Physical function and mobility in children with congenital myotonic dystrophy
| Autor: | Craig Campbell, Deanna L. Dibella, Russell J. Butterfield, Becky Crockett, Man Hung, Nicholas E. Johnson, Jerry Bounsanga, Melissa Dixon, Evan M Pucillo |
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| Rok vydání: | 2017 |
| Předmět: |
medicine.medical_specialty
Congenital Myotonic Dystrophy Physiology business.industry Physical function medicine.disease Myotonic dystrophy 03 medical and health sciences Cellular and Molecular Neuroscience Grip strength 0302 clinical medicine Physical medicine and rehabilitation 030225 pediatrics Physiology (medical) Hand strength Lean body mass Physical therapy Biomarker (medicine) Medicine Neurology (clinical) medicine.symptom business 030217 neurology & neurosurgery Muscle contraction |
| Zdroj: | Muscle & Nerve. 56:224-229 |
| ISSN: | 0148-639X |
| Popis: | Introduction Congenital myotonic dystrophy (CDM) occurs when symptoms of myotonic dystrophy present at birth. In this study we evaluated the relationship between physical function, muscle mass, and age to provide an assessment of the disease and help prepare for therapeutic trials. Methods CDM participants performed timed functional tests (TFTs), the first 2 minutes of 6-minute walk tests (2/6MWTs), and myometry tests, and also performed dual-energy X-ray absorption (DEXA) scans. Healthy controls (HCs) performed TFTs, 6MWTs, and myometry. Results Thirty-seven children with CDM and 27 HCs (age range 3-13 years) participated in the study. There were significant differences in the 10-meter walk (11.3 seconds in CDM vs. 6.8 seconds in HC) and 2MWT (91 meters in CDM vs. 193 meters in HCs). DEXA lean mass of the right arm correlated with grip strength (r = 0.91), and lean mass of the right leg correlated with 6MWT (r = 0.62). Conclusion Children with CDM have significant limitations in strength and mobility. The tests performed were reliable, and lean muscle mass may serve as a useful biomarker. Muscle Nerve 56: 224-229, 2017. |
| Databáze: | OpenAIRE |
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