{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,3]],"date-time":"2026-05-03T10:06:35Z","timestamp":1777802795185,"version":"3.51.4"},"reference-count":22,"publisher":"SAGE Publications","issue":"8","license":[{"start":{"date-parts":[[1999,8,1]],"date-time":"1999-08-01T00:00:00Z","timestamp":933465600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Child Neurol"],"published-print":{"date-parts":[[1999,8]]},"abstract":"<jats:p>\n                    Cerebral glucose metabolism was investigated in a 4.8-year-old boy with \u03b1- N-acetylgalactosaminidase deficiency using 2-[\n                    <jats:sup>18<\/jats:sup>\n                    F]fluoro-2-deoxy-D-glucose and positron emission tomography (PET). In comparison to normal values for age, the overall cerebral glucose metabolism was reduced and the regional cerebral glucose metabolism was decreased in proportion to the degree of atrophy. In the supratentorial cortical regions, the hypometabolism was asymmetric. However, the level of regional cerebral glucose metabolism in all cortical regions excluded a persistent vegetative state. In the lentiform nucleus and the head of the caudate, comparatively increased regional cerebral glucose metabolism was documented, similar to findings in neurodegenerative disorders with active epilepsy. In contrast, the infratentorial structures (cerebellar hemispheres, brain stem, mesencephalon, and hypothalamus), which are predominantly affected by the atrophic process, showed distinct and symmetric hypometabolism. Thus, the 2-[\n                    <jats:sup>18<\/jats:sup>\n                    F]-fluoro-2-deoxy-D-glucose PET scans provided additional insight into and correlation of the functional and structural disturbances in type I \u03b1- N-acetylgalactosaminidase deficiency, in addition to documenting the hypometabolism due to brain atrophy. (J Child Neurol 1999;14:543-547).\n                  <\/jats:p>","DOI":"10.1177\/088307389901400813","type":"journal-article","created":{"date-parts":[[2008,1,24]],"date-time":"2008-01-24T19:24:33Z","timestamp":1201202673000},"page":"543-547","source":"Crossref","is-referenced-by-count":9,"title":["Cerebral Glucose Metabolism in Type I \u03b1-N-Acetylgalactosaminidase Deficiency: An Infantile Neuroaxonal Dystrophy"],"prefix":"10.1177","volume":"14","author":[{"given":"Jobst","family":"Rudolf","sequence":"first","affiliation":[{"name":"Institute for Neurologic Research Cologne, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Grond","sequence":"additional","affiliation":[{"name":"Institute for Neurologic Research Cologne, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Max","family":"Planck","sequence":"additional","affiliation":[{"name":"Institute for Neurologic Research Cologne, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Detlev","family":"Schindler","sequence":"additional","affiliation":[{"name":"Department of Human Genetics University of Wtrzburg Wfirzburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolf-Dieter","family":"Heiss","sequence":"additional","affiliation":[{"name":"Max Planck Institute for Neurologic Research Cologne, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert J.","family":"Desnick","sequence":"additional","affiliation":[{"name":"Department of Human Genetics Mount Sinai School of Medicine New York, New York"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[1999,8,1]]},"reference":[{"key":"atypb1","unstructured":"Seitelberger F.: Neuroaxonal dystrophy: Its relation to aging and neurological disease, in Vinken PJ, Bruyn GW, Klawans HL (eds): Handbook of Clinical Neurology, vol 49 (rev ser 5). Amsterdam, Elsevier Science, 1986, pp 391-415."},{"key":"atypb2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(87)92542-6"},{"key":"atypb3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198906293202606"},{"key":"atypb4","doi-asserted-by":"publisher","DOI":"10.1172\/JCI115357"},{"key":"atypb5","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1993.01680250072009"},{"key":"atypb6","doi-asserted-by":"publisher","DOI":"10.1007\/BF00712015"},{"key":"atypb7","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)83281-0"},{"key":"atypb8","unstructured":"Desnick RJ, Wang AM: Schindler disease: \u03b1-N-acetylgalactosaminidase deficiency, in Scriver CR, Beaudet A, Sly W, Valle D (eds): The Molecular and Metabolic Bases of Inherited Disease, 7th ed. New York, McGraw-Hill , 1995, pp 2509-2528."},{"key":"atypb9","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410250416"},{"key":"atypb10","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.43.1_Part_1.110"},{"key":"atypb11","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.44.1.127"},{"key":"atypb12","doi-asserted-by":"publisher","DOI":"10.1038\/jcbfm.1984.30"},{"key":"atypb13","doi-asserted-by":"publisher","DOI":"10.1097\/00004728-198501000-00026"},{"key":"atypb14","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410220408"},{"key":"atypb15","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(95)00124-K"},{"key":"atypb16","first-page":"524","volume":"28","author":"Volkow ND","year":"1987","journal-title":"J Nucl Med"},{"key":"atypb17","doi-asserted-by":"publisher","DOI":"10.1016\/0887-8994(93)90074-M"},{"issue":"1","key":"atypb18","first-page":"S391","volume":"13","author":"Rudolf J.","year":"1993","journal-title":"J Cereb Blood Flow Metab"},{"key":"atypb19","first-page":"183","volume":"94","author":"deVolder AG","year":"1994","journal-title":"Acta Neurol Belg"},{"key":"atypb20","doi-asserted-by":"publisher","DOI":"10.1097\/00008506-199504000-00006"},{"key":"atypb21","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410310212"},{"key":"atypb22","doi-asserted-by":"publisher","DOI":"10.1177\/088307389000500205"}],"container-title":["Journal of Child Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/088307389901400813","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/088307389901400813","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T20:34:00Z","timestamp":1777494840000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/088307389901400813"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1999,8]]},"references-count":22,"journal-issue":{"issue":"8","published-print":{"date-parts":[[1999,8]]}},"alternative-id":["10.1177\/088307389901400813"],"URL":"https:\/\/doi.org\/10.1177\/088307389901400813","relation":{},"ISSN":["0883-0738","1708-8283"],"issn-type":[{"value":"0883-0738","type":"print"},{"value":"1708-8283","type":"electronic"}],"subject":[],"published":{"date-parts":[[1999,8]]}}}