Glycogen Storage Myopathy with Abnormal Lactate Dehydrogenase

Noriyuki Nagata; Teruhisa Miike; Ichiro Matsuda; Hisataka Shikama; Yukiko Tokumitsu; Michio Ui

doi:10.1055/s-2008-1059607

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Neuropediatrics 1982; 13(2): 103-107
DOI: 10.1055/s-2008-1059607

Glycogen Storage Myopathy with Abnormal Lactate Dehydrogenase

Noriyuki Nagata¹ , Teruhisa Miike¹ , Ichiro Matsuda¹ , Hisataka Shikama² , Yukiko Tokumitsu² , Michio Ui²

¹Department of Pediatrics, Kumamoto University Medical School, Kumamoto
²Department of Physiological Chemistry, Faculty of Pharmaceutical Science, Hokkaido University, Sappro, Japan

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Publication History

Publication Date:
14 May 2008 (online)

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Abstract

We studied a 3 year old girl with mental retardation and limb muscle weakness. The muscle glycogen content was 17.4 mg/g tissue, which was approximately three times higher than normal. There were no other known abnormalities noted in this child that could explain the cause of glycogen storage disease. Our in vitro glycolysis study showed marked increase of pyruvate, but no increase in lactate levels.

The observed results suggested to us that an abnormal lactate dehydrogenase might account for the abnormal accumulation of glycogen in the muscle.

Key words

Glycogen myopathy - Lactate dehydrogenase

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