Cerebral oxygen and glucose metabolism in glycogen storage disease with normal acid maltase: Case report

被引：12

作者：

Katsumi, Y

Fukuyama, H

Ogawa, M

Matsui, M

Tokonami, F

Aii, H

Sugie, H

Murakami, N

Nonaka, I

机构：

[1] HAMAMATSU ROSAI HOSP,DEPT NEUROL,HAMAMATSU,SHIZUOKA 430,JAPAN

[2] KYOTO UNIV,FAC MED,DEPT NEUROL,SAKYO KU,KYOTO 606,JAPAN

[3] HAMAMATSU CITY CTR DEV MED,DEPT PEDIAT NEUROL,HAMAKITA,SHIZUOKA 434,JAPAN

[4] HAMAMATSU CITY CTR DEV MED,NEUROMUSC LAB,HAMAKITA,SHIZUOKA 434,JAPAN

[5] NATL INST NEUROSCI,NCNP,DIV ULTRASTRUCT RES,KODAIRA,TOKYO 187,JAPAN

来源：

JOURNAL OF THE NEUROLOGICAL SCIENCES | 1996年 / 140卷 / 1-2期

关键词：

glycogen storage disease with normal acid maltase; mental retardation; proximal myopathy; cardiomyopathy; positron emission tomography;

D O I：

10.1016/0022-510X(96)00077-9

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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.

引用

页码：46 / 52

页数：7

共 21 条

[1] NON-INVASIVE TOMOGRAPHIC STUDY OF CEREBRAL BLOOD-FLOW AND OXYGEN-METABOLISM INVIVO - POTENTIALS, LIMITATIONS, AND CLINICAL-APPLICATIONS IN CEREBRAL ISCHEMIC DISORDERS [J].