title: Treatment responsive GABA(B)-receptor limbic encephalitis presenting as new-onset super-refractory status epilepticus (NORSE) in a deployed U.S. soldier.
abstract: A 23-year-old, previously healthy, deployed U.S. soldier presented with bilateral temporal lobe seizures recalcitrant to multiple antiepileptic drugs and anti-seizure anaesthetic agents. He received methylprednisolone, intravenous immunoglobulins, plasma exchange, and rituximab for presumed autoimmune encephalitis before achieving seizure freedom. Six weeks after presentation, the aetiology of his refractory seizures was found to be due to autoantibodies targeting the anti-GABA(B)-receptor. This case is noteworthy for being the first reported case of anti-GABA(B)-receptor limbic encephalitis presenting with new-onset refractory status epilepticus (NORSE), a clinical syndrome that often carries a grave prognosis and in which a treatable aetiology is often never discovered. Our case also supports testing for GABA-receptor autoantibodies and the upfront use of multi-modal immunotherapy in patients presenting with limbic encephalitis and new refractory seizures.
authors.affiliation: Walter Reed National Military Medical Center, Department of Neurology, Uniformed Services University of Health Sciences Department of Neurology.
authors.forename: Jeffrey Brian
authors.initials: JB
authors.lastname: Hainsworth
authors.affiliation: Walter Reed National Military Medical Center, Department of Internal Medicine, Uniformed Services University of Health Sciences Department of Medicine.
authors.forename: Akira
authors.initials: A
authors.lastname: Shishido
authors.affiliation: Walter Reed National Military Medical Center, Department of Neurology, Uniformed Services University of Health Sciences Department of Neurology, Walter Reed National Military Medical Center, John P. Murtha Cancer Center, Bethesda, Maryland, USA.
authors.forename: Brett James
authors.initials: BJ
authors.lastname: Theeler
authors.affiliation: Walter Reed National Military Medical Center, Department of Neurology, Uniformed Services University of Health Sciences Department of Neurology.
authors.forename: Craig Grason
authors.initials: CG
authors.lastname: Carroll
authors.affiliation: Walter Reed National Military Medical Center, Department of Neurology, Uniformed Services University of Health Sciences Department of Neurology.
authors.forename: Rebecca Ellen
authors.initials: RE
authors.lastname: Fasano
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D004569:Electroencephalography; D006801:Humans; D020363:Limbic Encephalitis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008889:Military Personnel; D018080:Receptors, GABA-B / Q000276:immunology*; D013226:Status Epilepticus / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D018080:Receptors, GABA-B
keywords: GABA(B)-receptor; all epilepsy/seizures; encephalitis; new-onset refractory status epilepticus (NORSE); status epilepticus