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024 7 _ |a 10.1002/mds.29324
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024 7 _ |a 0885-3185
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024 7 _ |a 1531-8257
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037 _ _ |a DZNE-2023-00461
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Oender, Demet
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245 _ _ |a Evolution of Clinical Outcome Measures and Biomarkers in Sporadic Adult-Onset Degenerative Ataxia.
260 _ _ |a New York, NY
|c 2023
|b Wiley
336 7 _ |a article
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520 _ _ |a Sporadic adult-onset ataxias without known genetic or acquired cause are subdivided into multiple system atrophy of cerebellar type (MSA-C) and sporadic adult-onset ataxia of unknown etiology (SAOA).To study the differential evolution of both conditions including plasma neurofilament light chain (NfL) levels and magnetic resonance imaging (MRI) markers.SPORTAX is a prospective registry of sporadic ataxia patients with an onset >40 years. Scale for the Assessment and Rating of Ataxia was the primary outcome measure. In subgroups, blood samples were taken and MRIs performed. Plasma NfL was measured via a single molecule assay. Regional brain volumes were automatically measured. To assess signal changes, we defined the pons and middle cerebellar peduncle abnormality score (PMAS). Using mixed-effects models, we analyzed changes on a time scale starting with ataxia onset.Of 404 patients without genetic diagnosis, 130 met criteria of probable MSA-C at baseline and 26 during follow-up suggesting clinical conversion to MSA-C. The remaining 248 were classified as SAOA. At baseline, NfL, cerebellar white matter (CWM) and pons volume, and PMAS separated MSA-C from SAOA. NfL decreased in MSA-C and did not change in SAOA. CWM and pons volume decreased faster, whereas PMAS increased faster in MSA-C. In MSA-C, pons volume had highest sensitivity to change, and PMAS was a predictor of faster progression. Fulfillment of possible MSA criteria, NfL and PMAS were risk factors, CWM and pons volume protective factors for conversion to MSA-C.This study provides detailed information on differential evolution and prognostic relevance of biomarkers in MSA-C and SAOA. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
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650 _ 7 |a multiple system atrophy
|2 Other
650 _ 7 |a natural history
|2 Other
650 _ 7 |a neurofilament light chain
|2 Other
650 _ 7 |a sporadic ataxia
|2 Other
650 _ 7 |a volumetric MRI
|2 Other
650 _ 7 |a Biomarkers
|2 NLM Chemicals
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Adult
|2 MeSH
650 _ 2 |a Cerebellar Ataxia: diagnosis
|2 MeSH
650 _ 2 |a Ataxia: genetics
|2 MeSH
650 _ 2 |a Cerebellum
|2 MeSH
650 _ 2 |a Multiple System Atrophy: diagnosis
|2 MeSH
650 _ 2 |a Biomarkers
|2 MeSH
700 1 _ |a Faber, Jennifer
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700 1 _ |a Wilke, Carlo
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700 1 _ |a Schaprian, Tamara
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700 1 _ |a Lakghomi, Asadeh
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700 1 _ |a Mengel, David
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700 1 _ |a Schöls, Ludger
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700 1 _ |a Traschuetz, Andreas
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700 1 _ |a Fleszar, Zofia
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700 1 _ |a Dufke, Claudia
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700 1 _ |a Vielhaber, Stefan
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700 1 _ |a Machts, Judith
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700 1 _ |a Giordano, Ilaria
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700 1 _ |a Grobe-Einsler, Marcus
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700 1 _ |a Klopstock, Thomas
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700 1 _ |a Stendel, Claudia
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700 1 _ |a Boesch, Sylvia
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700 1 _ |a Nachbauer, Wolfgang
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700 1 _ |a Timmann-Braun, Dagmar
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700 1 _ |a Thieme, Andreas Gustafsson
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700 1 _ |a Kamm, Christoph
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700 1 _ |a Dudesek, Ales
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700 1 _ |a Tallaksen, Chantal
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700 1 _ |a Wedding, Iselin
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700 1 _ |a Filla, Alessandro
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700 1 _ |a Schmid, Matthias
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700 1 _ |a Synofzik, Matthis
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700 1 _ |a Klockgether, Thomas
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773 _ _ |a 10.1002/mds.29324
|g Vol. 38, no. 4, p. 654 - 664
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