Cerebral Herniation and Sudden Death in Autoimmune Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy: A Case Report

We describe what, to our knowledge, is the third reported fatal outcome in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy secondary to cerebral edema and brainstem herniation. A 42-year-old woman presented with neck and back pain with tremors, progressing to confusion and memory impairment. Neuroimaging revealed encephalomyelitis. CSF revealed antibodies to GFAP. After an initial response to corticosteroids, she had recurrence of encephalopathy. Following a repeat lumbar puncture, she developed diffuse cerebral edema with herniation and brain death. Diffuse cerebral edema should be considered a rare but potentially fatal presentation of GFAP astrocytopathy. Clinicians involved in the care of patients diagnosed with or suspected to have GFAP astrocytopathy should remain vigilant about the development of cerebral edema throughout the diagnostic and treatment process and evaluate the patient with neuroimaging before initial and subsequent lumbar punctures.