ArticleJournal of neurology2026
Clinical characteristics of late-onset adult autoimmune glial fibrillary acidic protein astrocytopathy: a retrospective cohort study.
Article in Journal of neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundTo characterize the distinct clinical phenotype of late‑onset (onset age ≥ 50 years) adult autoimmune glial fibrillary acidic protein astrocytopathy (A‑GFAP‑A).
methodsThis single-center retrospective study included 104 adult patients diagnosed with A-GFAP-A, who were stratified into the late-onset group (LOG, n = 31) and the early-onset group (EOG, n = 73). Demographic, clinical, cerebrospinal fluid (CSF), neuroimaging, and short-term outcome data were compared. Multivariable logistic regression and hierarchical cluster analysis were performed.
resultsCompared with EOG, LOG showed a higher prevalence of hypertension (19.4% vs. 2.7%, p = 0.012), a higher proportion of moderately severe disability (modified Rankin Scale [mRS] score = 4) (29.0% vs. 12.3%, p = 0.039), and a lower proportion of severe disability (mRS = 5) (22.6% vs. 45.2%, p = 0.030). Meanwhile, LOG had a lower incidence of bowel/bladder dysfunction (54.8% vs. 76.7%, p = 0.026), a lower rate of elevated CSF opening pressure (25.8% vs. 64.4%, p < 0.001), and less thalamic involvement (16.1% vs. 38.4%, p = 0.026). After adjustment for hypertension, LOG was associated with higher odds of moderately severe disability (mRS = 4), as well as lower odds of elevated CSF opening pressure and thalamic involvement. Symptom clustering analysis revealed a looser and more discrete phenotypic architecture in the LOG. No significant intergroup differences were observed in mRS scores at short-term follow-up.
conclusionsLate-onset adult A-GFAP-A presents a distinct clinical phenotype. However, there was no significant difference in short-term outcomes between the groups.
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