Evolution of Clinical Outcome Measures and Biomarkers in Sporadic Adult-Onset Degenerative Ataxia.
In: Movement disorders 38(4), 654 - 664 (2023). doi:10.1002/mds.29324, 2023
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Zugriff:
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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Evolution of Clinical Outcome Measures and Biomarkers in Sporadic Adult-Onset Degenerative Ataxia.
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Autor/in / Beteiligte Person: | Oender, Demet ; Faber, Jennifer ; Vielhaber, Stefan ; Machts, Judith ; Giordano, Ilaria ; Grobe-Einsler, Marcus ; Klopstock, Thomas ; Stendel, Claudia ; Boesch, Sylvia ; Nachbauer, Wolfgang ; Timmann-Braun, Dagmar ; Thieme, Andreas Gustafsson ; Wilke, Carlo ; Kamm, Christoph ; Dudesek, Ales ; Tallaksen, Chantal ; Wedding, Iselin ; Filla, Alessandro ; Schmid, Matthias ; Synofzik, Matthis ; Klockgether, Thomas ; Schaprian, Tamara ; Lakghomi, Asadeh ; Mengel, David ; Schöls, Ludger ; Traschuetz, Andreas ; Fleszar, Zofia ; Dufke, Claudia |
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Zeitschrift: | Movement disorders 38(4), 654 - 664 (2023). doi:10.1002/mds.29324, 2023 |
Veröffentlichung: | Wiley, 2023 |
Medientyp: | academicJournal |
DOI: | 10.1002/mds.29324 |
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