De Volder A G, Cirelli S, de Barsy T, Brucher J M, Bol A, Michel C, Goffinet A M
Positron Tomography Laboratory, University of Louvain, Belgium.
J Neurol Neurosurg Psychiatry. 1990 Dec;53(12):1063-7. doi: 10.1136/jnnp.53.12.1063.
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.
利用正电子发射断层扫描（PET）和氟脱氧葡萄糖（FDG）对4名患有神经元蜡样脂褐质沉积症的兄弟姐妹进行了局部脑葡萄糖利用情况的研究。发现了一种一致的模式，即在所有灰质结构中葡萄糖利用均减少，但在丘脑和后联合皮质水平更为明显。代谢异常的严重程度与临床损害程度及病程相关；在年龄最大的患者中最为严重，该患者也是临床症状最严重的，另外两名患者症状中等，而在疾病发展时间最短的患者中症状最轻。这些观察结果表明，PET对于确定代谢性疾病的解剖学靶点及其病理生理学研究是有用的。
