NEURONAL CEROID-LIPOFUSCINOSIS - PREFERENTIAL METABOLIC ALTERATIONS IN THALAMUS AND POSTERIOR ASSOCIATION CORTEX DEMONSTRATED BY PET
NEURONAL CEROID-LIPOFUSCINOSIS - PREFERENTIAL METABOLIC ALTERATIONS IN THALAMUS AND POSTERIOR ASSOCIATION CORTEX DEMONSTRATED BY PET
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DOI：
10.1136/jnnp.53.12.1063
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发表时间：
1990-12-01
影响因子：
11
通讯作者：
GOFFINET, AM
中科院分区：
文献类型：
--
作者：
DEVOLDER, AG;CIRELLI, S;GOFFINET, AM
用正电子发射断层扫描(PET)和氟脱氧葡萄糖(FDG)研究了4例神经性蜡样脂褐素沉着症的兄弟姐妹的局部脑葡萄糖利用情况。发现了一个一致的模式，即所有灰质结构中葡萄糖利用的减少，但在丘脑和后联合皮质的水平上更明显。代谢异常的严重程度与临床损害程度和病程相关；在年龄最大的患者中，代谢异常最严重，在其他两名患者中，代谢异常的严重程度居中，在疾病发展周期最短的受试者中，代谢异常的严重程度最小。这些观察表明，PET对于确定代谢性疾病的解剖靶点和研究其病理生理学是有用的。
Regional brain glucose utilisation was investigated with positron emission tomography (PET) and fluorodeoxyglucose (FDG) in four siblings with neuronal ceroid-lipofuscinosis. A consistent pattern was found, namely a decrease of glucose utilisation in all grey structures but more marked at the level of the thalamus and posterior association cortex. The severity of metabolic anomalies was correlated with the degree of clinical impairment and with disease duration; they were the most severe in the oldest patient, who was also the most affected clinically, intermediate in two others, and minimal in the subject with the shortest period of development of the disease. These observations suggest that PET is useful for the definition of anatomical targets of metabolic diseases and for the investigation of their pathophysiology.