Evolution of Clinical Outcome Measures and Biomarkers in Sporadic Adult‐Onset Degenerative Ataxia
ABSTRACT Background 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). Objectives To study the differential evolution of both conditions including plas...
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Published in | Movement disorders Vol. 38; no. 4; pp. 654 - 664 |
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Main Authors | , , , , , , , , , , , , , , , , , , , , , , , , , , , |
Format | Journal Article |
Language | English Norwegian |
Published |
Hoboken, USA
John Wiley & Sons, Inc
01.04.2023
Wiley Subscription Services, Inc |
Subjects | |
Online Access | Get full text |
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Summary: | ABSTRACT
Background
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).
Objectives
To study the differential evolution of both conditions including plasma neurofilament light chain (NfL) levels and magnetic resonance imaging (MRI) markers.
Methods
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.
Results
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.
Conclusions
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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Bibliography: | Relevant conflicts of interest/financial disclosures Authors declare no conflicts of interest related to this work. Demet Önder and Jennifer Faber equally contributed to the work ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 |
ISSN: | 0885-3185 1531-8257 |
DOI: | 10.1002/mds.29324 |