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

被引：4

作者：

Sugai, Fuminobu ^{[1
]}

Kokunai, Yosuke ^{[1
]}

Yamamoto, Yoichi ^{[1
]}

Hashida, Goichi ^{[2
]}

Shimazu, Kengo ^{[2
]}

Mihara, Masahito ^{[1
]}

Inoue, Satoru ^{[2
]}

Sakoda, Saburo ^{[1
]}

机构：

[1] Osaka Univ, Grad Sch Med, Dept Neurol D4, Suita, Osaka 5650871, Japan

[2] Osaka Univ Hosp, Rehabil Unit, Osaka 553, Japan

来源：

JOURNAL OF NEUROLOGY | 2010年 / 257卷 / 03期

关键词：

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

D O I：

10.1007/s00415-009-5367-0

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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.

引用

页码：461 / 463

页数：3

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