{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T12:09:24Z","timestamp":1774440564664,"version":"3.50.1"},"reference-count":30,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T00:00:00Z","timestamp":1771200000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T00:00:00Z","timestamp":1771200000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Radiologie"],"published-print":{"date-parts":[[2026,4]]},"DOI":"10.1007\/s00117-026-01561-2","type":"journal-article","created":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T12:43:53Z","timestamp":1771245833000},"page":"258-263","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Trinukleotid-Erkrankungen","Trinucleotide repeat disorders"],"prefix":"10.1007","volume":"66","author":[{"given":"Armin","family":"Bachhuber","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,2,16]]},"reference":[{"issue":"3","key":"1561_CR1","doi-asserted-by":"publisher","first-page":"475","DOI":"10.1016\/j.nbd.2011.02.010","volume":"42","author":"I Bohanna","year":"2011","unstructured":"Bohanna\u00a0I, Georgiou-Karistianis\u00a0N, Egan\u00a0GF (2011) Connectivity-based segmentation of the striatum in Huntington\u2019s disease: vulnerability of motor pathways. Neurobiol Dis 42(3):475\u2013481. https:\/\/doi.org\/10.1016\/j.nbd.2011.02.010","journal-title":"Neurobiol Dis"},{"issue":"1","key":"1561_CR2","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1016\/S1474-4422(10)70276-3","volume":"10","author":"SJ Tabrizi","year":"2011","unstructured":"Tabrizi\u00a0SJ, Scahill\u00a0RI, Durr\u00a0A, Roos\u00a0RA, Leavitt\u00a0BR, Jones\u00a0R et\u00a0al (2011) Biological and clinical changes in premanifest and early stage Huntington\u2019s disease in the TRACK-HD study: the 12-month longitudinal analysis. Lancet Neurol 10(1):31\u201342. https:\/\/doi.org\/10.1016\/S1474-4422(10)70276-3","journal-title":"Lancet Neurol"},{"issue":"10","key":"1561_CR3","doi-asserted-by":"publisher","first-page":"613","DOI":"10.1038\/nrn.2017.92","volume":"18","author":"HL Paulson","year":"2017","unstructured":"Paulson\u00a0HL, Shakkottai\u00a0VG, Clark\u00a0HB, Orr\u00a0HT (2017) Polyglutamine spinocerebellar ataxias\u2014from genes to potential treatments. Nat Rev Neurosci 18(10):613\u2013626. https:\/\/doi.org\/10.1038\/nrn.2017.92","journal-title":"Nat Rev Neurosci"},{"key":"1561_CR4","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1016\/B978-0-444-52014-2.00004-5","volume":"100","author":"JP Vonsattel","year":"2011","unstructured":"Vonsattel\u00a0JP, Keller\u00a0C, Cortes Ramirez\u00a0EP (2011) Huntington\u2019s disease\u2014neuropathology. Handb Clin Neurol 100:83\u2013100. https:\/\/doi.org\/10.1016\/B978-0-444-52014-2.00004-5","journal-title":"Handb Clin Neurol"},{"issue":"5","key":"1561_CR5","doi-asserted-by":"publisher","first-page":"789","DOI":"10.3174\/ajnr.A4617","volume":"37","author":"JM van Bergen","year":"2016","unstructured":"van Bergen\u00a0JM, Hua\u00a0J, Unschuld\u00a0PG, Lim\u00a0IA, Jones\u00a0CK, Margolis\u00a0RL et\u00a0al (2016) Quantitative Susceptibility Mapping Suggests Altered Brain Iron in Premanifest Huntington Disease. AJNR Am J Neuroradiol 37(5):789\u2013796. https:\/\/doi.org\/10.3174\/ajnr.A4617","journal-title":"AJNR Am J Neuroradiol"},{"issue":"2","key":"1561_CR6","doi-asserted-by":"publisher","first-page":"160","DOI":"10.1002\/ana.24406","volume":"78","author":"J Drouin-Ouellet","year":"2015","unstructured":"Drouin-Ouellet\u00a0J, Sawiak\u00a0SJ, Cisbani\u00a0G, Lagace\u00a0M, Kuan\u00a0WL, Saint-Pierre\u00a0M et\u00a0al (2015) Cerebrovascular and blood-brain barrier impairments in Huntington\u2019s disease: Potential implications for its pathophysiology. Ann Neurol 78(2):160\u2013177. https:\/\/doi.org\/10.1002\/ana.24406","journal-title":"Ann Neurol"},{"issue":"1","key":"1561_CR7","doi-asserted-by":"publisher","first-page":"40","DOI":"10.1002\/mrm.22775","volume":"66","author":"J Hua","year":"2011","unstructured":"Hua\u00a0J, Qin\u00a0Q, Donahue\u00a0MJ, Zhou\u00a0J, Pekar\u00a0JJ, van Zijl\u00a0PC (2011) Inflow-based vascular-space-occupancy (iVASO) MRI. Magn Reson Med 66(1):40\u201356. https:\/\/doi.org\/10.1002\/mrm.22775","journal-title":"Magn Reson Med"},{"issue":"7","key":"1561_CR8","doi-asserted-by":"publisher","first-page":"1206","DOI":"10.1162\/jocn.2007.19.7.1206","volume":"19","author":"CM Kipps","year":"2007","unstructured":"Kipps\u00a0CM, Duggins\u00a0AJ, McCusker\u00a0EA, Calder\u00a0AJ (2007) Disgust and happiness recognition correlate with anteroventral insula and amygdala volume respectively in preclinical Huntington\u2019s disease. J\u00a0Cogn Neurosci 19(7):1206\u20131217. https:\/\/doi.org\/10.1162\/jocn.2007.19.7.1206","journal-title":"J Cogn Neurosci"},{"issue":"1","key":"1561_CR9","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1002\/ana.410310113","volume":"31","author":"GJ Harris","year":"1992","unstructured":"Harris\u00a0GJ, Pearlson\u00a0GD, Peyser\u00a0CE, Aylward\u00a0EH, Roberts\u00a0J, Barta\u00a0PE et\u00a0al (1992) Putamen volume reduction on magnetic resonance imaging exceeds caudate changes in mild Huntington\u2019s disease. Ann Neurol 31(1):69\u201375. https:\/\/doi.org\/10.1002\/ana.410310113","journal-title":"Ann Neurol"},{"issue":"1","key":"1561_CR10","doi-asserted-by":"publisher","first-page":"68","DOI":"10.1016\/0006-3223(91)90211-4","volume":"29","author":"TL Jernigan","year":"1991","unstructured":"Jernigan\u00a0TL, Salmon\u00a0DP, Butters\u00a0N, Hesselink\u00a0JR (1991) Cerebral structure on MRI, Part II: Specific changes in Alzheimer\u2019s and Huntington\u2019s diseases. Biol Psychiatry 29(1):68\u201381. https:\/\/doi.org\/10.1016\/0006-3223(91)90211-4","journal-title":"Biol Psychiatry"},{"issue":"2","key":"1561_CR11","doi-asserted-by":"publisher","first-page":"317","DOI":"10.3174\/ajnr.A3303","volume":"34","author":"SJ van den Bogaard","year":"2013","unstructured":"van den Bogaard\u00a0SJ, Dumas\u00a0EM, Hart\u00a0EP, Milles\u00a0J, Reilmann\u00a0R, Stout\u00a0JC et\u00a0al (2013) Magnetization transfer imaging in premanifest and manifest huntington disease: a 2\u2011year follow-up. AJNR Am J Neuroradiol 34(2):317\u2013322. https:\/\/doi.org\/10.3174\/ajnr.A3303","journal-title":"AJNR Am J Neuroradiol"},{"issue":"6","key":"1561_CR12","doi-asserted-by":"publisher","first-page":"2582","DOI":"10.1002\/hbm.22351","volume":"35","author":"CJ Werner","year":"2014","unstructured":"Werner\u00a0CJ, Dogan\u00a0I, Sass\u00a0C, Mirzazade\u00a0S, Schiefer\u00a0J, Shah\u00a0NJ et\u00a0al (2014) Altered resting-state connectivity in Huntington\u2019s disease. Hum Brain Mapp 35(6):2582\u20132593. https:\/\/doi.org\/10.1002\/hbm.22351","journal-title":"Hum Brain Mapp"},{"issue":"12","key":"1561_CR13","doi-asserted-by":"publisher","first-page":"2230","DOI":"10.1007\/s00415-011-6099-5","volume":"258","author":"SJ van den Bogaard","year":"2011","unstructured":"van den Bogaard\u00a0SJ, Dumas\u00a0EM, Teeuwisse\u00a0WM, Kan\u00a0HE, Webb\u00a0A, Roos\u00a0RA, van der Grond\u00a0J (2011) Exploratory 7\u2011Tesla magnetic resonance spectroscopy in Huntington\u2019s disease provides in vivo evidence for impaired energy metabolism. J\u00a0Neurol 258(12):2230\u20132239. https:\/\/doi.org\/10.1007\/s00415-011-6099-5","journal-title":"J Neurol"},{"key":"1561_CR14","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2024.106574","volume":"199","author":"Y Jing","year":"2024","unstructured":"Jing\u00a0Y, Dogan\u00a0I, Reetz\u00a0K, Romanzetti\u00a0S (2024) Neurochemical changes in the progression of Huntington\u2019s disease: A meta-analysis of in vivo(1)H-MRS studies. Neurobiol Dis 199:106574. https:\/\/doi.org\/10.1016\/j.nbd.2024.106574","journal-title":"Neurobiol Dis"},{"issue":"6","key":"1561_CR15","doi-asserted-by":"publisher","DOI":"10.1093\/braincomms\/fcac258","volume":"4","author":"AJ Lowe","year":"2022","unstructured":"Lowe\u00a0AJ, Rodrigues\u00a0FB, Arridge\u00a0M, De Vita\u00a0E, Johnson\u00a0EB, Scahill\u00a0RI et\u00a0al (2022) Longitudinal evaluation of proton magnetic resonance spectroscopy metabolites as biomarkers in Huntington\u2019s disease. Brain Commun 4(6):fcac258. https:\/\/doi.org\/10.1093\/braincomms\/fcac258","journal-title":"Brain Commun"},{"issue":"13","key":"1561_CR16","doi-asserted-by":"publisher","first-page":"1931","DOI":"10.1002\/mds.22245","volume":"23","author":"NC Reynolds","year":"2008","unstructured":"Reynolds\u00a0NC, Prost\u00a0RW, Mark\u00a0LP, Joseph\u00a0SA (2008) MR-spectroscopic findings in juvenile-onset Huntington\u2019s disease. Mov Disord Clin Pract 23(13):1931\u20131935. https:\/\/doi.org\/10.1002\/mds.22245","journal-title":"Mov Disord"},{"issue":"4","key":"1561_CR17","doi-asserted-by":"publisher","first-page":"377","DOI":"10.3233\/JHD-140117","volume":"3","author":"SJ van den Bogaard","year":"2014","unstructured":"van den Bogaard\u00a0SJ, Dumas\u00a0EM, Teeuwisse\u00a0WM, Kan\u00a0HE, Webb\u00a0A, van Buchem\u00a0MA et\u00a0al (2014) Longitudinal metabolite changes in Huntington\u2019s disease during disease onset. J\u00a0Huntingtons Dis 3(4):377\u2013386. https:\/\/doi.org\/10.3233\/JHD-140117","journal-title":"J Huntingtons Dis"},{"issue":"19","key":"1561_CR18","doi-asserted-by":"publisher","first-page":"1702","DOI":"10.1212\/WNL.0b013e3181fc27e4","volume":"75","author":"A Sturrock","year":"2010","unstructured":"Sturrock\u00a0A, Laule\u00a0C, Decolongon\u00a0J, Dar Santos\u00a0R, Coleman\u00a0AJ, Creighton\u00a0S et\u00a0al (2010) Magnetic resonance spectroscopy biomarkers in premanifest and early Huntington disease. Neurology 75(19):1702\u20131710. https:\/\/doi.org\/10.1212\/WNL.0b013e3181fc27e4","journal-title":"Neurology"},{"issue":"3","key":"1561_CR19","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1002\/mds.26118","volume":"30","author":"A Sturrock","year":"2015","unstructured":"Sturrock\u00a0A, Laule\u00a0C, Wyper\u00a0K, Milner\u00a0RA, Decolongon\u00a0J, Dar Santos\u00a0R et\u00a0al (2015) A longitudinal study of magnetic resonance spectroscopy Huntington\u2019s disease biomarkers. Mov Disord Clin Pract 30(3):393\u2013401. https:\/\/doi.org\/10.1002\/mds.26118","journal-title":"Mov Disord"},{"issue":"9","key":"1561_CR20","doi-asserted-by":"publisher","first-page":"1133","DOI":"10.1177\/0883073812448230","volume":"27","author":"MB Delatycki","year":"2012","unstructured":"Delatycki\u00a0MB, Corben\u00a0LA (2012) Clinical features of Friedreich ataxia. J\u00a0Child Neurol 27(9):1133\u20131137. https:\/\/doi.org\/10.1177\/0883073812448230","journal-title":"J Child Neurol"},{"key":"1561_CR21","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1016\/j.nicl.2014.08.018","volume":"6","author":"K Solbach","year":"2014","unstructured":"Solbach\u00a0K, Kraff\u00a0O, Minnerop\u00a0M, Beck\u00a0A, Schols\u00a0L, Gizewski\u00a0ER et\u00a0al (2014) Cerebellar pathology in Friedreich\u2019s ataxia: atrophied dentate nuclei with normal iron content. Neuroimage Clin 6:93\u201399. https:\/\/doi.org\/10.1016\/j.nicl.2014.08.018","journal-title":"Neuroimage Clin"},{"key":"1561_CR22","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2017.00472","volume":"8","author":"LC Velazquez-Perez","year":"2017","unstructured":"Velazquez-Perez\u00a0LC, Rodriguez-Labrada\u00a0R, Fernandez-Ruiz\u00a0J (2017) Spinocerebellar Ataxia Type 2: Clinicogenetic Aspects, Mechanistic Insights, and Management Approaches. Front Neurol 8:472. https:\/\/doi.org\/10.3389\/fneur.2017.00472","journal-title":"Front Neurol"},{"issue":"5","key":"1561_CR23","doi-asserted-by":"publisher","first-page":"890","DOI":"10.3174\/ajnr.A2387","volume":"32","author":"L Eichler","year":"2011","unstructured":"Eichler\u00a0L, Bellenberg\u00a0B, Hahn\u00a0HK, Koster\u00a0O, Schols\u00a0L, Lukas\u00a0C (2011) Quantitative assessment of brain stem and cerebellar atrophy in spinocerebellar ataxia types 3 and 6: impact on clinical status. AJNR Am J Neuroradiol 32(5):890\u2013897. https:\/\/doi.org\/10.3174\/ajnr.A2387","journal-title":"AJNR Am J Neuroradiol"},{"issue":"1","key":"1561_CR24","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1080\/13816810802454081","volume":"30","author":"T Hugosson","year":"2009","unstructured":"Hugosson\u00a0T, Granse\u00a0L, Ponjavic\u00a0V, Andreasson\u00a0S (2009) Macular dysfunction and morphology in spinocerebellar ataxia type 7 (SCA 7). Ophthalmic Genet 30(1):1\u20136. https:\/\/doi.org\/10.1080\/13816810802454081","journal-title":"Ophthalmic Genet"},{"issue":"4","key":"1561_CR25","doi-asserted-by":"publisher","first-page":"513","DOI":"10.1111\/j.1468-1331.2008.02524.x","volume":"16","author":"YC Lee","year":"2009","unstructured":"Lee\u00a0YC, Liu\u00a0CS, Wu\u00a0HM, Wang\u00a0PS, Chang\u00a0MH, Soong\u00a0BW (2009) The \u2019hot cross bun\u2018 sign in the patients with spinocerebellar ataxia. Eur J Neurol 16(4):513\u2013516. https:\/\/doi.org\/10.1111\/j.1468-1331.2008.02524.x","journal-title":"Eur J Neurol"},{"issue":"11","key":"1561_CR26","doi-asserted-by":"publisher","first-page":"1521","DOI":"10.1002\/mds.20529","volume":"20","author":"CT Loy","year":"2005","unstructured":"Loy\u00a0CT, Sweeney\u00a0MG, Davis\u00a0MB, Wills\u00a0AJ, Sawle\u00a0GV, Lees\u00a0AJ, Tabrizi\u00a0SJ (2005) Spinocerebellar ataxia type 17: extension of phenotype with putaminal rim hyperintensity on magnetic resonance imaging. Mov Disord Clin Pract 20(11):1521\u20131523. https:\/\/doi.org\/10.1002\/mds.20529","journal-title":"Mov Disord"},{"key":"1561_CR27","unstructured":"Amnesty International (2025) Glossar f\u00fcr diskriminierungssensible Sprache. https:\/\/www.amnesty.de\/glossar-fuer-diskriminierungssensible-sprache"},{"issue":"2","key":"1561_CR28","doi-asserted-by":"publisher","first-page":"140","DOI":"10.1016\/0022-510x(95)00283-8","volume":"135","author":"D Guidetti","year":"1996","unstructured":"Guidetti\u00a0D, Vescovini\u00a0E, Motti\u00a0L, Ghidoni\u00a0E, Gemignani\u00a0F, Marbini\u00a0A et\u00a0al (1996) X\u2011linked bulbar and spinal muscular atrophy, or Kennedy disease: clinical, neurophysiological, neuropathological, neuropsychological and molecular study of a large family. J\u00a0Neurol Sci 135(2):140\u2013148. https:\/\/doi.org\/10.1016\/0022-510x(95)00283-8","journal-title":"J Neurol Sci"},{"issue":"11","key":"1561_CR29","doi-asserted-by":"publisher","first-page":"1209","DOI":"10.1136\/jnnp.2006.112532","volume":"78","author":"J Kassubek","year":"2007","unstructured":"Kassubek\u00a0J, Juengling\u00a0FD, Sperfeld\u00a0AD (2007) Widespread white matter changes in Kennedy disease: a voxel based morphometry study. BMJ Neurol Open 78(11):1209\u20131212. https:\/\/doi.org\/10.1136\/jnnp.2006.112532","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"11","key":"1561_CR30","doi-asserted-by":"publisher","first-page":"1727","DOI":"10.1002\/hbm.20971","volume":"31","author":"A Unrath","year":"2010","unstructured":"Unrath\u00a0A, Muller\u00a0HP, Riecker\u00a0A, Ludolph\u00a0AC, Sperfeld\u00a0AD, Kassubek\u00a0J (2010) Whole brain-based analysis of regional white matter tract alterations in rare motor neuron diseases by diffusion tensor imaging. Hum Brain Mapp 31(11):1727\u20131740. https:\/\/doi.org\/10.1002\/hbm.20971","journal-title":"Hum Brain Mapp"}],"container-title":["Die Radiologie"],"original-title":[],"language":"de","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00117-026-01561-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00117-026-01561-2","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00117-026-01561-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T08:57:47Z","timestamp":1774429067000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00117-026-01561-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,16]]},"references-count":30,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,4]]}},"alternative-id":["1561"],"URL":"https:\/\/doi.org\/10.1007\/s00117-026-01561-2","relation":{},"ISSN":["2731-7048","2731-7056"],"issn-type":[{"value":"2731-7048","type":"print"},{"value":"2731-7056","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,16]]},"assertion":[{"value":"27 October 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 January 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 February 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Einhaltung ethischer Richtlinien"}},{"value":"A.\u00a0Bachhuber gibt an, dass kein Interessenkonflikt besteht.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Interessenkonflikt"}},{"value":"F\u00fcr diesen Beitrag wurden von den Autor\/-innen keine Studien an Menschen oder Tieren durchgef\u00fchrt. F\u00fcr die aufgef\u00fchrten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Interessenkonflikt"}}]}}