PT  - JOURNAL ARTICLE
AU  - A G De Volder
AU  - S Cirelli
AU  - T de Barsy
AU  - J M Brucher
AU  - A Bol
AU  - C Michel
AU  - A M Goffinet
TI  - Neuronal ceroid-lipofuscinosis: preferential metabolic alterations in thalamus and posterior association cortex demonstrated by PET.
AID  - 10.1136/jnnp.53.12.1063
DP  - 1990 Dec 01
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - 1063--1067
VI  - 53
IP  - 12
4099  - http://jnnp.bmj.com/content/53/12/1063.short
4100  - http://jnnp.bmj.com/content/53/12/1063.full
SO  - J Neurol Neurosurg Psychiatry1990 Dec 01; 53
AB  - 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.