{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T00:01:05Z","timestamp":1790208065973,"version":"4.1.0"},"reference-count":41,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2015,3,15]],"date-time":"2015-03-15T00:00:00Z","timestamp":1426377600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2015,4,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Spinocerebellar ataxias (SCAs) belong to polyglutamine repeat disorders and are characterized by a predominant atrophy of the cerebellum and the pons. Proton magnetic resonance spectroscopy (<jats:sup>1<\/jats:sup>H MRS) using an optimized semiadiabatic localization by adiabatic selective refocusing (semi\u2010LASER) protocol was performed at 3 T to determine metabolite concentrations in the cerebellar vermis and pons of a cohort of patients with SCA1 (n\u2009=\u200916), SCA2 (n\u2009=\u200912), SCA3 (n\u2009=\u200921), and SCA7 (n\u2009=\u200912) and healthy controls (n\u2009=\u200933). Compared with controls, patients displayed lower total <jats:italic>N<\/jats:italic>\u2010acetylaspartate and, to a lesser extent, lower glutamate, reflecting neuronal loss\/dysfunction, whereas the glial marker, myoinositol (<jats:italic>myo<\/jats:italic>\u2010Ins), was elevated. Patients also showed higher total creatine as reported in Huntington's disease, another polyglutamine repeat disorder. A strong correlation was found between the Scale for the Assessment and Rating of Ataxia and the neurometabolites in both affected regions of patients. Principal component analyses confirmed that neuronal metabolites (total <jats:italic>N<\/jats:italic>\u2010acetylaspartate and glutamate) were inversely correlated in the vermis and the pons to glial (<jats:italic>myo<\/jats:italic>\u2010Ins) and energetic (total creatine) metabolites, as well as to disease severity (motor scales). Neurochemical plots with selected metabolites also allowed the separation of SCA2 and SCA3 from controls. The neurometabolic profiles detected in patients underlie cell\u2010specific changes in neuronal and astrocytic compartments that cannot be assessed by other neuroimaging modalities. The inverse correlation between metabolites from these two compartments suggests a metabolic attempt to compensate for neuronal damage in SCAs. Because these biomarkers reflect dynamic aspects of cellular metabolism, they are good candidates for proof\u2010of\u2010concept therapeutic trials. \u00a9 2015 International Parkinson and Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.26181","type":"journal-article","created":{"date-parts":[[2015,3,15]],"date-time":"2015-03-15T11:19:49Z","timestamp":1426418389000},"page":"662-670","source":"Crossref","is-referenced-by-count":72,"title":["In vivo neurometabolic profiling in patients with spinocerebellar ataxia types 1, 2, 3, and 7"],"prefix":"10.1002","volume":"30","author":[{"given":"Isaac M.","family":"Adanyeguh","sequence":"first","affiliation":[{"name":"INSERM U 1127, Sorbonne Universit\u00e9s, UPMC Univ Paris Institut du Cerveau et de la Moelle \u00e9pini\u00e8re, ICM Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre\u2010Gilles","family":"Henry","sequence":"additional","affiliation":[{"name":"Center for Magnetic Resonance Research, University of Minnesota Minneapolis MN United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tra M.","family":"Nguyen","sequence":"additional","affiliation":[{"name":"INSERM U 1127, Sorbonne Universit\u00e9s, UPMC Univ Paris Institut du Cerveau et de la Moelle \u00e9pini\u00e8re, ICM Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daisy","family":"Rinaldi","sequence":"additional","affiliation":[{"name":"INSERM U 1127, Sorbonne Universit\u00e9s, UPMC Univ Paris Institut du Cerveau et de la Moelle \u00e9pini\u00e8re, ICM Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Celine","family":"Jauffret","sequence":"additional","affiliation":[{"name":"INSERM U 1127, Sorbonne Universit\u00e9s, UPMC Univ Paris Institut du Cerveau et de la Moelle \u00e9pini\u00e8re, ICM Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romain","family":"Valabregue","sequence":"additional","affiliation":[{"name":"Center for NeuroImaging Research, Institut du Cerveau et de la Moelle \u00e9pini\u00e8re Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uzay E.","family":"Emir","sequence":"additional","affiliation":[{"name":"Center for Magnetic Resonance Research, University of Minnesota Minneapolis MN United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dinesh K.","family":"Deelchand","sequence":"additional","affiliation":[{"name":"Center for Magnetic Resonance Research, University of Minnesota Minneapolis MN United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexis","family":"Brice","sequence":"additional","affiliation":[{"name":"INSERM U 1127, Sorbonne Universit\u00e9s, UPMC Univ Paris Institut du Cerveau et de la Moelle \u00e9pini\u00e8re, ICM Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynn E.","family":"Eberly","sequence":"additional","affiliation":[{"name":"Division of Biostatistics School of Public Health, University of Minnesota Minneapolis MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00fclin","family":"\u00d6z","sequence":"additional","affiliation":[{"name":"Center for Magnetic Resonance Research, University of Minnesota Minneapolis MN United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Durr","sequence":"additional","affiliation":[{"name":"INSERM U 1127, Sorbonne Universit\u00e9s, UPMC Univ Paris Institut du Cerveau et de la Moelle \u00e9pini\u00e8re, ICM Paris France"},{"name":"Assistance Publique\u2010H\u00f4pitaux de Paris, F\u00e9d\u00e9ration de G\u00e9n\u00e9tique, La Piti\u00e9\u2010Salp\u00eatri\u00e8re University Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fanny","family":"Mochel","sequence":"additional","affiliation":[{"name":"INSERM U 1127, Sorbonne Universit\u00e9s, UPMC Univ Paris Institut du Cerveau et de la Moelle \u00e9pini\u00e8re, ICM Paris France"},{"name":"Assistance Publique\u2010H\u00f4pitaux de Paris, F\u00e9d\u00e9ration de G\u00e9n\u00e9tique, La Piti\u00e9\u2010Salp\u00eatri\u00e8re University Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,3,15]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-007-0287-5"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(13)70104-2"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(10)70183-6"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000219042.60538.92"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12311-011-0316-8"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12311-008-0026-z"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/7854_2011_166"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.5612-09.2010"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh201"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0047925"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.2232010722"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0047479"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awf278"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/nbm.1940080402"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.23067"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12311-010-0213-6"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.22708"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/nbm.1641"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1522-2594(200002)43:2<319::AID-MRM22>3.0.CO;2-1"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1006\/jmrb.1993.1055"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.1910300604"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrm.25295"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)74053-2"},{"key":"e_1_2_9_25_1","volume-title":"Basic Neurochemistry: Molecular, Cellular and Medical Aspects","author":"Siegel GJ","year":"1999"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.13130531"},{"key":"e_1_2_9_27_1","unstructured":"ProvencherS.LCModel and LCMgui User's Manual. 2014. [Accessed 2014 December 20]. Available from:http:\/\/s\u2010provencher.com\/pub\/LCModel\/manual\/manual.pdf."},{"key":"e_1_2_9_28_1","first-page":"65","article-title":"A simple sequentially rejective multiple test procedure","volume":"6","author":"Holm S","year":"1979","journal-title":"Scand J Statist"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.parkreldis.2004.02.006"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/aws369"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1007\/7854_2011_197"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1159\/000111347"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tins.2010.12.001"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/jmri.21804"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2000.0741968.x"},{"key":"e_1_2_9_36_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0030554"},{"key":"e_1_2_9_37_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M604123200"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrg1691"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M111.309849"},{"key":"e_1_2_9_40_1","doi-asserted-by":"publisher","DOI":"10.1038\/jcbfm.2011.157"},{"key":"e_1_2_9_41_1","doi-asserted-by":"publisher","DOI":"10.1038\/jcbfm.2012.104"},{"key":"e_1_2_9_42_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.25009"}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.26181","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.26181","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T10:57:51Z","timestamp":1760957871000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.26181"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,3,15]]},"references-count":41,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2015,4,15]]}},"alternative-id":["10.1002\/mds.26181"],"URL":"https:\/\/doi.org\/10.1002\/mds.26181","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"value":"0885-3185","type":"print"},{"value":"1531-8257","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,3,15]]}}}