Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics

Area of Science:

• Neuroimmunology
• Autoimmune disorders of the Central Nervous System (CNS)

Background:

• Glial fibrillary acidic protein (GFAP) astrocytopathy is a newly identified autoimmune inflammatory CNS disorder.
• It involves antibodies targeting GFAP in mature astrocytes, with a significant paraneoplastic association (20-34%), often linked to ovarian teratomas.

Purpose of the Study:

• To provide a comprehensive review of GFAP astrocytopathy.
• To elucidate its clinical, radiographic, and histopathologic features.
• To highlight neuroimaging findings for differential diagnosis from other neuroinflammatory diseases.

Main Methods:

• Literature review focusing on clinical presentations, pathogenesis, diagnostic methods, and neuroimaging.
• Analysis of characteristic imaging findings, including perivascular enhancement and other CNS abnormalities.
• Comparison with overlapping neuroinflammatory disorders like neuromyelitis optica spectrum disorder.

Main Results:

• GFAP astrocytopathy presents as acute/subacute encephalomyelitis.
• Diagnosis relies on GFAP-Immunoglobulin (GFAP-IgG) in CSF and specific imaging patterns (e.g., radial perivascular enhancement).
• Significant overlap exists with other neuroinflammatory conditions, posing diagnostic challenges.

Conclusions:

• GFAP astrocytopathy is a distinct neuroinflammatory entity with specific diagnostic markers and imaging features.
• While responsive to steroids, relapse is common, necessitating further research for standardized criteria and treatment.
• Accurate differentiation from other CNS inflammatory disorders is crucial for appropriate management.