{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,5]],"date-time":"2026-01-05T22:12:38Z","timestamp":1767651158803},"reference-count":9,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[1993,7,1]],"date-time":"1993-07-01T00:00:00Z","timestamp":741484800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1993,7]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The progress and extent of myelination can be assessed using magnetic resonance imaging (MRI). Myelination is delayed or diminished in several inherited metabolic abnormalities presenting in early life. Only minimal myelination of the CNS occurs in Pelizaeus\u2010Merzbacher disease. Dysmyelination tends to produce fairly symmetrical lesions affecting white matter. In many mitochondrial enzyme and some lysosomal defects, the grey matter is also involved. The appearances and in particular the distribution on MRI and\/or CT are characteristic in some conditions and the diagnosis is limited in others. Demyelination due to inflammatory disorders typically causes multifocal white matter lesions, recurrent in multiple sclerosis, monophasic in acute disseminated encephalomyelitis, extending in progressive multifocal leukoencephalopathy and classically involving the pons or corpus callosum in myelinolysis. Hypoxic ischaemic lesions may be metabolically induced and simulate primary demyelinating disorders. Mitochondrial enzyme defects in particular may present with stroke\u2010like appearances. In many of these conditions, diagnosis is biochemical, but imaging has a significant role in suggesting the diagnosis, and documenting progression, response to therapy or complications.<\/jats:p>","DOI":"10.1007\/bf00711909","type":"journal-article","created":{"date-parts":[[2004,11,30]],"date-time":"2004-11-30T10:29:25Z","timestamp":1101810565000},"page":"771-786","source":"Crossref","is-referenced-by-count":25,"title":["Inborn errors and demyelination: MRI and the diagnosis of white matter disease"],"prefix":"10.1002","volume":"16","author":[{"given":"B. E.","family":"Kendall","sequence":"first","affiliation":[{"name":"Hospital for Sick Children  Great Ormond Street London WC1N 3BG UK"},{"name":"National Hospital for Neurology and Neurosurgery  Queen Square London WC1N 3BG UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1993,7]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"97","volume-title":"Myelin","author":"Brown PE","year":"1984"},{"key":"e_1_2_1_3_1","unstructured":"BarkovichAJ Jackson DEJr (1989)MRI assessment of normal and abnormal brain myelination.MRI Divisions19\u201325.."},{"key":"e_1_2_1_4_1","first-page":"387","article-title":"Partial albinism with immunodeficiency: a rare syndrome with prominent white matter changes","volume":"13","author":"Brismar J","year":"1992","journal-title":"AJNR"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00598954"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00598620"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00588156"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00598955"},{"key":"e_1_2_1_9_1","first-page":"621","article-title":"Disorders of lysosomes, peroxisomes, and mitochondria","volume":"13","author":"Kendall BE","year":"1992","journal-title":"AJNR"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/113.2.291"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF00711909","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF00711909","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T18:26:00Z","timestamp":1694802360000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/BF00711909"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,7]]},"references-count":9,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1993,7]]}},"alternative-id":["10.1007\/BF00711909"],"URL":"https:\/\/doi.org\/10.1007\/bf00711909","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1993,7]]}}}