Motor function and respiratory capacity in patients with late‐onset pompe disease |
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| Authors: | Zsolt Illes MD PhD Andrea Mike MD PhD Anita Trauninger MD PhD Katalin Várdi MD Márk Váczi PhD |
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| Institution: | 1. Department of Neurology, Odense University Hospital, Institute of Clinical Research, University of Southern Denmark, , Odense, Denmark;2. Department of Neurology, University of Pecs, , Pecs, Hungary;3. Department of Pulmonary Rehabilitation and Sleep Medicine, Torokbalint Chest Hospital, , Torokbalint, Hungary;4. Institute of Sport Science and Physical Education, University of Pecs, , Pecs, Hungary |
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| Abstract: | Introduction: The relationship between skeletal muscle strength and respiratory dysfunction in Pompe disease has not been examined by quantitative methods. We investigated correlations among lower extremity proximal muscle strength, respiratory function, and motor performance. Methods: Concentric strength of the knee extensor and flexor muscles was measured with a dynamometer, and pulmonary function was evaluated using spirometry in 7 adult patients. The 6‐minute walk test and the 4‐step stair‐climb test were used for assessing aerobic endurance and anaerobic power, respectively. Results: Anaerobic motor performance correlated with strength of both thigh muscles. Respiratory function did not correlate with either muscle strength or motor function performance. Conclusions: Respiratory and lower extremity proximal muscles could be affected differentially by the disease in individual patients. Motor performance is influenced by thigh muscle strength and is less dependent of respiratory capacity in our cohort of ambulatory patients. Muscle Nerve 49:603–606, 2014 |
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| Keywords: | motor function Pompe disease quantitative dynamometry respiratory function thigh muscle |
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