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. (J Child Neurol 1990;5:98-100).

An 8 year-old-boy with adrenoleukodystrophy (ALD) was subject to an x-ray computed tomography (CT) scan, magnetic resonance imaging (MRI), positron emission tomography (PET) using 18F-fluorodeoxy glucose and evoked potential studies, about 3 months after the onset of neurologic symptoms. A CT showed decreased radiodensity over the white matter of both occipital lobes, and high density areas on the periventricular space. No contrast enhancement was observed around the low dense white matter, while occipital grey matter seemed to be almost normal. MRI demonstrated a larger and more obvious lesion of prolonged T2 relaxation time which was 170-250 msec than that detected by CT scan. The PET demonstrated wide and severe hypometabolism for glucose over the grey and the white matter of bilateral occipital lobes. The visual evoked potential (VEP) was scarcely elicitable, while the auditory brainstem response (ABR) and short latency somatosensory evoked potential (SSEP) was normal. These imaging studies suggest that extensive and profound demyelination exists in the occipital white matter. For detection of demyelinizing process, MRI may be more effective than CT in this disorder. The PET study revealed that there may have been an abnormality of the grey matter as well as the white matter in the occipital lobe. The VEP disappeared in the relatively early stage. The brainstem may not be involved at such early stage in light of normal ABR and SSEP.

Psychiatry and Clinical NeurosciencesVolume 42, Issue 3 p. 611-612 Alteration of Regional Cerebral Metabolic Rate for Glucose at Interictal and Ictal Stages in a Case of Epilepsy with Electrical Status Epilepticus during Slow Sleep N. Fueki M.D., N. Fueki M.D. Department of Pediatrics, Tohoku University School of Medicine, SendaiSearch for more papers by this authorK. Iinuma M.D., K. Iinuma M.D. Department of Pediatrics, Tohoku University School of Medicine, SendaiSearch for more papers by this authorA. Kojima M.D., A. Kojima M.D. Department of Pediatrics, Tohoku University School of Medicine, SendaiSearch for more papers by this authorK. Haginoya M.D., K. Haginoya M.D. Department of Pediatrics, Tohoku University School of Medicine, SendaiSearch for more papers by this authorK. Yanai M.D., K. Yanai M.D. *Cyclotron and Radioisotope Center, Tohoku University, SendaiSearch for more papers by this authorK. Tada M.D., K. Tada M.D. Department of Pediatrics, Tohoku University School of Medicine, SendaiSearch for more papers by this authorT. Ido M.D., T. Ido M.D. *Cyclotron and Radioisotope Center, Tohoku University, SendaiSearch for more papers by this authorT. Matsuzawa M.D., T. Matsuzawa M.D. **Department of Radiology and Nuclear Medicine, Institute for Tuberculosis and Cancer, SendaiSearch for more papers by this author N. Fueki M.D., N. Fueki M.D. Department of Pediatrics, Tohoku University School of Medicine, SendaiSearch for more papers by this authorK. Iinuma M.D., K. Iinuma M.D. Department of Pediatrics, Tohoku University School of Medicine, SendaiSearch for more papers by this authorA. Kojima M.D., A. Kojima M.D. Department of Pediatrics, Tohoku University School of Medicine, SendaiSearch for more papers by this authorK. Haginoya M.D., K. Haginoya M.D. Department of Pediatrics, Tohoku University School of Medicine, SendaiSearch for more papers by this authorK. Yanai M.D., K. Yanai M.D. *Cyclotron and Radioisotope Center, Tohoku University, SendaiSearch for more papers by this authorK. Tada M.D., K. Tada M.D. Department of Pediatrics, Tohoku University School of Medicine, SendaiSearch for more papers by this authorT. Ido M.D., T. Ido M.D. *Cyclotron and Radioisotope Center, Tohoku University, SendaiSearch for more papers by this authorT. Matsuzawa M.D., T. Matsuzawa M.D. **Department of Radiology and Nuclear Medicine, Institute for Tuberculosis and Cancer, SendaiSearch for more papers by this author First published: September 1988 https://doi.org/10.1111/j.1440-1819.1988.tb01379.xCitations: 1AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume42, Issue3September 1988Pages 611-612 RelatedInformation