000155578 001__ 155578
000155578 005__ 20230915092330.0
000155578 0247_ $$2pmc$$apmc:PMC9521507
000155578 0247_ $$2doi$$a10.1002/mds.28610
000155578 0247_ $$2pmid$$apmid:33951232
000155578 0247_ $$2ISSN$$a0885-3185
000155578 0247_ $$2ISSN$$a1531-8257
000155578 0247_ $$2altmetric$$aaltmetric:105366313
000155578 037__ $$aDZNE-2021-00756
000155578 041__ $$aEnglish
000155578 082__ $$a610
000155578 1001_ $$0P:(DE-2719)2811327$$aFaber, Jennifer$$b0$$eFirst author
000155578 245__ $$aRegional Brain and Spinal Cord Volume Loss in Spinocerebellar Ataxia Type 3.
000155578 260__ $$aNew York, NY$$bWiley$$c2021
000155578 3367_ $$2DRIVER$$aarticle
000155578 3367_ $$2DataCite$$aOutput Types/Journal article
000155578 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1681215355_16322
000155578 3367_ $$2BibTeX$$aARTICLE
000155578 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000155578 3367_ $$00$$2EndNote$$aJournal Article
000155578 520__ $$aGiven that new therapeutic options for spinocerebellar ataxias are on the horizon, there is a need for markers that reflect disease-related alterations, in particular, in the preataxic stage, in which clinical scales are lacking sensitivity.The objective of this study was to quantify regional brain volumes and upper cervical spinal cord areas in spinocerebellar ataxia type 3 in vivo across the entire time course of the disease.We applied a brain segmentation approach that included a lobular subsegmentation of the cerebellum to magnetic resonance images of 210 ataxic and 48 preataxic spinocerebellar ataxia type 3 mutation carriers and 63 healthy controls. In addition, cervical cord cross-sectional areas were determined at 2 levels.The metrics of cervical spinal cord segments C3 and C2, medulla oblongata, pons, and pallidum, and the cerebellar anterior lobe were reduced in preataxic mutation carriers compared with controls. Those of cervical spinal cord segments C2 and C3, medulla oblongata, pons, midbrain, cerebellar lobules crus II and X, cerebellar white matter, and pallidum were reduced in ataxic compared with nonataxic carriers. Of all metrics studied, pontine volume showed the steepest decline across the disease course. It covaried with ataxia severity, CAG repeat length, and age. The multivariate model derived from this analysis explained 46.33% of the variance of pontine volume.Regional brain and spinal cord tissue loss in spinocerebellar ataxia type 3 starts before ataxia onset. Pontine volume appears to be the most promising imaging biomarker candidate for interventional trials that aim at slowing the progression of spinocerebellar ataxia type 3. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
000155578 536__ $$0G:(DE-HGF)POF4-353$$a353 - Clinical and Health Care Research (POF4-353)$$cPOF4-353$$fPOF IV$$x0
000155578 588__ $$aDataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
000155578 650_7 $$2Other$$aMRI
000155578 650_7 $$2Other$$abiomarker
000155578 650_7 $$2Other$$aspinocerebellar ataxia
000155578 650_7 $$2Other$$avolumetry
000155578 650_2 $$2MeSH$$aBrain: diagnostic imaging
000155578 650_2 $$2MeSH$$aCerebellum
000155578 650_2 $$2MeSH$$aHumans
000155578 650_2 $$2MeSH$$aMachado-Joseph Disease
000155578 650_2 $$2MeSH$$aSpinocerebellar Ataxias: diagnostic imaging
000155578 650_2 $$2MeSH$$aSpinocerebellar Ataxias: genetics
000155578 693__ $$0EXP:(DE-2719)ESMI-20140101$$5EXP:(DE-2719)ESMI-20140101$$eEuropean Spinocerebellar Ataxia Type 3/Machado-Joseph Disease Initiative$$x0
000155578 7001_ $$0P:(DE-2719)2812594$$aSchaprian, Tamara$$b1
000155578 7001_ $$0P:(DE-2719)9001550$$aBerkan, Koyak$$b2$$udzne
000155578 7001_ $$00000-0002-9730-9228$$aReetz, Kathrin$$b3
000155578 7001_ $$aFrança, Marcondes Cavalcante$$b4
000155578 7001_ $$ade Rezende, Thiago Junqueira Ribeiro$$b5
000155578 7001_ $$00000-0003-2812-4120$$aHong, Jiang$$b6
000155578 7001_ $$aLiao, Weihua$$b7
000155578 7001_ $$avan de Warrenburg, Bart$$b8
000155578 7001_ $$avan Gaalen, Judith$$b9
000155578 7001_ $$0P:(DE-2719)9000429$$aDürr, Alexandra$$b10
000155578 7001_ $$aMochel, Fanny$$b11
000155578 7001_ $$aGiunti, Paola$$b12
000155578 7001_ $$aGarcia-Moreno, Hector$$b13
000155578 7001_ $$aSchoels, Ludger$$b14
000155578 7001_ $$0P:(DE-2719)2811940$$aHengel, Holger$$b15
000155578 7001_ $$0P:(DE-2719)2811275$$aSynofzik, Matthis$$b16
000155578 7001_ $$0P:(DE-2719)9001506$$aBender, Benjamin$$b17
000155578 7001_ $$aOz, Gulin$$b18
000155578 7001_ $$aJoers, James$$b19
000155578 7001_ $$ade Vries, Jereon J$$b20
000155578 7001_ $$aKang, Jun-Suk$$b21
000155578 7001_ $$aTimmann-Braun, Dagmar$$b22
000155578 7001_ $$0P:(DE-2719)2811564$$aJacobi, Heike$$b23
000155578 7001_ $$aInfante, Jon$$b24
000155578 7001_ $$aJoules, Richard$$b25
000155578 7001_ $$aRomanzetti, Sandro$$b26
000155578 7001_ $$aDiedrichsen, Jorn$$b27
000155578 7001_ $$0P:(DE-2719)2811245$$aSchmid, Matthias$$b28
000155578 7001_ $$aWolz, Robin$$b29
000155578 7001_ $$0P:(DE-2719)2810314$$aKlockgether, Thomas$$b30$$eLast author
000155578 773__ $$0PERI:(DE-600)2041249-6$$a10.1002/mds.28610$$gp. mds.28610$$n10$$p2273-2281$$tMovement disorders$$v36$$x1531-8257$$y2021
000155578 8564_ $$uhttps://movementdisorders.onlinelibrary.wiley.com/doi/10.1002/mds.28610
000155578 8564_ $$uhttps://pub.dzne.de/record/155578/files/19594.pdf$$yOpenAccess
000155578 8564_ $$uhttps://pub.dzne.de/record/155578/files/19594.pdf?subformat=pdfa$$xpdfa$$yOpenAccess
000155578 909CO $$ooai:pub.dzne.de:155578$$pdnbdelivery$$pdriver$$pVDB$$popen_access$$popenaire
000155578 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2811327$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b0$$kDZNE
000155578 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2812594$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b1$$kDZNE
000155578 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)9001550$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b2$$kDZNE
000155578 9101_ $$0I:(DE-HGF)0$$6P:(DE-2719)9000429$$aExternal Institute$$b10$$kExtern
000155578 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2811940$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b15$$kDZNE
000155578 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2811275$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b16$$kDZNE
000155578 9101_ $$0I:(DE-HGF)0$$6P:(DE-2719)9001506$$aExternal Institute$$b17$$kExtern
000155578 9101_ $$0I:(DE-HGF)0$$6P:(DE-2719)2811564$$aExternal Institute$$b23$$kExtern
000155578 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2811245$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b28$$kDZNE
000155578 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2810314$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b30$$kDZNE
000155578 9131_ $$0G:(DE-HGF)POF4-353$$1G:(DE-HGF)POF4-350$$2G:(DE-HGF)POF4-300$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lNeurodegenerative Diseases$$vClinical and Health Care Research$$x0
000155578 9130_ $$0G:(DE-HGF)POF3-345$$1G:(DE-HGF)POF3-340$$2G:(DE-HGF)POF3-300$$3G:(DE-HGF)POF3$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lErkrankungen des Nervensystems$$vPopulation Studies and Genetics$$x0
000155578 9130_ $$0G:(DE-HGF)POF3-344$$1G:(DE-HGF)POF3-340$$2G:(DE-HGF)POF3-300$$3G:(DE-HGF)POF3$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lErkrankungen des Nervensystems$$vClinical and Health Care Research$$x1
000155578 9141_ $$y2021
000155578 915__ $$0LIC:(DE-HGF)CCBYNC4$$2HGFVOC$$aCreative Commons Attribution-NonCommercial CC BY-NC 4.0
000155578 915__ $$0StatID:(DE-HGF)3001$$2StatID$$aDEAL Wiley$$d2021-01-30$$wger
000155578 915__ $$0StatID:(DE-HGF)0113$$2StatID$$aWoS$$bScience Citation Index Expanded$$d2021-01-30
000155578 915__ $$0StatID:(DE-HGF)0510$$2StatID$$aOpenAccess
000155578 915__ $$0StatID:(DE-HGF)0160$$2StatID$$aDBCoverage$$bEssential Science Indicators$$d2021-01-30
000155578 915__ $$0StatID:(DE-HGF)0420$$2StatID$$aNationallizenz$$d2022-11-11$$wger
000155578 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bMOVEMENT DISORD : 2021$$d2022-11-11
000155578 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS$$d2022-11-11
000155578 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline$$d2022-11-11
000155578 915__ $$0StatID:(DE-HGF)0600$$2StatID$$aDBCoverage$$bEbsco Academic Search$$d2022-11-11
000155578 915__ $$0StatID:(DE-HGF)0030$$2StatID$$aPeer Review$$bASC$$d2022-11-11
000155578 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List$$d2022-11-11
000155578 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection$$d2022-11-11
000155578 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine$$d2022-11-11
000155578 915__ $$0StatID:(DE-HGF)1030$$2StatID$$aDBCoverage$$bCurrent Contents - Life Sciences$$d2022-11-11
000155578 915__ $$0StatID:(DE-HGF)9905$$2StatID$$aIF >= 5$$bMOVEMENT DISORD : 2021$$d2022-11-11
000155578 9201_ $$0I:(DE-2719)1011001$$kAG Klockgether$$lClinical Research Coordination$$x0
000155578 9201_ $$0I:(DE-2719)1210000$$kAG Gasser 1$$lParkinson Genetics$$x1
000155578 980__ $$ajournal
000155578 980__ $$aVDB
000155578 980__ $$aUNRESTRICTED
000155578 980__ $$aI:(DE-2719)1011001
000155578 980__ $$aI:(DE-2719)1210000
000155578 9801_ $$aFullTexts