{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,3]],"date-time":"2026-05-03T09:58:40Z","timestamp":1777802320728,"version":"3.51.4"},"reference-count":21,"publisher":"SAGE Publications","issue":"6","license":[{"start":{"date-parts":[[1997,9,1]],"date-time":"1997-09-01T00:00:00Z","timestamp":873072000000},"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":[[1997,9]]},"abstract":"<jats:p>\n                    Twelve living patients (aged 19 months to 32 years) with aspartylglucosaminuria were examined by magnetic resonance imaging (MRI), and the magnetic resonance (MR) images of 16 healthy volunteers (aged 4 to 32 years) were used as controls. One patient was examined twice. Postmortem MRI and histopathologic analysis were done on the brains of four additional adult patients. Signal intensities determined quantitatively on T\n                    <jats:sub>2<\/jats:sub>\n                    -weighted images differed significantly between patients and controls, being higher from the white matter (P &lt; .0002) and lower from the thalami (P &lt; .03) in the patients. The generally increased signal intensity of the white matter was most obvious in the young patients, with many focal areas of very high signal intensity in the subcortical white matter. The subcortical white matter showed a somewhat increased signal intensity even at the age of 32 years. In two of the four postmortem MR images, the distinction between the gray and white matter was still poor. At histopathologic analysis, the basic cortical cytoarchitecture was generally preserved but most neurons contained vacuoles, which were also found in the neurons of the deep gray matter. In two of the four autopsy cases the white matter showed diffuse pallor of myelin staining and some gliosis. Thus aspartylglucosaminuria is primarily a gray-matter disease also affecting white matter by delaying myelination. (J Child Neurol 1997;12:369-375).\n                  <\/jats:p>","DOI":"10.1177\/088307389701200606","type":"journal-article","created":{"date-parts":[[2008,12,24]],"date-time":"2008-12-24T02:13:25Z","timestamp":1230084805000},"page":"369-375","source":"Crossref","is-referenced-by-count":27,"title":["Aspartylglucosaminuria: Radiologic Course of the Disease With Histopathologic Correlation"],"prefix":"10.1177","volume":"12","author":[{"given":"Taina","family":"Autti","sequence":"first","affiliation":[{"name":"Department of Pediatric Neurology Children's Hospital, University of Helsinki, Department of Radiology Helsinki University Central Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raili","family":"Raininko","sequence":"additional","affiliation":[{"name":"Department of Radiology University of Uppsala"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matti","family":"Haltia","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Helsinki"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leena","family":"Lauronen","sequence":"additional","affiliation":[{"name":"Department of Radiology Helsinki University Central Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sanna-Leena","family":"Vanhanen","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology Children's Hospital, University of Helsinki"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oili","family":"Salonen","sequence":"additional","affiliation":[{"name":"Department of Radiology Helsinki University Central Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hannu J.","family":"Aronen","sequence":"additional","affiliation":[{"name":"Department of Radiology Helsinki University Central Hospital"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kari","family":"Wirtavuori","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Children's Hospital, University of Helsinki, Helsinki, Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pirkko","family":"Santavuori","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology Children's Hospital, University of Helsinki"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[1997,9,1]]},"reference":[{"key":"atypb1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1993.tb12761.x"},{"key":"atypb2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1993.tb12725.x"},{"key":"atypb3","first-page":"1215","volume":"36","author":"Aula P.","year":"1984","journal-title":"Am J Hum Genet"},{"key":"atypb4","doi-asserted-by":"publisher","DOI":"10.1007\/BF00193202"},{"key":"atypb5","doi-asserted-by":"publisher","DOI":"10.1042\/bj1410141"},{"key":"atypb6","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(66)90344-5"},{"key":"atypb7","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1973.tb01161.x"},{"key":"atypb8","doi-asserted-by":"publisher","DOI":"10.1016\/0888-7543(92)90452-X"},{"key":"atypb9","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.1993.tb04114.x"},{"key":"atypb10","doi-asserted-by":"publisher","DOI":"10.1007\/BF00684563"},{"key":"atypb11","doi-asserted-by":"publisher","DOI":"10.1007\/BF00342387"},{"key":"atypb12","doi-asserted-by":"publisher","DOI":"10.1007\/BF00496982"},{"key":"atypb13","unstructured":"Barcovich J.: Normal development of the neonatal and infant brain, skull, and spine , in Barcovich J (ed): Pediatric Neuroimaging . New York, Raven Press, 1995, pp 9-54."},{"key":"atypb14","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199511023331808"},{"key":"atypb15","doi-asserted-by":"publisher","DOI":"10.1007\/BF00600432"},{"key":"atypb16","first-page":"47","volume":"1","author":"Autio S.","year":"1972","journal-title":"J Ment Defic Res Monogr Series"},{"key":"atypb17","first-page":"1443","volume":"15","author":"Vanhanen S-L.","year":"1994","journal-title":"AJNR Am J Neuro-radiol"},{"key":"atypb18","doi-asserted-by":"publisher","DOI":"10.1177\/028418519203300503"},{"key":"atypb19","unstructured":"Barcovich J. : Toxic and metabolic brain disorders, in Barcovich J (ed): Pediatric Neuroimaging . New York , Raven Press, 1995, pp 55-105."},{"key":"atypb20","doi-asserted-by":"publisher","DOI":"10.1016\/0887-8994(92)90032-T"},{"key":"atypb21","doi-asserted-by":"publisher","DOI":"10.1177\/088307389501000604"}],"container-title":["Journal of Child Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/088307389701200606","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/088307389701200606","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T20:32:26Z","timestamp":1777494746000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/088307389701200606"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1997,9]]},"references-count":21,"journal-issue":{"issue":"6","published-print":{"date-parts":[[1997,9]]}},"alternative-id":["10.1177\/088307389701200606"],"URL":"https:\/\/doi.org\/10.1177\/088307389701200606","relation":{},"ISSN":["0883-0738","1708-8283"],"issn-type":[{"value":"0883-0738","type":"print"},{"value":"1708-8283","type":"electronic"}],"subject":[],"published":{"date-parts":[[1997,9]]}}}