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 were measured with a dynamometer, and pulmonary function was evaluated using spirometry in 7 adult patients. The six-minute walk test and the four-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 differentially affected 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 © 2013 Wiley Periodicals, Inc.
Illes, Z., Mike, A., Trauninger, A., Várdi, K., & Váczi, M. (2014). Motor function and respiratory capacity in patients with late-onset pompe disease. Muscle & Nerve, 49(4), 603–606. https://doi.org/10.1002/mus.24142