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Background <p>The aim was to describe the clinical course and long-term outcomes of the French cohort of patients with glial fibrillary acidic protein (GFAP) astrocytopathy.</p> Methods <p>Patients with positive CSF GFAP antibody test were identified between May 2017 and February 2023. Those whose clinical presentation occurred &lt; 2&#xa0;years before the initiation of the study, with other diagnosis than GFAP astrocytopathy, and with missing clinical information were excluded.</p> Results <p>Among the 74 patients included, 71 were alive at last follow-up. The median age at onset was 43&#xa0;years (range 6–84), 44 patients were male (62%), and 11 (15%) had a neoplasia. The main initial syndrome was meningo-encephalitis (<i>n</i> = 41, 58%). The median follow-up was 28&#xa0;months (range 1–129). The median mRS at presentation was 4 (range 1–5) and at last follow-up was 1 (range 0–4). Forty patients reported disability at last follow-up (56%). The most frequent sequelae were cognitive complaints (20/40, 50%) and gait disorder (19/40, 48%). 38/55 patients (69%) returned to school/work. Long-term immunoactive treatment was introduced in 40 patients (56%); the most commonly administered were oral corticosteroids (<i>n</i> = 35, 49%). Relapses were documented in 10 patients (14%) and occurred after a median follow-up of 9&#xa0;months (range 3–46). The presence of concomitant tumor at onset was associated with relapse (HR 4.55, 95% CI 1.28–16.14, <i>p</i> = 0.03).</p> Conclusions <p>This study suggests a greater impact than previously described in long-term outcomes of patients with GFAP astrocytopathy and reports concomitant tumor at presentation as a risk factor for relapse.</p>

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Clinical course and long-term outcomes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年5月26日

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  • Joaquín Arzalluz-Luque,
  • Pauline Dumez,
  • Géraldine Picard,
  • Marie Benaiteau,
  • Maxime Bonjour,
  • Pierre Lardeux,
  • Julian Theuriet,
  • Florian Lamblin,
  • Marie Rafiq,
  • Jerome Honnorat,
  • Romain Marignier

摘要

Background

The aim was to describe the clinical course and long-term outcomes of the French cohort of patients with glial fibrillary acidic protein (GFAP) astrocytopathy.

Methods

Patients with positive CSF GFAP antibody test were identified between May 2017 and February 2023. Those whose clinical presentation occurred < 2 years before the initiation of the study, with other diagnosis than GFAP astrocytopathy, and with missing clinical information were excluded.

Results

Among the 74 patients included, 71 were alive at last follow-up. The median age at onset was 43 years (range 6–84), 44 patients were male (62%), and 11 (15%) had a neoplasia. The main initial syndrome was meningo-encephalitis (n = 41, 58%). The median follow-up was 28 months (range 1–129). The median mRS at presentation was 4 (range 1–5) and at last follow-up was 1 (range 0–4). Forty patients reported disability at last follow-up (56%). The most frequent sequelae were cognitive complaints (20/40, 50%) and gait disorder (19/40, 48%). 38/55 patients (69%) returned to school/work. Long-term immunoactive treatment was introduced in 40 patients (56%); the most commonly administered were oral corticosteroids (n = 35, 49%). Relapses were documented in 10 patients (14%) and occurred after a median follow-up of 9 months (range 3–46). The presence of concomitant tumor at onset was associated with relapse (HR 4.55, 95% CI 1.28–16.14, p = 0.03).

Conclusions

This study suggests a greater impact than previously described in long-term outcomes of patients with GFAP astrocytopathy and reports concomitant tumor at presentation as a risk factor for relapse.