%0 Journal Article
%A Oender, Demet
%A Faber, Jennifer
%A Wilke, Carlo
%A Schaprian, Tamara
%A Lakghomi, Asadeh
%A Mengel, David
%A Schöls, Ludger
%A Traschuetz, Andreas
%A Fleszar, Zofia
%A Dufke, Claudia
%A Vielhaber, Stefan
%A Machts, Judith
%A Giordano, Ilaria
%A Grobe-Einsler, Marcus
%A Klopstock, Thomas
%A Stendel, Claudia
%A Boesch, Sylvia
%A Nachbauer, Wolfgang
%A Timmann-Braun, Dagmar
%A Thieme, Andreas Gustafsson
%A Kamm, Christoph
%A Dudesek, Ales
%A Tallaksen, Chantal
%A Wedding, Iselin
%A Filla, Alessandro
%A Schmid, Matthias
%A Synofzik, Matthis
%A Klockgether, Thomas
%T Evolution of Clinical Outcome Measures and Biomarkers in Sporadic Adult-Onset Degenerative Ataxia.
%J Movement disorders
%V 38
%N 4
%@ 0885-3185
%C New York, NY
%I Wiley
%M DZNE-2023-00461
%P 654 - 664
%D 2023
%X 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.
%K Humans
%K Adult
%K Cerebellar Ataxia: diagnosis
%K Ataxia: genetics
%K Cerebellum
%K Multiple System Atrophy: diagnosis
%K Biomarkers
%K multiple system atrophy (Other)
%K natural history (Other)
%K neurofilament light chain (Other)
%K sporadic ataxia (Other)
%K volumetric MRI (Other)
%K Biomarkers (NLM Chemicals)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:36695111
%R 10.1002/mds.29324
%U https://pub.dzne.de/record/257585