Neuronal ceroid-lipofuscinosis: preferential metabolic alterations in thalamus and posterior association cortex demonstrated by PET. Issue 12 (December 1990)

• Record Type: Journal Article
• Authors: De Volder, A G; Cirelli, S; de Barsy, T; Brucher, J M; Bol, A; Michel, C; Goffinet, A M
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 53:Issue 12(1990)
• Page Start: 1063
• Page End: 1067
• Publication Date: 1990-12
• Subjects: Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp.53.12.1063
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : 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.