Objective: Our study aims to investigate the clinical parameters associated with faster conversion from late-onset cerebellar ataxia (ILOCA) to multiple system atrophy cerebellar type (MSA-C) and predictive ability of neurofilament light chain (NFL).

Background: Patients with ILOCA may later progress to MSA-C. However, studies about the time to conversion, clinical factors associated with faster progression, and the role of NFL in this process remain unclear.

Method: This retrospective study included patients with ILOCA at the initial visit and the conversion to MSA-C and its time to progression. Median time to conversion was estimated using Kaplan-Meier test. The role of baseline orthostatic dizziness (oDz), REM sleep behavior disorder (RBD), and the hot cross bun (HCB) sign on conversion were analyzed. In a subset of patients whose plasma was collected, the NFL level was measured to examine its ability in predicting the conversion.

Results: Seventy-two patients with ILOCA at initial visit were included and among them 32 patients converted to MSA-C. Median time to conversion was 5.0 years. RBD or oDz at baseline was associated with faster conversion, whereas HCB sign had no effect. ROC analysis showed only a moderate discriminative ability when NFL, RBD, oDz, and age at plasma collection were all included.

Conclusion: In this study, we identified the progression time from ILOCA to MSA-C and found that the presence of orthostatic dizziness and RBD, which can be easily assessed in an outpatient clinic, is associated with a more rapid progression.

« Back to 2025 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/predictive-clinical-factors-for-the-faster-progression-from-idiopathic-late-onset-cerebellar-ataxia-to-multiple-system-atrophy-cerebellar-type/