{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T02:43:14Z","timestamp":1776652994416,"version":"3.51.2"},"reference-count":31,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2026,3,17]],"date-time":"2026-03-17T00:00:00Z","timestamp":1773705600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2026,3,17]],"date-time":"2026-03-17T00:00:00Z","timestamp":1773705600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP 24K10827"],"award-info":[{"award-number":["JP 24K10827"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP 22H04923"],"award-info":[{"award-number":["JP 22H04923"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Intramural Research Grant for Neurological and Psychiatric Disorders of the National Center of Neurology and Psychiatry","award":["6-10"],"award-info":[{"award-number":["6-10"]}]},{"name":"Integrated Research Initiative for Living Well with Dementia (IRIDE) of the Tokyo Metropolitan Institute for Geriatrics and Gerontology IRIDE"},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP24wm0425019"],"award-info":[{"award-number":["JP24wm0425019"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP24dk0207074h0001"],"award-info":[{"award-number":["JP24dk0207074h0001"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"name":"MHLW Research on rare and intractable diseases Program","award":["JPMH23FC1008"],"award-info":[{"award-number":["JPMH23FC1008"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2026,4]]},"DOI":"10.1007\/s00415-026-13744-x","type":"journal-article","created":{"date-parts":[[2026,3,17]],"date-time":"2026-03-17T14:51:27Z","timestamp":1773759087000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Quantitative susceptibility mapping of dentate nucleus iron in SCA6 and SCA31: comparison with pathological findings"],"prefix":"10.1007","volume":"273","author":[{"given":"Risa","family":"Kagaya","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9305-7131","authenticated-orcid":false,"given":"Noriko","family":"Sato","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuko","family":"Saito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroyuki","family":"Maki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hirohito","family":"Kan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yukio","family":"Kimura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoko","family":"Shigemoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuichi","family":"Morita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshito","family":"Saito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuji","family":"Takahashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ukihide","family":"Tateishi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,3,17]]},"reference":[{"key":"13744_CR1","doi-asserted-by":"publisher","first-page":"135","DOI":"10.5692\/clinicalneurol.cn-001931","volume":"64","author":"I Yabe","year":"2024","unstructured":"Yabe I (2024) Recent clinical advances in hereditary spinocerebellar degeneration. Rinsho Shinkeigaku 64:135\u2013147. https:\/\/doi.org\/10.5692\/clinicalneurol.cn-001931","journal-title":"Rinsho Shinkeigaku"},{"key":"13744_CR2","doi-asserted-by":"publisher","first-page":"174","DOI":"10.1159\/000358801","volume":"42","author":"L Ruano","year":"2014","unstructured":"Ruano L, Melo C, Silva MC, Coutinho P (2014) The global epidemiology of hereditary ataxia and spastic paraplegia: a systematic review of prevalence studies. Neuroepidemiology 42:174\u2013183. https:\/\/doi.org\/10.1159\/000358801","journal-title":"Neuroepidemiology"},{"key":"13744_CR3","doi-asserted-by":"publisher","first-page":"1973","DOI":"10.1093\/hmg\/6.11.1973","volume":"6","author":"C Jodice","year":"1997","unstructured":"Jodice C, Mantuano E, Veneziano L, Trettel F, Sabbadini G, Calandriello L et al (1997) Episodic Ataxia Type 2 (EA2) and Spinocerebellar Ataxia Type 6 (SCA6) due to CAG repeat expansion in the CACNA1A gene on chromosome 19p. Hum Mol Genet 6:1973\u20131978. https:\/\/doi.org\/10.1093\/hmg\/6.11.1973","journal-title":"Hum Mol Genet"},{"key":"13744_CR4","doi-asserted-by":"publisher","first-page":"544","DOI":"10.1016\/j.ajhg.2009.09.019","volume":"85","author":"N Sato","year":"2009","unstructured":"Sato N, Amino T, Kobayashi K, Asakawa S, Ishiguro T, Tsunemi T et al (2009) Spinocerebellar Ataxia Type 31 is associated with \u201cinserted\u201d penta-nucleotide repeats containing (TGGAA)n. Am J Hum Genet 85:544\u2013557. https:\/\/doi.org\/10.1016\/j.ajhg.2009.09.019","journal-title":"Am J Hum Genet"},{"key":"13744_CR5","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2022.120527","volume":"444","author":"J Saucier","year":"2023","unstructured":"Saucier J, Al-Qadi M, Amor MB, Ishikawa K, Chamard-Witkowski L (2023) Spinocerebellar Ataxia Type 31: a clinical and radiological literature review. J Neurol Sci 444:120527. https:\/\/doi.org\/10.1016\/j.jns.2022.120527","journal-title":"J Neurol Sci"},{"key":"13744_CR6","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1136\/jnnp.64.1.67","volume":"64","author":"L Sch\u00f6ls","year":"1998","unstructured":"Sch\u00f6ls L, Kr\u00fcger R, Amoiridis G, Przuntek H, Epplen JT, Riess O (1998) Spinocerebellar Ataxia Type 6: genotype and phenotype in German kindreds. J Neurol Neurosurg Psychiatry 64:67\u201373. https:\/\/doi.org\/10.1136\/jnnp.64.1.67","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"13744_CR7","doi-asserted-by":"publisher","first-page":"1045","DOI":"10.1016\/s1474-4422(14)70117-6","volume":"13","author":"RJ Ward","year":"2014","unstructured":"Ward RJ, Zucca FA, Duyn JH, Crichton RR, Zecca L (2014) The role of iron in brain ageing and neurodegenerative disorders. Lancet Neurol 13:1045\u20131060. https:\/\/doi.org\/10.1016\/s1474-4422(14)70117-6","journal-title":"Lancet Neurol"},{"key":"13744_CR8","doi-asserted-by":"publisher","first-page":"1139","DOI":"10.1038\/s41380-023-02399-z","volume":"29","author":"S Levi","year":"2024","unstructured":"Levi S, Ripamonti M, Moro AS, Cozzi A (2024) Iron imbalance in neurodegeneration. Mol Psychiatry 29:1139\u20131152. https:\/\/doi.org\/10.1038\/s41380-023-02399-z","journal-title":"Mol Psychiatry"},{"key":"13744_CR9","doi-asserted-by":"publisher","first-page":"2625","DOI":"10.1016\/j.neuroimage.2011.08.077","volume":"59","author":"B Bilgic","year":"2012","unstructured":"Bilgic B, Pfefferbaum A, Rohlfing T, Sullivan EV, Adalsteinsson E (2012) MRI estimates of brain iron concentration in normal aging using quantitative susceptibility mapping. Neuroimage 59:2625\u20132635. https:\/\/doi.org\/10.1016\/j.neuroimage.2011.08.077","journal-title":"Neuroimage"},{"key":"13744_CR10","doi-asserted-by":"publisher","DOI":"10.1093\/braincomms\/fcab306","volume":"4","author":"A Deistung","year":"2022","unstructured":"Deistung A, J\u00e4schke D, Draganova R, Pfaffenrot V, Hulst T, Steiner KM et al (2022) Quantitative susceptibility mapping reveals alterations of dentate nuclei in common types of degenerative cerebellar ataxias. Brain Commun 4:fcab306. https:\/\/doi.org\/10.1093\/braincomms\/fcab306","journal-title":"Brain Commun"},{"key":"13744_CR11","doi-asserted-by":"publisher","DOI":"10.1093\/braincomms\/fcac007","volume":"4","author":"IH Harding","year":"2022","unstructured":"Harding IH, Ward PGD (2022) Brain susceptibility imaging provides valuable in vivo insights into cerebellar diseases, but biological interpretations remain elusive. Brain Commun 4:fcac007. https:\/\/doi.org\/10.1093\/braincomms\/fcac007","journal-title":"Brain Commun"},{"key":"13744_CR12","doi-asserted-by":"publisher","DOI":"10.3390\/bioengineering12050473","author":"F Vaccarino","year":"2025","unstructured":"Vaccarino F, Quattrocchi CC, Parillo M (2025) Susceptibility-weighted imaging (SWI): technical aspects and applications in brain MRI for neurodegenerative disorders. Bioengineering. https:\/\/doi.org\/10.3390\/bioengineering12050473","journal-title":"Bioengineering"},{"key":"13744_CR13","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2019.116525","volume":"407","author":"A Sugiyama","year":"2019","unstructured":"Sugiyama A, Sato N, Kimura Y, Fujii H, Maikusa N, Shigemoto Y et al (2019) Quantifying iron deposition in the cerebellar subtype of Multiple System Atrophy and Spinocerebellar Ataxia Type 6 by quantitative susceptibility mapping. J Neurol Sci 407:116525. https:\/\/doi.org\/10.1016\/j.jns.2019.116525","journal-title":"J Neurol Sci"},{"key":"13744_CR14","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1002\/mds.27606","volume":"34","author":"PGD Ward","year":"2019","unstructured":"Ward PGD, Harding IH, Close TG, Corben LA, Delatycki MB, Storey E et al (2019) Longitudinal evaluation of iron concentration and atrophy in the dentate nuclei in Friedreich ataxia. mov disord 34:335\u2013343. https:\/\/doi.org\/10.1002\/mds.27606","journal-title":"mov disord"},{"key":"13744_CR15","doi-asserted-by":"publisher","DOI":"10.3389\/fnins.2022.919765","volume":"16","author":"CL Marvel","year":"2022","unstructured":"Marvel CL, Chen L, Joyce MR, Morgan OP, Iannuzzelli KG, LaConte SM et al (2022) Quantitative susceptibility mapping of basal ganglia iron is associated with cognitive and motor functions that distinguish spinocerebellar ataxia type 6 and type 3. front neurosci 16:919765. https:\/\/doi.org\/10.3389\/fnins.2022.919765","journal-title":"front neurosci"},{"key":"13744_CR16","doi-asserted-by":"publisher","first-page":"649","DOI":"10.1002\/jmri.21230","volume":"27","author":"H Bagher-Ebadian","year":"2008","unstructured":"Bagher-Ebadian H, Jiang Q, Ewing JR (2008) A modified Fourier-based phase unwrapping algorithm with an application to MRI venography. j magn reson imaging 27:649\u2013652. https:\/\/doi.org\/10.1002\/jmri.21230","journal-title":"j magn reson imaging"},{"key":"13744_CR17","doi-asserted-by":"publisher","DOI":"10.1002\/nbm.3550","author":"PS \u00d6zbay","year":"2017","unstructured":"\u00d6zbay PS, Deistung A, Feng X, Nanz D, Reichenbach JR, Schweser F (2017) A comprehensive numerical analysis of background phase correction with V-SHARP. NMR Biomed. https:\/\/doi.org\/10.1002\/nbm.3550","journal-title":"NMR Biomed"},{"key":"13744_CR18","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1016\/j.neuroimage.2011.07.019","volume":"59","author":"B Wu","year":"2012","unstructured":"Wu B, Li W, Avram AV, Gho SM, Liu C (2012) Fast and tissue-optimized mapping of magnetic susceptibility and T2* with multi-echo and multi-shot spirals. Neuroimage 59:297\u2013305. https:\/\/doi.org\/10.1016\/j.neuroimage.2011.07.019","journal-title":"Neuroimage"},{"key":"13744_CR19","doi-asserted-by":"publisher","first-page":"111","DOI":"10.1016\/j.neuroimage.2014.12.043","volume":"108","author":"W Li","year":"2015","unstructured":"Li W, Wang N, Yu F, Han H, Cao W, Romero R et al (2015) A method for estimating and removing streaking artifacts in quantitative susceptibility mapping. Neuroimage 108:111\u2013122. https:\/\/doi.org\/10.1016\/j.neuroimage.2014.12.043","journal-title":"Neuroimage"},{"key":"13744_CR20","doi-asserted-by":"publisher","first-page":"2795","DOI":"10.1002\/mrm.26946","volume":"79","author":"Z Liu","year":"2018","unstructured":"Liu Z, Spincemaille P, Yao Y, Zhang Y, Wang Y (2018) MEDI+0: Morphology enabled dipole inversion with automatic uniform cerebrospinal fluid zero reference for quantitative susceptibility mapping. magn reson med 79:2795\u20132803. https:\/\/doi.org\/10.1002\/mrm.26946","journal-title":"magn reson med"},{"key":"13744_CR21","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1016\/j.neuroimage.2007.07.007","volume":"38","author":"J Ashburner","year":"2007","unstructured":"Ashburner J (2007) A fast diffeomorphic image registration algorithm. Neuroimage 38:95\u2013113. https:\/\/doi.org\/10.1016\/j.neuroimage.2007.07.007","journal-title":"Neuroimage"},{"key":"13744_CR22","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2023.119950","volume":"270","author":"D J\u00e4schke","year":"2023","unstructured":"J\u00e4schke D, Steiner KM, Chang DI, Claa\u00dfen J, Uslar E, Thieme A et al (2023) Age-related differences of cerebellar cortex and nuclei: MRI findings in healthy controls and its application to spinocerebellar ataxia (SCA6) patients. Neuroimage 270:119950. https:\/\/doi.org\/10.1016\/j.neuroimage.2023.119950","journal-title":"Neuroimage"},{"key":"13744_CR23","doi-asserted-by":"publisher","first-page":"2492","DOI":"10.1046\/j.1471-4159.1998.70062492.x","volume":"70","author":"KL Double","year":"1998","unstructured":"Double KL, Maywald M, Schmittel M, Riederer P, Gerlach M (1998) In vitro studies of ferritin iron release and neurotoxicity. j neurochem 70:2492\u20132499. https:\/\/doi.org\/10.1046\/j.1471-4159.1998.70062492.x","journal-title":"j neurochem"},{"key":"13744_CR24","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1097\/00005072-199507000-00001","volume":"54","author":"BB Gelman","year":"1995","unstructured":"Gelman BB (1995) Iron in CNS disease. j neuropathol exp neurol 54:477\u2013486. https:\/\/doi.org\/10.1097\/00005072-199507000-00001","journal-title":"j neuropathol exp neurol"},{"key":"13744_CR25","doi-asserted-by":"publisher","DOI":"10.1016\/j.fsisyn.2025.100627","volume":"11","author":"A Sonoda","year":"2025","unstructured":"Sonoda A, Nihei M, Shinkawa N, Kakizaki E, Yukawa N (2025) Perls\u2019 Prussian blue staining and chemistry of Prussian blue and Turnbull blue. Forensic Sci Int Synerg 11:100627. https:\/\/doi.org\/10.1016\/j.fsisyn.2025.100627","journal-title":"Forensic Sci Int Synerg"},{"key":"13744_CR26","doi-asserted-by":"publisher","DOI":"10.1016\/j.nicl.2019.102103","volume":"25","author":"Z Cheng","year":"2020","unstructured":"Cheng Z, He N, Huang P, Li Y, Tang R, Sethi SK et al (2020) Imaging the Nigrosome 1 in the Substantia nigra using susceptibility weighted imaging and quantitative susceptibility mapping: An application to Parkinson\u2019s disease. Neuroimage Clin 25:102103. https:\/\/doi.org\/10.1016\/j.nicl.2019.102103","journal-title":"Neuroimage Clin"},{"key":"13744_CR27","doi-asserted-by":"publisher","first-page":"2282","DOI":"10.1002\/mds.29613","volume":"38","author":"R Satoh","year":"2023","unstructured":"Satoh R, Weigand SD, Pham NTT, Ali F, Arani A, Senjem ML et al (2023) Magnetic susceptibility in Progressive Supranuclear Palsy variants, Parkinson\u2019s Disease, and Corticobasal Syndrome. Mov Disord 38:2282\u20132290. https:\/\/doi.org\/10.1002\/mds.29613","journal-title":"Mov Disord"},{"key":"13744_CR28","doi-asserted-by":"publisher","DOI":"10.1002\/mdc3.70153","author":"S Haji","year":"2025","unstructured":"Haji S, Sako W, Hanada K, Matsubara T, Osaki Y, Matsumoto Y et al (2025) Magnetic susceptibility and area in the Subthalamic Nucleus measured by QSM for diagnosis of Progressive Supranuclear Palsy. Mov Disord Clin Pract. https:\/\/doi.org\/10.1002\/mdc3.70153","journal-title":"Mov Disord Clin Pract"},{"key":"13744_CR29","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1016\/j.ejrad.2018.11.035","volume":"110","author":"S Ronsin","year":"2019","unstructured":"Ronsin S, Hannoun S, Thobois S, Petiot P, Vighetto A, Cotton F et al (2019) A new MRI marker of ataxia with oculomotor apraxia. Eur J Radiol 110:187\u2013192. https:\/\/doi.org\/10.1016\/j.ejrad.2018.11.035","journal-title":"Eur J Radiol"},{"key":"13744_CR30","doi-asserted-by":"publisher","first-page":"542","DOI":"10.1016\/j.nicl.2013.03.018","volume":"2","author":"S Frismand","year":"2013","unstructured":"Frismand S, Salem H, Panouilleres M, P\u00e9lisson D, Jacobs S, Vighetto A et al (2013) MRI findings in AOA2: Cerebellar atrophy and abnormal iron detection in Dentate Nucleus. Neuroimage Clin 2:542\u2013548. https:\/\/doi.org\/10.1016\/j.nicl.2013.03.018","journal-title":"Neuroimage Clin"},{"key":"13744_CR31","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1186\/s12868-019-0507-7","volume":"20","author":"A Reinert","year":"2019","unstructured":"Reinert A, Morawski M, Seeger J, Arendt T, Reinert T (2019) Iron concentrations in neurons and glial cells with estimates on ferritin concentrations. BMC Neurosci 20:25. https:\/\/doi.org\/10.1186\/s12868-019-0507-7","journal-title":"BMC Neurosci"}],"container-title":["Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-026-13744-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00415-026-13744-x","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-026-13744-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T01:48:41Z","timestamp":1776649721000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00415-026-13744-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,17]]},"references-count":31,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,4]]}},"alternative-id":["13744"],"URL":"https:\/\/doi.org\/10.1007\/s00415-026-13744-x","relation":{},"ISSN":["0340-5354","1432-1459"],"issn-type":[{"value":"0340-5354","type":"print"},{"value":"1432-1459","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,17]]},"assertion":[{"value":"23 October 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"12 February 2026","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 February 2026","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 March 2026","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no conflicts of interest directly relevant to the content of this article.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"This retrospective MR study was approved by the Ethics Committee of the National Center of Neurology and Psychiatry Hospital, Tokyo, Japan, with the requirement for informed consent waived. The Ethics Committee approval number is A2025-048. MRI data from the control group were also used, and secondary-use consent was obtained under Ethics Committee approval number A2023-045. As this was a prospected study, all participants provided written informed consent prior to participation.Autopsied brain tissue registration with the Brain Bank for Aging Research (BBAR) at the Tokyo Metropolitan Institute for Geriatrics and Gerontology was performed after informed consent was obtained from the next of kin. Its Brain Bank Ethics Committee approval number is 2005\u201316.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}],"article-number":"213"}}