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1.
Muscle Nerve ; 52(4): 559-67, 2015 Oct.
Article in English | MEDLINE | ID: mdl-25597614

ABSTRACT

INTRODUCTION: Preliminary evidence in adults with spinal muscular atrophy (SMA) and in SMA animal models suggests exercise has potential benefits in improving or stabilizing muscle strength and motor function. METHODS: We evaluated feasibility, safety, and effects on strength and motor function of a home-based, supervised progressive resistance strength training exercise program in children with SMA types II and III. Up to 14 bilateral proximal muscles were exercised 3 times weekly for 12 weeks. RESULTS: Nine children with SMA, aged 10.4 ± 3.8 years, completed the resistance training exercise program. Ninety percent of visits occurred per protocol. Training sessions were pain-free (99.8%), and no study-related adverse events occurred. Trends in improved strength and motor function were observed. CONCLUSIONS: A 12-week supervised, home-based, 3-day/week progressive resistance training exercise program is feasible, safe, and well tolerated in children with SMA. These findings can inform future studies of exercise in SMA.


Subject(s)
Muscular Atrophy, Spinal/rehabilitation , Resistance Training/methods , Treatment Outcome , Adolescent , Child , Child, Preschool , Female , Humans , Male , Motor Activity/physiology , Muscle Strength , Muscle, Skeletal/physiopathology , Muscular Atrophy, Spinal/physiopathology , Reflex/physiology , Reproducibility of Results
2.
Pediatr Phys Ther ; 25(2): 140-8; discussion 149, 2013.
Article in English | MEDLINE | ID: mdl-23542189

ABSTRACT

PURPOSE: This study examined the reliability and validity of the Test of Infant Motor Performance Screening Items (TIMPSI) in infants with type I spinal muscular atrophy (SMA). METHODS: After training, 12 evaluators scored 4 videos of infants with type I SMA to assess interrater reliability. Intrarater and test-retest reliability was further assessed for 9 evaluators during a SMA type I clinical trial, with 9 evaluators testing a total of 38 infants twice. Relatedness of the TIMPSI score to ability to reach and ventilatory support was also examined. RESULTS: Excellent interrater video score reliability was noted (intraclass correlation coefficient, 0.97-0.98). Intrarater reliability was excellent (intraclass correlation coefficient, 0.91-0.98) and test-retest reliability ranged from r = 0.82 to r = 0.95. The TIMPSI score was related to the ability to reach (P ≤ .05). CONCLUSION: The TIMPSI can reliably be used to assess motor function in infants with type I SMA. In addition, the TIMPSI scores are related to the ability to reach, an important functional skill in children with type I SMA.


Subject(s)
Physical Therapy Modalities , Spinal Muscular Atrophies of Childhood/diagnosis , Child, Preschool , Female , Humans , Infant, Newborn , Male , Motor Skills , Observer Variation , Reproducibility of Results , Spinal Muscular Atrophies of Childhood/rehabilitation , Surveys and Questionnaires , Time Factors
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