晚期婴儿型神经元蜡样脂褐质沉积症一例终末期局部脑葡萄糖代谢率降低

Reduced regional cerebral metabolic rate for glucose at the terminal stage in a case of late infantile neuronal ceroid lipofuscinosis.

作者信息

Fueki N, Iinuma K, Kojima A, Yanai K, Haginoya K, Tada K, Ido T, Ito M

机构信息

Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.

出版信息

J Child Neurol. 1990 Apr;5(2):98-100. doi: 10.1177/088307389000500205.

DOI:10.1177/088307389000500205

PMID:2345285

Abstract

Regional cerebral metabolic rate for glucose was determined for six different areas of the gray matter in an 8-year-old girl with late infantile neuronal ceroid lipofuscinosis. In all regions, the rates were almost half of the control values. The regional cerebral metabolic rate for glucose was relatively preserved in the striatal region and severely reduced in the frontal cortex.

摘要

对一名患有晚期婴儿型神经元蜡样脂褐质沉积症的8岁女孩的六个不同灰质区域进行了局部脑葡萄糖代谢率测定。在所有区域，代谢率几乎是对照值的一半。纹状体区域的局部脑葡萄糖代谢率相对保持，而额叶皮质则严重降低。