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

Abstract

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