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01-03-2010 | Letter to the editors

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

Authors: Fuminobu Sugai, Yosuke Kokunai, Yoichi Yamamoto, Goichi Hashida, Kengo Shimazu, Masahito Mihara, Satoru Inoue, Saburo Sakoda

Published in: Journal of Neurology | Issue 3/2010

Abstract

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.

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Title: Use of the muscle volume analyzer to evaluate enzyme replacement therapy in late-onset Pompe disease
Authors: Fuminobu Sugai
Yosuke Kokunai
Yoichi Yamamoto
Goichi Hashida
Kengo Shimazu
Masahito Mihara
Satoru Inoue
Saburo Sakoda
Publication date: 01-03-2010
Publisher: Springer-Verlag
Published in: Journal of Neurology / Issue 3/2010
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-009-5367-0

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

Abstract

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

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