Use of the muscle volume analyzer to evaluate enzyme replacement therapy in late-onset Pompe disease

作者：Sugai Fuminobu; Kokunai Yosuke; Yamamoto Yoichi^*; Hashida Goichi; Shimazu Kengo; Mihara Masahito; Inoue Satoru; Sakoda Saburo

来源：Journal of Neurology, 2010, 257(3): 461-463.

DOI：10.1007/s00415-009-5367-0

• Pompe disease
• Late onset
• Muscle volume analyzer
• Bioelectric impedance
• Handheld dynamometer

The muscle volume analyzer (MVA) can predict limb muscle weight based on bioelectric impedance analysis, whereas the conventional handheld dynamometer (HHD) measures muscle strength. In this study, a 26-year-old female on invasive ventilation due to late-onset Pompe disease was treated with enzyme replacement therapy (ERT) for 12 months. MVA measurements demonstrated time-dependent improvement from the baseline compared to HHD measurements, showing remarkably fluctuating muscle strength. Thus, the MVA can be used as an alternative, particularly for patients suffering from severe limb muscle weakness.

• 出版日期2010-3