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.