{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T02:11:19Z","timestamp":1790820679596,"version":"4.1.0"},"reference-count":14,"publisher":"Wiley","issue":"S3","license":[{"start":{"date-parts":[[2010,10,1]],"date-time":"2010-10-01T00:00:00Z","timestamp":1285891200000},"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":[[2010,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Neurological involvement in X\u2010linked mucopolysaccharidosis type II (Hunter syndrome) is indicative of more severe disease, but is not attenuated by current enzyme replacement therapy which does not significantly penetrate the blood\u2013brain barrier. Magnetic resonance spectroscopy is an objective method of determining brain metabolites and has the potential to identify disease biomarkers with utility in evaluating current and novel therapies. MRS studies from seven patients with MPSII all receiving enzyme replacement therapy were compared with a large cohort of children with various neurocognitive disorders with normal MR imaging. All studies were completed on 1.5Tesla clinical MR scanners. Brain metabolite concentrations were determined from basal ganglia and parieto\u2010occipital white matter using LCModel quantification. Serial trends in brain metabolites were analysed. Examination of mean spectra and quantitative metabolite concentrations demonstrated significantly decreased white matter N\u2010acetylaspartate (a neuronal marker), total choline and glutamate, and elevated myo\u2010inositol (glial marker) in MPSII patients. Analysis of serial determinations of white matter N\u2010acetylaspartate demonstrated no change in two patients with stable MR imaging features but decreasing N\u2010acetylaspartate in two patients more severely affected or deteriorating. These data demonstrate the utility of MRS to monitor serial alterations in brain metabolites including N\u2010acetylaspartate which could be used as biomarkers of progressive neurological disease in MPSII. Integrated as an adjunct to MRI, such an approach could aid the evaluation of the efficacy of current ERT and also novel CNS\u2010targeted therapies in MPSII.<\/jats:p>","DOI":"10.1007\/s10545-010-9197-0","type":"journal-article","created":{"date-parts":[[2010,9,30]],"date-time":"2010-09-30T06:03:58Z","timestamp":1285826638000},"page":"395-399","source":"Crossref","is-referenced-by-count":15,"title":["Quantitative in vivo brain magnetic resonance spectroscopic monitoring of neurological involvement in mucopolysaccharidosis type II (Hunter Syndrome)"],"prefix":"10.1002","volume":"33","author":[{"given":"James E.","family":"Davison","sequence":"first","affiliation":[{"name":"Clinical Inherited Metabolic Disorders Birmingham Children's Hospital NHS Foundation Trust Birmingham UK"},{"name":"Clinical &amp; Experimental Medicine, College of Medical &amp; Dental Sciences University of Birmingham Birmingham UK"},{"name":"Institute of Child Health Birmingham Children's Hospital NHS Foundation Trust Birmingham B4 6NH UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian J.","family":"Hendriksz","sequence":"additional","affiliation":[{"name":"Clinical Inherited Metabolic Disorders Birmingham Children's Hospital NHS Foundation Trust Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu","family":"Sun","sequence":"additional","affiliation":[{"name":"Cancer Sciences, College of Medical &amp; Dental Sciences University of Birmingham Birmingham UK"},{"name":"Birmingham Children's Hospital NHS Foundation Trust Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nigel P.","family":"Davies","sequence":"additional","affiliation":[{"name":"Cancer Sciences, College of Medical &amp; Dental Sciences University of Birmingham Birmingham UK"},{"name":"Birmingham Children's Hospital NHS Foundation Trust Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Gissen","sequence":"additional","affiliation":[{"name":"Clinical Inherited Metabolic Disorders Birmingham Children's Hospital NHS Foundation Trust Birmingham UK"},{"name":"Clinical &amp; Experimental Medicine, College of Medical &amp; Dental Sciences University of Birmingham Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew C.","family":"Peet","sequence":"additional","affiliation":[{"name":"Cancer Sciences, College of Medical &amp; Dental Sciences University of Birmingham Birmingham UK"},{"name":"Birmingham Children's Hospital NHS Foundation Trust Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,10]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-008-0878-x"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrc1390"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/nbm.792"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-007-0325-3"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-009-1119-7"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-005-0020-z"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.gim.0000232477.37660.fb"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.252784899"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/nbm.698"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.1098"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.52.8.1663"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000269782.80107.fe"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A0510"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-008-1040-5"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-010-9197-0","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T06:21:55Z","timestamp":1694758915000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-010-9197-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,10]]},"references-count":14,"journal-issue":{"issue":"S3","published-print":{"date-parts":[[2010,12]]}},"alternative-id":["10.1007\/s10545-010-9197-0"],"URL":"https:\/\/doi.org\/10.1007\/s10545-010-9197-0","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,10]]}}}