the ambulatory and non-ambulatory SMA cohorts (ROC = 0.88). CMAP had excellent test-retest reliability (ICC = 0.96-0.97, n = 64) and moderate to strong correlation with the MHFMS and MHFMS-Extend (r = 0.61-0.73, n = 68, P < 0.001). Maximum ulnar CMAP amplitude and area is a feasible, valid, and reliable outcome measure for use in pediatric multicenter clinical trials in SMA. CMAP correlates well with motor function and has potential value as a relevant surrogate for disease status.
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Potenciales de Acción
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Atrofias Musculares Espinales de la Infancia
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Músculo Esquelético
Tipo de estudio:
Clinical_trials
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Prognostic_studies
Límite:
Adolescent
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Child
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Child, preschool
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Female
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Humans
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Male
Idioma:
En
Revista:
Muscle Nerve
Año:
2010
Tipo del documento:
Article
País de afiliación:
Estados Unidos