The clinical features of autoimmune glial fibrillary acidic protein astrocytopathy
10.3760/cma.j.cn113694-20250124-00056
- VernacularTitle:自身免疫性胶质纤维酸性蛋白星形细胞病的临床特点分析
- Author:
Lu ZHENG
1
;
Xiaonan ZHONG
1
;
Yaqing SHU
1
;
Wei QIU
1
;
Zhengqi LU
1
;
Yuge WANG
1
;
Rui LI
1
Author Information
1. 中山大学附属第三医院神经内科，广州510630
- Publication Type:Journal Article
- Keywords:
Autoimmune diseases of the nervous system;
Encephalitis;
Meningoencephalitis;
Myelitis;
Glial fibrillary acidic protein
- From:
Chinese Journal of Neurology
2025;58(7):742-749
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the clinical symptoms, imaging characteristics, cerebrospinal fluid (CSF) features, as well as the treatment and prognosis of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.Methods:Sixty-one patients with anti-GFAP astrocyte antibody (GFAP-IgG) single-positive autoimmune encephalitis who were treated at the Third Affiliated Hospital, Sun Yat-sen University between January 2017 and September 2023 were retrospectively collected. The demographic characteristics (age at onset, sex), clinical symptoms (core symptoms, neurological deficits, psychiatric behavioral abnormalities, and autonomic dysfunction), imaging features [brain/spinal cord/optic nerve magnetic resonance imaging (MRI) lesion distribution and enhancement patterns], and CSF parameters were analyzed. Acute-phase treatments, including methylprednisolone pulse therapy, intravenous immunoglobulin (IVIG), etc, along with the follow-up outcomes [modified Rankin Scale (mRS) score] were recorded.Results:The onset age was 40 (30, 55) years, and 68.9% (42/61) of the patients were male. The most common clinical manifestations were fever (65.6%, 40/61), headache (60.7%, 37/61), and urinary/defecatory abnormalities (45.9%, 28/61). Brain MRI revealed lesions predominantly in the cerebral cortex and subcortical white matter (57.4%, 35/61), periventricular white matter (50.8%, 31/61), and basal ganglia (36.1%, 22/61). Periventricular linear-radiating enhancement was the predominant MRI enhancement pattern (55.7%, 34/61). Spinal MRI showed lesions mainly in the cervical (42.6%, 26/61) and thoracic spinal cord (32.8%, 30/61), with leptomeningeal enhancement (31.1%, 19/61) and scattered punctate/patchy enhancements (21.3%, 13/61). Optic neuropathy was observed in 6 cases (9.8%). CSF analysis demonstrated a pressure of 180 (133, 240) mmH 2O (1 mmH 2O=0.009 8 kPa), white blood cell count of 29 (4, 156)×10?/L, and protein level of 0.72 (0.40, 1.44) g/L. Nineteen patients (31.1%) experienced rapid progression of meningoencephalitis or myelitis within 3 days of admission. All patients received methylprednisolone pulse therapy, with 47.5% (29/61) additionally treated with IVIG. At a follow-up of 12 (3, 28) months, 12 cases (19.7%) relapsed, and 75.4% (46/61) had favorable outcomes (mRS score 0-2). Poor prognosis (mRS score>2) was observed in 4 cases, including 3 with cervical spinal cord involvement and status epilepticus, 1 elderly patient with lung cancer. Conclusions:GFAP astrocytopathy predominantly affects young adults, with a male predominance. Spinal cord involvement is common, manifesting as myelitis and myelopathy. Rapid progression of meningoencephalitis or myelitis may occur early in the disease course. Periventricular linear-radiating enhancement on brain MRI is a key diagnostic clue. Leukocyte and protein levels in the cerebrospinal fluid are generally mildly to moderately elevated. Most patients respond well to corticosteroids and immunotherapy, with favorable outcomes. However, advanced age and cervical spinal cord involvement are associated with poor prognosis.
