Glial Fibrillary Acidic Protein Astrocytopathy Based on a Two-Center Chinese Cohort Study

Area of Science:

• Neuroimmunology
• Neurology
• Autoimmune Diseases

Background:

• Glial fibrillary acidic protein astrocytopathy (GFAP-A) is a newly identified autoimmune inflammatory disorder affecting the central nervous system (CNS).
• Understanding its clinical spectrum, diagnostic markers, and therapeutic responses is crucial for patient management.

Purpose of the Study:

• To characterize the clinical and MRI features, treatment strategies, and prognosis of GFAP-A in a Chinese cohort.
• To identify potential predictors of disease recurrence.

Main Methods:

• Retrospective analysis of 38 adult patients diagnosed with GFAP-A between June 2019 and September 2024.
• Collection and review of clinical data, GFAP antibody test results, MRI findings, treatment outcomes, and follow-up information.

Main Results:

• The cohort (n=38) had a median age of 49.5 years, with 24 males. Clinical phenotypes included encephalomyelitis, myelitis, encephalitis, and meningitis.
• MRI revealed enhancement of cerebral meninges (10.5%), ependyma (5.3%), and spinal cord pia mater (13.2%).
• 77.1% responded to glucocorticoids, 65.8% had a monophasic course, and CSF-specific oligoclonal bands correlated with 1-year relapse (p=0.003).

Conclusions:

• GFAP-A exhibits diverse clinical manifestations, including encephalitis, myelitis, and meningitis.
• MRI findings of spinal pia mater and ependymal enhancement are notable.
• Glucocorticoid therapy is effective for most patients, and CSF oligoclonal bands may predict recurrence.