000282298 001__ 282298
000282298 005__ 20251117095728.0
000282298 0247_ $$2doi$$a10.1212/WNL.0000000000214316
000282298 0247_ $$2pmid$$apmid:41232058
000282298 0247_ $$2pmc$$apmc:PMC12615010
000282298 0247_ $$2ISSN$$a0028-3878
000282298 0247_ $$2ISSN$$a1526-632X
000282298 037__ $$aDZNE-2025-01268
000282298 041__ $$aEnglish
000282298 082__ $$a610
000282298 1001_ $$0P:(DE-2719)9003066$$aWilkens, Ida$$b0
000282298 245__ $$aMultiple System Atrophy Without Dysautonomia: An Autopsy-Confirmed Study.
000282298 260__ $$aPhiladelphia, Pa.$$bWolters Kluwer$$c2025
000282298 3367_ $$2DRIVER$$aarticle
000282298 3367_ $$2DataCite$$aOutput Types/Journal article
000282298 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1763369585_17166
000282298 3367_ $$2BibTeX$$aARTICLE
000282298 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000282298 3367_ $$00$$2EndNote$$aJournal Article
000282298 520__ $$aMultiple system atrophy (MSA) is a neurodegenerative disorder characterized by 3 core symptom complexes: parkinsonism, cerebellar syndrome, and dysautonomia. Recent Movement Disorder Society (MDS) criteria allow for the clinical diagnosis of MSA based solely on motor symptoms, without requiring dysautonomia. This study aimed to evaluate the frequency and disease trajectory of MSA patients without dysautonomia compared with those with autonomic involvement.A multicenter cohort of autopsy-confirmed patients with MSA was analyzed for demographic characteristics, symptom onset, and progression of parkinsonism, cerebellar syndrome, and dysautonomia. Clinical data were collected through standardized chart reviews across participating centers and categorized using the MDS-MSA criteria. Patients were grouped according to their initial symptom complex and tracked for the evolution of additional symptoms. Analyses included time to development of further symptom complexes, age at symptom onset, disease duration, and phenotype at the last recorded visit. Patients with motor symptoms only were matched to patients with similar demographics but with dysautonomia. Statistical methods included ANOVA, t tests, Welch t tests, and χ2 tests, with significance set at p < 0.05.Among 140 patients (mean age at onset 62.3 ± 8.9 years; 44% female), 81 (58%) initially presented without dysautonomia (57 with parkinsonism only, 17 with cerebellar syndrome only, 7 with both). At final follow-up, 12 patients (9%) had not developed dysautonomia. These patients showed significantly longer disease duration (mean 8.1 ± 2.1 years) than matched patients with dysautonomia (mean 6.3 ± 2.6 years; p = 0.035). Overall, 51% of patients developed all 3 symptom complexes. Patients with cerebellar onset progressed more rapidly to multisystem involvement than those with parkinsonian onset (mean interval to second symptom: 2.0 vs 3.4 years; p < 0.05).The MDS-MSA criteria expand the diagnostic scope by identifying a motor-only subgroup with a distinct and potentially slower disease course. These findings underscore the importance of including motor-only patients in natural history and interventional studies. Limitations include retrospective data collection and potential variability in symptom documentation.
000282298 536__ $$0G:(DE-HGF)POF4-353$$a353 - Clinical and Health Care Research (POF4-353)$$cPOF4-353$$fPOF IV$$x0
000282298 536__ $$0G:(DE-HGF)POF4-352$$a352 - Disease Mechanisms (POF4-352)$$cPOF4-352$$fPOF IV$$x1
000282298 588__ $$aDataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
000282298 650_2 $$2MeSH$$aHumans
000282298 650_2 $$2MeSH$$aMultiple System Atrophy: pathology
000282298 650_2 $$2MeSH$$aMultiple System Atrophy: diagnosis
000282298 650_2 $$2MeSH$$aMultiple System Atrophy: physiopathology
000282298 650_2 $$2MeSH$$aFemale
000282298 650_2 $$2MeSH$$aMale
000282298 650_2 $$2MeSH$$aMiddle Aged
000282298 650_2 $$2MeSH$$aAged
000282298 650_2 $$2MeSH$$aAutopsy
000282298 650_2 $$2MeSH$$aPrimary Dysautonomias
000282298 650_2 $$2MeSH$$aDisease Progression
000282298 650_2 $$2MeSH$$aCohort Studies
000282298 650_2 $$2MeSH$$aParkinsonian Disorders
000282298 7001_ $$aBebermeier, Sarah$$b1
000282298 7001_ $$aHeine, Johanne$$b2
000282298 7001_ $$aRuf, Viktoria Constanze$$b3
000282298 7001_ $$aCompta, Yaroslau$$b4
000282298 7001_ $$aMolina Porcel, Laura$$b5
000282298 7001_ $$aTroakes, Claire$$b6
000282298 7001_ $$aVamanu, Albert$$b7
000282298 7001_ $$aDownes, Sophia$$b8
000282298 7001_ $$00000-0002-5599-5098$$aIrwin, David John$$b9
000282298 7001_ $$00000-0003-2554-5181$$aCohen, Jesse$$b10
000282298 7001_ $$aLee, Edward B$$b11
000282298 7001_ $$aNilsson, Christer F$$b12
000282298 7001_ $$aEnglund, Elisabet M$$b13
000282298 7001_ $$0P:(DE-2719)9002938$$aNemati, Mojtaba$$b14$$udzne
000282298 7001_ $$0P:(DE-2719)9001160$$aKatzdobler, Sabrina$$b15$$udzne
000282298 7001_ $$0P:(DE-2719)2811659$$aLevin, Johannes$$b16
000282298 7001_ $$0P:(DE-2719)9002620$$aBernhardt, Alexander Maximilian$$b17$$udzne
000282298 7001_ $$00000-0002-6427-7485$$aPantelyat, Alexander$$b18
000282298 7001_ $$00000-0001-7742-8759$$aSeemiller, Joseph$$b19
000282298 7001_ $$aBerger, Stephen$$b20
000282298 7001_ $$aVan Swieten, John C$$b21
000282298 7001_ $$00000-0002-4737-6289$$aDopper, Elise G P$$b22
000282298 7001_ $$aRozemuller, Annemieke J M$$b23
000282298 7001_ $$00000-0003-3841-5511$$aKovacs, Gabor G$$b24
000282298 7001_ $$00000-0003-2167-5667$$aBendahan, Nathaniel$$b25
000282298 7001_ $$00000-0003-1229-3667$$aLang, Anthony E$$b26
000282298 7001_ $$0P:(DE-2719)2810441$$aHerms, Jochen$$b27$$udzne
000282298 7001_ $$0P:(DE-2719)2811373$$aHöglinger, Günter U$$b28
000282298 7001_ $$0P:(DE-2719)9003372$$aHopfner, Franziska$$b29$$udzne
000282298 773__ $$0PERI:(DE-600)1491874-2$$a10.1212/WNL.0000000000214316$$gVol. 105, no. 11, p. e214316$$n11$$pe214316$$tNeurology$$v105$$x0028-3878$$y2025
000282298 8564_ $$uhttps://pub.dzne.de/record/282298/files/DZNE-2025-01268_SUPP1.pdf
000282298 8564_ $$uhttps://pub.dzne.de/record/282298/files/DZNE-2025-01268_SUPP2.pdf
000282298 8564_ $$uhttps://pub.dzne.de/record/282298/files/DZNE-2025-01268.pdf$$yRestricted
000282298 8564_ $$uhttps://pub.dzne.de/record/282298/files/DZNE-2025-01268_SUPP1.pdf?subformat=pdfa$$xpdfa
000282298 8564_ $$uhttps://pub.dzne.de/record/282298/files/DZNE-2025-01268_SUPP2.pdf?subformat=pdfa$$xpdfa
000282298 8564_ $$uhttps://pub.dzne.de/record/282298/files/DZNE-2025-01268.pdf?subformat=pdfa$$xpdfa$$yRestricted
000282298 9101_ $$0I:(DE-HGF)0$$6P:(DE-2719)9003066$$aExternal Institute$$b0$$kExtern
000282298 9101_ $$0I:(DE-HGF)0$$6P:(DE-2719)9002938$$aExternal Institute$$b14$$kExtern
000282298 9101_ $$0I:(DE-HGF)0$$6P:(DE-2719)9001160$$aExternal Institute$$b15$$kExtern
000282298 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2811659$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b16$$kDZNE
000282298 9101_ $$0I:(DE-HGF)0$$6P:(DE-2719)9002620$$aExternal Institute$$b17$$kExtern
000282298 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2810441$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b27$$kDZNE
000282298 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2811373$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b28$$kDZNE
000282298 9101_ $$0I:(DE-HGF)0$$6P:(DE-2719)9003372$$aExternal Institute$$b29$$kExtern
000282298 9131_ $$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$$x0
000282298 9131_ $$0G:(DE-HGF)POF4-352$$1G:(DE-HGF)POF4-350$$2G:(DE-HGF)POF4-300$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lNeurodegenerative Diseases$$vDisease Mechanisms$$x1
000282298 915__ $$0StatID:(DE-HGF)0410$$2StatID$$aAllianz-Lizenz$$d2025-01-02$$wger
000282298 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bNEUROLOGY : 2022$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)1050$$2StatID$$aDBCoverage$$bBIOSIS Previews$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)0160$$2StatID$$aDBCoverage$$bEssential Science Indicators$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)1030$$2StatID$$aDBCoverage$$bCurrent Contents - Life Sciences$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)1190$$2StatID$$aDBCoverage$$bBiological Abstracts$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)0113$$2StatID$$aWoS$$bScience Citation Index Expanded$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection$$d2025-01-02
000282298 915__ $$0StatID:(DE-HGF)9905$$2StatID$$aIF >= 5$$bNEUROLOGY : 2022$$d2025-01-02
000282298 9201_ $$0I:(DE-2719)1111015$$kClinical Research (Munich)$$lClinical Research (Munich)$$x0
000282298 9201_ $$0I:(DE-2719)1111016$$kAG Levin$$lClinical Neurodegeneration$$x1
000282298 9201_ $$0I:(DE-2719)1110001$$kAG Herms$$lTranslational Brain Research$$x2
000282298 980__ $$ajournal
000282298 980__ $$aEDITORS
000282298 980__ $$aVDBINPRINT
000282298 980__ $$aI:(DE-2719)1111015
000282298 980__ $$aI:(DE-2719)1111016
000282298 980__ $$aI:(DE-2719)1110001
000282298 980__ $$aUNRESTRICTED