TY  - JOUR
AU  - Wilkens, Ida
AU  - Bebermeier, Sarah
AU  - Heine, Johanne
AU  - Ruf, Viktoria Constanze
AU  - Compta, Yaroslau
AU  - Molina Porcel, Laura
AU  - Troakes, Claire
AU  - Vamanu, Albert
AU  - Downes, Sophia
AU  - Irwin, David John
AU  - Cohen, Jesse
AU  - Lee, Edward B
AU  - Nilsson, Christer F
AU  - Englund, Elisabet M
AU  - Nemati, Mojtaba
AU  - Katzdobler, Sabrina
AU  - Levin, Johannes
AU  - Bernhardt, Alexander Maximilian
AU  - Pantelyat, Alexander
AU  - Seemiller, Joseph
AU  - Berger, Stephen
AU  - Van Swieten, John C
AU  - Dopper, Elise G P
AU  - Rozemuller, Annemieke J M
AU  - Kovacs, Gabor G
AU  - Bendahan, Nathaniel
AU  - Lang, Anthony E
AU  - Herms, Jochen
AU  - Höglinger, Günter U
AU  - Hopfner, Franziska
TI  - Multiple System Atrophy Without Dysautonomia: An Autopsy-Confirmed Study.
JO  - Neurology
VL  - 105
IS  - 11
SN  - 0028-3878
CY  - Philadelphia, Pa.
PB  - Wolters Kluwer
M1  - DZNE-2025-01268
SP  - e214316
PY  - 2025
AB  - Multiple 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
KW  - Humans
KW  - Multiple System Atrophy: pathology
KW  - Multiple System Atrophy: diagnosis
KW  - Multiple System Atrophy: physiopathology
KW  - Female
KW  - Male
KW  - Middle Aged
KW  - Aged
KW  - Autopsy
KW  - Primary Dysautonomias
KW  - Disease Progression
KW  - Cohort Studies
KW  - Parkinsonian Disorders
LB  - PUB:(DE-HGF)16
C6  - pmid:41232058
C2  - pmc:PMC12615010
DO  - DOI:10.1212/WNL.0000000000214316
UR  - https://pub.dzne.de/record/282298
ER  -