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J Clin Neuromuscul Dis. 2014 Jun;15(4):152-6. doi: 10.1097/CND.0000000000000029.

Sixty six-month follow-up of muscle power and respiratory function in a case with adult-type Pompe disease treated with enzyme replacement therapy.

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  • 1Departments of *Neurology and †Rehabilitation, Kyoto Prefectural University of Medicine, Kyoto, Japan; and ‡Kyoto Prefectural Support Center of Rehabilitation, Kyoto, Japan.


We report a patient with adult-type Pompe disease treated with enzyme replacement therapy (ERT) for 5.5 years. We evaluated pulmonary function and muscle strength using 6-minute walk test, manual muscle test, and dynamometer-based measurement. The long-term ERT resulted in a substantial improvement in the pulmonary function and a possible stabilization followed by mild deterioration in muscle power measured by dynamometer and 6-minute walk test. Our data may rationalize the long-term use of ERT for adult-type Pompe disease in terms of maintaining pulmonary function.

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