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Prognostic and relapsing factors of primary autoimmune cerebellar ataxia: a prospective cohort study.
- Source :
- Journal of Neurology; Mar2024, Vol. 271 Issue 3, p1072-1079, 8p
- Publication Year :
- 2024
-
Abstract
- Objective: The objective of this study was to investigate the factors influencing relapse and prognosis in patients with primary autoimmune cerebellar ataxia (PACA), an area previously not well understood. Methods: This prospective cohort study included patients who satisfied the modified diagnostic criteria of PACA. A modified Rankin scale score ≤ 2 at the last follow-up was defined as a favorable prognosis. Cox and Logistic regression were utilized to identify relapsing and prognostic factors, respectively. Results: A total of 68 patients were included and 35.3% were male. The median onset age was 42.9 years (IQR 22.1–54.0). Neuronal autoantibodies were detected in 33 (50.8%) patients. Of the 65 patients who received first-line immunotherapy, 55 (84.6%) were responsive and 10 (15.4%) were not. Responsiveness to first-line immunotherapy emerged as an independent factor for favorable prognosis (HR 16.762; 95% CI 2.877–97.655; p = 0.002), as did the absence of peripheral neuropathy/radiculopathy (HR 14.286; 95% CI 2.41–83.333; p = 0.003). Relapses occurred in 19 (27.9%) patients. Onset age ≤ 43 years (HR 5.245; 95% CI 1.499–18.35; p = 0.009), presence of peripheral neuropathy/radiculopathy (HR 4.280; 95% CI 1.622–11.298; p = 0.003) and elevated cerebrospinal fluid (CSF) protein concentration (HR 3.443; 95% CI 1.083–10.951; p = 0.036) were statistically significant relapsing factors. Conclusion: This study identified younger onset age, presence of peripheral neuropathy/radiculopathy and elevated CSF protein concentration as relapsing factors, and absence of peripheral neuropathy/radiculopathy and responsiveness to first-line immunotherapy as independent factors for favorable prognosis in PACA patients. These findings may guide individualized treatment strategies and potentially improve patient outcomes. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 03405354
- Volume :
- 271
- Issue :
- 3
- Database :
- Complementary Index
- Journal :
- Journal of Neurology
- Publication Type :
- Academic Journal
- Accession number :
- 175675394
- Full Text :
- https://doi.org/10.1007/s00415-023-12128-9