ABSTRACT
Status epilepticus is a neurological emergency with unknown pathogenesis, controversial treatment options, and poor prognosis. In recent years, with the development of 18F-flurodeoxyglucose positron emission tomography ( 18F-FDG PET) imaging technology, further studies on status epilepticus have been carried out from the perspective of molecular metabolism. This article reviews the current role of 18F-FDG PET in patients with status epilepticus on etiology, disease activity, electric activity, location of epileptogenic foci, and prognosis evaluation.
ABSTRACT
New-onset refractory status epilepticus(NORSE)is a clinical syndrome,not a specific diagnosis of diseases in patients with new onset of refractive status epilepticus without active epilepsy or other preexisting relevant neurological disorders and is not associated with a clearly acute or active structrue,toxic or metabolic cause. Febrile in-fection-related epilepsy syndrome(FIRES)is a subcategory of NORSE,applicable for all ages,occurring in a prior fe-brile infection starting between 24 hours and 2 weeks before onset of refractory status epilepticus( RSE)with fever or not. Proposed consensus definitions are also applicable for infantile hemiconvulsion - hemiplegia and epilepsy syn-drome,RSE,super-RSE,prolonged RSE and prolonged super-RSE. These consensus definitions will promote much more effective communication and multicenter research,and ultimately improve recognition and treatment of these condi-tions.
ABSTRACT
@#To date, there are no clear guidelines for treatment of cryptogenic new onset refractory status epilepticus (NORSE) syndrome. Immunotherapy was effective for some patients, but the initiation time was often somewhat late. We experienced two cryptogenic NORSE syndrome patients with favorable outcomes with early immunotherapy. A 59 years old male patient and a 58 years old female patient visited our hospital in status epilepticus, who were later diagnosed as cryptogenic NORSE after full evaluation. Since seizures were not controlled by two or more antiepileptic drugs in both patients, immunotherapy began on the fifth day of hospitalization. There was improvement on EEG and clinical symptoms, one or two days after initiation of immunotherapy. Early diagnosis of NORSE and early immunotherapy may result in favorable outcome.
ABSTRACT
New-onset refractory status epilepticus(NORSE) is a clinical syndrome, not a specific diagnosis of diseases in patients with new onset of refractive status epilepticus without active epilepsy or other preexisting relevant neurological disorders and is not associated with a clearly acute or active structrue, toxic or metabolic cause.Febrile infection-related epilepsy syndrome(FIRES) is a subcategory of NORSE, applicable for all ages, occurring in a prior febrile infection starting between 24 hours and 2 weeks before onset of refractory status epilepticus(RSE) with fever or not.Proposed consensus definitions are also applicable for infantile hemiconvulsion-hemiplegia and epilepsy syndrome, RSE, super-RSE, prolonged RSE and prolonged super-RSE.These consensus definitions will promote much more effective communication and multicenter research, and ultimately improve recognition and treatment of these conditions.
ABSTRACT
@#New–onset refractory status epilepticus (NORSE) is a rare and challenging disease entity that is associated with high morbidity and mortality. Among the NORSE cases with a proven aetiology, autoimmune encephalitis has been identified as a leading cause. Here we describe a young man who presented with NORSE and was eventually found to have serum autoantibodies against Neurexin-3a, a synaptic molecule that has recently been implicated as a target in the pathogenesis of a novel form of autoimmune encephalitis. The patient’s seizures responded to plasmapheresis, though the patient eventually died from sepsis. This case shows that Neurexin-3a encephalitis is a possible cause of NORSE, and plasmapheresis may be an effective treatment.