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000257585 1001_ $$0P:(DE-2719)9000685$$aOender, Demet$$b0$$eFirst author$$udzne
000257585 245__ $$aEvolution of Clinical Outcome Measures and Biomarkers in Sporadic Adult-Onset Degenerative Ataxia.
000257585 260__ $$aNew York, NY$$bWiley$$c2023
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000257585 520__ $$aSporadic 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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000257585 650_7 $$2Other$$amultiple system atrophy
000257585 650_7 $$2Other$$anatural history
000257585 650_7 $$2Other$$aneurofilament light chain
000257585 650_7 $$2Other$$asporadic ataxia
000257585 650_7 $$2Other$$avolumetric MRI
000257585 650_7 $$2NLM Chemicals$$aBiomarkers
000257585 650_2 $$2MeSH$$aHumans
000257585 650_2 $$2MeSH$$aAdult
000257585 650_2 $$2MeSH$$aCerebellar Ataxia: diagnosis
000257585 650_2 $$2MeSH$$aAtaxia: genetics
000257585 650_2 $$2MeSH$$aCerebellum
000257585 650_2 $$2MeSH$$aMultiple System Atrophy: diagnosis
000257585 650_2 $$2MeSH$$aBiomarkers
000257585 7001_ $$0P:(DE-2719)2811327$$aFaber, Jennifer$$b1$$udzne
000257585 7001_ $$0P:(DE-2719)2814101$$aWilke, Carlo$$b2
000257585 7001_ $$0P:(DE-2719)2812594$$aSchaprian, Tamara$$b3$$udzne
000257585 7001_ $$aLakghomi, Asadeh$$b4
000257585 7001_ $$0P:(DE-2719)9000375$$aMengel, David$$b5$$udzne
000257585 7001_ $$0P:(DE-2719)2810795$$aSchöls, Ludger$$b6$$udzne
000257585 7001_ $$0P:(DE-2719)9000792$$aTraschuetz, Andreas$$b7$$udzne
000257585 7001_ $$0P:(DE-2719)9000074$$aFleszar, Zofia$$b8$$udzne
000257585 7001_ $$aDufke, Claudia$$b9
000257585 7001_ $$0P:(DE-2719)2000035$$aVielhaber, Stefan$$b10$$udzne
000257585 7001_ $$0P:(DE-2719)2810317$$aMachts, Judith$$b11$$udzne
000257585 7001_ $$0P:(DE-2719)2811662$$aGiordano, Ilaria$$b12$$udzne
000257585 7001_ $$0P:(DE-2719)9001510$$aGrobe-Einsler, Marcus$$b13
000257585 7001_ $$0P:(DE-2719)2810704$$aKlopstock, Thomas$$b14$$udzne
000257585 7001_ $$0P:(DE-2719)2812141$$aStendel, Claudia$$b15$$udzne
000257585 7001_ $$aBoesch, Sylvia$$b16
000257585 7001_ $$aNachbauer, Wolfgang$$b17
000257585 7001_ $$aTimmann-Braun, Dagmar$$b18
000257585 7001_ $$aThieme, Andreas Gustafsson$$b19
000257585 7001_ $$0P:(DE-2719)9000871$$aKamm, Christoph$$b20$$udzne
000257585 7001_ $$0P:(DE-2719)2811832$$aDudesek, Ales$$b21$$udzne
000257585 7001_ $$aTallaksen, Chantal$$b22
000257585 7001_ $$aWedding, Iselin$$b23
000257585 7001_ $$aFilla, Alessandro$$b24
000257585 7001_ $$0P:(DE-2719)2811245$$aSchmid, Matthias$$b25$$udzne
000257585 7001_ $$0P:(DE-2719)2811275$$aSynofzik, Matthis$$b26
000257585 7001_ $$0P:(DE-2719)2810314$$aKlockgether, Thomas$$b27$$eLast author
000257585 773__ $$0PERI:(DE-600)2041249-6$$a10.1002/mds.29324$$gVol. 38, no. 4, p. 654 - 664$$n4$$p654 - 664$$tMovement disorders$$v38$$x0885-3185$$y2023
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