000282565 001__ 282565
000282565 005__ 20251202144730.0
000282565 0247_ $$2doi$$a10.1038/s41582-025-01136-0
000282565 0247_ $$2pmid$$apmid:40983776
000282565 0247_ $$2ISSN$$a1759-4758
000282565 0247_ $$2ISSN$$a1745-834X
000282565 0247_ $$2ISSN$$a1745-8358
000282565 0247_ $$2ISSN$$a1759-4766
000282565 037__ $$aDZNE-2025-01328
000282565 041__ $$aEnglish
000282565 082__ $$a610
000282565 1001_ $$00000-0002-4427-5765$$aWirth, Thomas$$b0
000282565 245__ $$aProgress and challenges in sporadic late-onset cerebellar ataxias.
000282565 260__ $$aLondon$$bMacmillan Publishers Limited, part of Springer Nature$$c2025
000282565 3367_ $$2DRIVER$$aarticle
000282565 3367_ $$2DataCite$$aOutput Types/Journal article
000282565 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1764683139_28227$$xReview Article
000282565 3367_ $$2BibTeX$$aARTICLE
000282565 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000282565 3367_ $$00$$2EndNote$$aJournal Article
000282565 520__ $$aSporadic late-onset cerebellar ataxia (SLOCA) is a syndrome defined by subacute or chronic and progressive ataxia occurring after the age of 40 years in individuals without a family history of ataxia. The 2022 publication of revised consensus diagnostic criteria for multiple system atrophy and the emergence of promising biomarkers provides a thorough diagnostic framework that now enables the diagnosis of numerous acquired causes of SLOCA, including autoimmune disorders and neurodegenerative diseases. The ongoing development and increased availability of DNA sequencing technology have uncovered several molecular causes of SLOCA besides spastic paraplegia type 7 and very late-onset Friedreich ataxia. These additional causes include sporadic genetic disorders, such as spinocerebellar atrophy type 27B, caused by GAA expansion in the FGF14 gene, and cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS), caused by biallelic expansions in the RFC1 gene. This Review presents an updated clinical approach to the diagnosis and management of SLOCA that focuses on the most important developments in this field. Future challenges are also discussed, including the identification of additional missing genetic causes of SLOCA, especially via the use of long-read genome sequencing, improvements in SLOCA prognostication and the implementation of clinical trials of neuroprotective interventions.
000282565 536__ $$0G:(DE-HGF)POF4-353$$a353 - Clinical and Health Care Research (POF4-353)$$cPOF4-353$$fPOF IV$$x0
000282565 588__ $$aDataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
000282565 650_2 $$2MeSH$$aHumans
000282565 650_2 $$2MeSH$$aCerebellar Ataxia: diagnosis
000282565 650_2 $$2MeSH$$aCerebellar Ataxia: genetics
000282565 650_2 $$2MeSH$$aCerebellar Ataxia: therapy
000282565 650_2 $$2MeSH$$aAge of Onset
000282565 7001_ $$0P:(DE-2719)2811327$$aFaber, Jennifer$$b1
000282565 7001_ $$00000-0002-7212-9554$$aDepienne, Christel$$b2
000282565 7001_ $$aRoze, Emmanuel$$b3
000282565 7001_ $$00000-0002-4721-5952$$aHonnorat, Jérôme$$b4
000282565 7001_ $$00000-0003-2172-7527$$aMeissner, Wassilios G$$b5
000282565 7001_ $$aGiunti, Paola$$b6
000282565 7001_ $$aTranchant, Christine$$b7
000282565 7001_ $$0P:(DE-2719)2810314$$aKlockgether, Thomas$$b8
000282565 7001_ $$00000-0001-8121-0605$$aAnheim, Mathieu$$b9
000282565 773__ $$0PERI:(DE-600)2491518-X$$a10.1038/s41582-025-01136-0$$gVol. 21, no. 12, p. 687 - 705$$n12$$p687 - 705$$tNature reviews / Neurology$$v21$$x1759-4758$$y2025
000282565 8564_ $$uhttps://pub.dzne.de/record/282565/files/DZNE-2025-01328_Restricted.pdf
000282565 8564_ $$uhttps://pub.dzne.de/record/282565/files/DZNE-2025-01328_Restricted.pdf?subformat=pdfa$$xpdfa
000282565 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2811327$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b1$$kDZNE
000282565 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2810314$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b8$$kDZNE
000282565 9131_ $$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$$x0
000282565 915__ $$0StatID:(DE-HGF)0420$$2StatID$$aNationallizenz$$d2025-01-02$$wger
000282565 915__ $$0StatID:(DE-HGF)3003$$2StatID$$aDEAL Nature$$d2025-01-02$$wger
000282565 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS$$d2025-01-02
000282565 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline$$d2025-01-02
000282565 915__ $$0StatID:(DE-HGF)0600$$2StatID$$aDBCoverage$$bEbsco Academic Search$$d2025-01-02
000282565 915__ $$0StatID:(DE-HGF)0030$$2StatID$$aPeer Review$$bASC$$d2025-01-02
000282565 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List$$d2025-01-02
000282565 915__ $$0StatID:(DE-HGF)0160$$2StatID$$aDBCoverage$$bEssential Science Indicators$$d2025-01-02
000282565 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine$$d2025-01-02
000282565 915__ $$0StatID:(DE-HGF)0113$$2StatID$$aWoS$$bScience Citation Index Expanded$$d2025-01-02
000282565 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection$$d2025-01-02
000282565 9201_ $$0I:(DE-2719)1011001$$kClinical Research (Bonn)$$lClinical Research Coordination$$x0
000282565 9201_ $$0I:(DE-2719)1011101$$kPatient Studies (Bonn)$$lPatient Studies (Bonn)$$x1
000282565 980__ $$ajournal
000282565 980__ $$aEDITORS
000282565 980__ $$aVDBINPRINT
000282565 980__ $$aI:(DE-2719)1011001
000282565 980__ $$aI:(DE-2719)1011101
000282565 980__ $$aUNRESTRICTED