Abstract Details

Title Anti-GABAB Receptor Autoimmune Encephalitis: First Case Report in Central America

Topic Autoimmune Neurology

Presentation(s) P3 - Poster Session 3 (12:00 PM-1:00 PM)

Poster/Presentation
Number 019

Objective To describe the first case of anti-gamma-aminobutyric-acid-B receptor (GABABR) encephalitis in Central America.

Background Autoimmune encephalitis (AE) is a rare disease. There have been very few reports of anti-GABABR encephalitis, and no case of this subtype has ever been reported in Central America.

Design/Methods NA

Results A 21-year-old male with unremarkable previous medical history was hospitalized because of a new onset of seizures and status epilepticus. Central nervous system infections, neoplastic disorders, cerebrovascular disease, septic and metabolic encephalopathy, and drug toxicity were ruled out. Cerebrospinal fluid (CSF) revealed lymphocytic pleocytosis and oligoclonal bands. Initial head computed tomography (CT) scans with and without contrast were normal. An electroencephalogram showed slow waves and spike waves in the frontal and temporal area. During hospitalization, encephalopathy progressed, along with seizures and altered mental status requiring mechanical ventilation and admission to the Intensive Care Unit. Intravenous valproic acid and phenytoin for seizure control were given. Magnetic resonance imaging revealed hyperintense signals on T2-weighted imaging and fluid-attenuated inversion recovery sequences that were mainly located in the paraventricular region. The unexplained seizures, persisting altered mental status despite reduction of sedatives, CSF pleocytosis and oligoclonal bands, MRI findings, along with reasonable exclusion of alternative disorders, suggested AE. Diagnosis was confirmed with positive anti-GABAB1-B2 receptor antibody titers in serum and CSF. Whole body CT scan showed increased pancreatic head size, but endoscopic ultrasonography ruled out malignancy, and a normal IgG4 range excluded IgG4 disease. The patient received treatment with methylprednisolone, plasmapheresis, and immunoglobulin therapy, with excellent response. The patient has been followed up for 7 months, taking immunomodulation with mycophenolate. He is seizure free with valproic acid and levetiracetam treatment, and is receiving cognitive rehabilitation after mild cognitive decline was noted in psychometric analysis.

Conclusions We describe the first case of autoimmune encephalitis with anti-GABAB1-B2 receptor antibodies in Central America.

Authors/Disclosures
Ana Santos, MD PRESENTER	Ms. Santos has nothing to disclose.
Anthony Hong, MD	Dr. Hong has nothing to disclose.
Jose D. Villegas, MD (CCSS)	Dr. Villegas has nothing to disclose.

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