European journal of neurology · 2026
Investigation of Prognostic Factors in Patients With Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy.
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Kimura Akio, Takekoshi Akira, Maekawa Yoichi, Tanaka Keiko, Yamano Yoshihisa, Saito Kuniaki, Takemura Masao, Shimohata Takayoshi
Аннотация
BACKGROUND: Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is a newly recognized autoimmune disorder of the central nervous system, characterized by the presence of GFAP-IgG in the cerebrospinal fluid. Although corticosteroid therapy typically yields favorable responses, limited therapeutic efficacy is observed in some patients who subsequently develop poor clinical outcomes. This study aimed to identify prognostic factors using clinical data of patients with GFAP-A.
METHODS: This retrospective cohort study included 233 patients with GFAP-A, who were followed up for more than 6 months. At 6 months after admission, patients were classified into unfavorable outcome [modified Rankin Scale (mRS) score ≥ 3] and favorable outcome (mRS ≤ 2) groups. Clinical data were compared between the two groups using univariate analysis. Variables that showed statistically significant differences were subsequently analyzed using multiple regression analysis to identify the individual contribution of each predictor to the unfavorable outcomes at 6 months after admission.
RESULTS: In the multiple regression analysis, age (p < 0.001), seizures (p = 0.050), motor paralysis (p = 0.042), and mRS scores at admission (p = 0.001) were positively associated with unfavorable outcomes. In contrast, fever (p = 0.020) was negatively associated with unfavorable outcomes.
CONCLUSIONS: In conclusion, advanced age, comorbid seizures, comorbid motor paralysis, lack of febrile episodes, and higher mRS scores at admission were associated with unfavorable outcomes in our cohort. These findings highlight the need for future studies to elucidate the underlying pathophysiological mechanisms associated with these clinical characteristics and to develop effective alternatives to corticosteroid therapy.
METHODS: This retrospective cohort study included 233 patients with GFAP-A, who were followed up for more than 6 months. At 6 months after admission, patients were classified into unfavorable outcome [modified Rankin Scale (mRS) score ≥ 3] and favorable outcome (mRS ≤ 2) groups. Clinical data were compared between the two groups using univariate analysis. Variables that showed statistically significant differences were subsequently analyzed using multiple regression analysis to identify the individual contribution of each predictor to the unfavorable outcomes at 6 months after admission.
RESULTS: In the multiple regression analysis, age (p < 0.001), seizures (p = 0.050), motor paralysis (p = 0.042), and mRS scores at admission (p = 0.001) were positively associated with unfavorable outcomes. In contrast, fever (p = 0.020) was negatively associated with unfavorable outcomes.
CONCLUSIONS: In conclusion, advanced age, comorbid seizures, comorbid motor paralysis, lack of febrile episodes, and higher mRS scores at admission were associated with unfavorable outcomes in our cohort. These findings highlight the need for future studies to elucidate the underlying pathophysiological mechanisms associated with these clinical characteristics and to develop effective alternatives to corticosteroid therapy.