A Fatal Refractory Status Epilepticus Due to A GAD65 Positive Autoimmune Limbic Encephalitis

• DOI: 10.26502/acmcr.96550095
• Publication Date: 2019-07-27T17:26:56Z
• Authors: Zied Hajjej; Walid Sellami; Aicha Rebai; Jamel Zaoueli; Ridha Mrissa; Hedi Gharsallah; Iheb Labbene; Mustapha Ferjani
• Keywords: Limbic Encephalitis; Autoimmune encephalitis; Plasmapheresis; Refractory (planetary science)

Autoimmune encephalitis is a dysimmune disease characterized by widely clinical polymorphism, ranging from the insidious onset of cognitive disorder to more complex forms with refractory status epilepticus. We are dealing here case limbic encephalitis, which initially presented sudden convulsions preceded essentially some psychiatric manifestations (episodes disorientation, mood and bizarre behaviors). The classic paraneoplastic investigation was negative. Cerebrospinal fluid (CSF) serum examination showed elevated titers glutamic decarboxylase 65 (GAD65) antibodies leading diagnosis non-paraneoplastic encephalitis. Evolution fatal following prolonged stay in intensive care unit for epilepticus despite immunotherapy regimens including steroid, intravenous immunoglobulin (IVIg), plasma exchange.