Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy underlies Autoimmune Epilepsy in the setting of Multi-Organ Failure (P4.288)

Objective: Report a GFAP astrocytopathy causing unexplained seizures in the setting of multi-organ failure. Background: GFAP astrocytopathy is an autoimmune CNS disorder. The predominant clinical presentations are related to meningitis, encephalitis, and myelitis. Encephalopathy, psychiatric symptoms, seizures, and ataxia are among the most common presenting symptoms. In addition to GFAP antibodies in CSF and serum, diagnostic findings include linear perivascular radial post-contrast enhancement on MRI. The condition can be associated with malignancy. Design/Methods: GFAP Antibody was detected in the CSF of a 58-year-old woman who presented with new onset retractable seizures, skin rash and multi-organ failure including chronic pancreatitis, colitis, and heart failure. MRI was negative for acute pathology and seizures were refractory to multiple AED’s, causing concern for an autoimmune process. The patient was treated with IVIG and steroids, with clinical improvement. She developed diffuse maculopapular rash likely secondary to phenytoin and shortly after suffered from acute hepatic and renal failure. Her Dilantin was stopped concerning for hypersensitivity reaction and methylprednisolone was given at 250 mg q6 hours. She continued to have focal status epilepticus, requiring intubation and sedation. PET CT and MRI showed FLAIR hyperintensity in the right temporoparietal lobe, EEG with temporoparietal PLEDS, LP with GFAP antibody positivity in CSF studies. She continued to improve clinically. Results: This case highlights retractable seizures as presenting symptoms for GFAP astrocytopathy. Additionally, this may represent the first reported case to include multi-system organ failure, without underlying malignancy, in the initial presentation. Finally, this case highlights the role for predictive models, such as the antibody prevalence in epilepsy (APE) and Response to Immunotherapy in Epilepsy(RITE) in the evaluation and management of autoimmune epilepsy. Conclusions: Detected GFAP antibody in the CSF was likely causing meningoencephalitis and autoimmune epilepsy. Study Supported by: citations for Fang 2016 and Flanagan 2017 Disclosure: Dr. Dababneh has nothing to disclose. Dr. Notch has nothing to disclose. Dr. Kafaie has nothing to disclose.