GFAP Autoimmune Astrocytopathy: Clinical and Immunological Characteristics

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an immunotherapy-responsive inflammatory disorder of the central nervous system (CNS) associated with autoantibodies targeting GFAP. It presents as acute to subacute meningoencephalitis, affecting various CNS regions. The condition primarily affects individuals over 40 years old and shows a slight female predominance. Symptoms include fever, headache, encephalopathy, and ataxia, with one-third of cases linked to malignancies, mainly ovarian teratomas. Coexisting autoimmune diseases and neuronal autoantibodies are common. This chapter explores the clinical and immunological characteristics, as well as treatment options, for GFAP autoimmune astrocytopathy.