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Updated: Oct 21, 2026

Biochemical Titration of Glycogen In vitro
Published on: November 24, 2013
Cerebral oxygen and glucose metabolism in glycogen storage disease with normal acid maltase: case report
Y Katsumi1, H Fukuyama, M Ogawa
1Department of Neurology, Hamamatsu Rosai Hospital 25 Shogen-cho, Shizuoka, Japan.
Abstract:
A 26-year-old male with cardiomyopathy, cervical muscle weakness and mental retardation was diagnosed as having glycogen storage disease with normal acid maltase on the basis of his clinical, pathological and biochemical findings. Positron emission tomography showed that cerebral oxygen metabolism was normal, while cerebral glucose metabolism was decreased in the cerebral cortexes. The decrease of the glucose metabolic rate may reflect an abnormality of cerebral glucose metabolism in this disorder and may be related to mental retardation, which is one of the characteristic symptoms.
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