Multiple sclerosis and related disorders · 2026
Evaluation of blood-brain barrier integrity and clinical characteristics in autoimmune glial fibrillary acidic protein astrocytopathy.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Chen Yanlan, Wu Yu, Que Xianting, Liang Manli, Jiang Ailing, Lin Ziqun, Shi Danli, Huang Yanzhen, Liu Chao, Huang Wen
Аннотация
OBJECTIVES: Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a rare autoimmune disorder of unknown pathogenesis. This study aimed to investigate the association between blood-brain barrier (BBB) disruption and the clinical features, disease severity, and prognosis in GFAP-A, and to evaluate its potential clinical relevance. Furthermore, we sought to delineate the distinct clinical profiles of GFAP-A through a comparative analysis with GFAP antibody-overlap syndromes and AQP4-IgG-positive neuromyelitis optica spectrum disorder (AQP4-IgG+NMOSD).
METHODS: This retrospective study included 61 patients with isolated GFAP-IgG positivity (CSF GFAP positivity without overlapping antibodies) from a multicenter cohort in Guangxi Province between March 2019 and April 2025. Patients were stratified by cerebrospinal fluid (CSF)/serum albumin quotient (Qalb) levels (Elevated, n = 43; Normal, n = 18). Patients were stratified by outcome at 6 months after discharge on the modified Rankin Scale (mRS) into poor outcome (mRS ≥ 3) and good outcome (mRS < 3) groups, and key prognostic determinants were identified through multivariable regression. Subsequently, a comparative analysis was conducted with two other cohorts: GFAP-IgG-positive patients with overlapping antibodies (n = 29) and a cohort of AQP4-IgG+NMOSD patients (n = 81).
RESULTS: In the isolated GFAP cohort, the elevated Qalb group exhibited more severe clinical manifestations, including prodromal symptoms and autonomic dysfunction, alongside significantly higher CSF white blood cell (WBC) count, protein, albumin, and IgG levels. Conversely, chloride levels were significantly decreased in CSF. This group also demonstrated consistently higher mRS scores from admission through the 6-month follow-up. Multivariable regression identified elevated Qalb (OR 13.487, 95% CI 1.094-166.256; P = 0.042) and decreased CSF chloride (OR 0.884, 95% CI 0.786-0.993; P = 0.038) as independent predictors of poor outcomes. Receiver operating characteristic (ROC) curve analysis showed that their combination provided excellent predictive accuracy (AUC=0.858, 95% CI: 0.751-0.965). Compared to the overlap syndrome cohort, isolated GFAP-positive patients demonstrated a higher prevalence of male, fever, and elevated CSF parameters (WBC count, protein, and IgG). In contrast to AQP4-IgG+NMOSD, Patients with GFAP-A exhibited a distinct meningoencephalitis phenotype with inflammatory CSF shifts and the novel finding of CSF hypochloremia.
CONCLUSIONS: BBB disruption (as indicated by elevated Qalb) correlates with heightened disease severity and an unfavorable prognosis in GFAP-A, supporting its potential role as a clinical predictor.
METHODS: This retrospective study included 61 patients with isolated GFAP-IgG positivity (CSF GFAP positivity without overlapping antibodies) from a multicenter cohort in Guangxi Province between March 2019 and April 2025. Patients were stratified by cerebrospinal fluid (CSF)/serum albumin quotient (Qalb) levels (Elevated, n = 43; Normal, n = 18). Patients were stratified by outcome at 6 months after discharge on the modified Rankin Scale (mRS) into poor outcome (mRS ≥ 3) and good outcome (mRS < 3) groups, and key prognostic determinants were identified through multivariable regression. Subsequently, a comparative analysis was conducted with two other cohorts: GFAP-IgG-positive patients with overlapping antibodies (n = 29) and a cohort of AQP4-IgG+NMOSD patients (n = 81).
RESULTS: In the isolated GFAP cohort, the elevated Qalb group exhibited more severe clinical manifestations, including prodromal symptoms and autonomic dysfunction, alongside significantly higher CSF white blood cell (WBC) count, protein, albumin, and IgG levels. Conversely, chloride levels were significantly decreased in CSF. This group also demonstrated consistently higher mRS scores from admission through the 6-month follow-up. Multivariable regression identified elevated Qalb (OR 13.487, 95% CI 1.094-166.256; P = 0.042) and decreased CSF chloride (OR 0.884, 95% CI 0.786-0.993; P = 0.038) as independent predictors of poor outcomes. Receiver operating characteristic (ROC) curve analysis showed that their combination provided excellent predictive accuracy (AUC=0.858, 95% CI: 0.751-0.965). Compared to the overlap syndrome cohort, isolated GFAP-positive patients demonstrated a higher prevalence of male, fever, and elevated CSF parameters (WBC count, protein, and IgG). In contrast to AQP4-IgG+NMOSD, Patients with GFAP-A exhibited a distinct meningoencephalitis phenotype with inflammatory CSF shifts and the novel finding of CSF hypochloremia.
CONCLUSIONS: BBB disruption (as indicated by elevated Qalb) correlates with heightened disease severity and an unfavorable prognosis in GFAP-A, supporting its potential role as a clinical predictor.