Introduction:
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an inflammatory disease of the central nervous system (CNS). It can affect optic nerve, cerebral and cerebellar white matter, cortex, spinal cord and meninges producing a wide spectrum of clinical manifestations. Meningoencephalitis is the most common clinical presentation. Diagnosis is confirmed by the presence of CSF immunoglobulin-G (IgG) against GFAP, an intracellular astrocytic intermediate filament protein.(1)
Common clinical presentation includes encephalopathy, psychiatric disorders, tremor, ataxia, seizures, myelopathy, and meningitis. (2,3)
This disorder is equally common in both men and women.(4) Association with other autoimmune disorders like autoimmune thyroid disease, Type-1 diabetes mellitus, and rheumatoid arthritis is seen in about 20% of the cases. (3) Neoplasm is found in about 25% of cases, most common being ovarian teratoma. (3)
Brain imaging studies reveal a characteristic pattern of linear and radial perivascular enhancement in the cerebral white matter, originating from GFAP-enriched periventricular areas. Occasionally a similar pattern of radial enhancement is seen in the cerebellum. (3,5) CSF analysis invariably shows distinct inflammatory changes. (3) An extensive inflammation is seen around microvessels in pathological studies, which is in correlation with the radial inflammatory changes seen in brain MRI. (6) Here we report a case of GFAP associated meningo-encephalitis in a case of rheumatoid arthritis.