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

Author(s): Zied Hajjej, Walid Sellami, Aicha Rebai, Jamel Zaoueli, Ridha Mrissa, Hedi Gharsallah, Iheb Labbene, Mustapha Ferjani

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