A Rapidly Fatal Case of Anti-GFAP Receptor Encephalitis Due to Acute Brain Edema and Herniation

Objective Glial fibrillary acidic protein antibody (GFAP) is a newly recognized biomarker for an immunotherapy responsive autoimmune meningo-encephalomyelitis with wide variety of clinical presentations. We report the second GFAP positive case in young man who died despite appropriate and aggressive immunomodulatory treatment. Background 29 year old previously healthy male childhood immune disorder presented 3 week history acute progressive worsening headaches, bloody emesis, nausea, blurry vision generalized weakness. Exam was significant downbeating nystagmus, limb ataxia tremor later progressing into inattention, confusion, urinary retention, asymmetric pupils, hyprreflexia lack motor or sensory response. Lumbar puncture revealed lymphocytic pleocytosis elevated opening pressure 36 cm H20. MRI demonstrated areas restricted diffusions symmetrically involving white matter corpus callosum, middle cerebellar peduncle, bilaterally as well within pons centrally. Patient started on intravenous immunogammaglobulin (IVIG) pulse corticosteroids along broad spectrum antimicrobial therapy. After initial apparent response to treatment, repeat head CT showed Diffuse Sulci effacement. Shortly after, He rapidly decompensated findings indicating brainstem herniation, cardiac arrest brain death diagnosed. CSF studies subsequently were reported antibodies. An autopsy cause tonsillar herniation secondary diffuse cerebral edema. all sections perivascular inflammation gliosis. Design/Methods NA. Results Conclusions This anti-GFAP unusual rapid onset edema progression occurring only 4 weeks after symptom onset. While this may be rare complication Anti encephalitis, clinicians should vigilant acutely increased intracranial patients encephalitis general.