An acute encephalopathy with inflammation-mediated status epilepticus characterised by an acute refractory status epilepticus, typically of the tonic-clonic type, following prodromal symptoms of confusion, fever, fatigue, headache, symptoms of gastrointestinal or upper respiratory tract infection, behavioural changes or hallucinations. Brain MRI abnormalities and abnormal findings in cerebrospinal fluid, including pleocytosis and/or elevated protein levels, are frequently found during acute episode. Treatment-resistant epilepsy, cognitive and psychiatric impairments are usual consequences. [from
SNOMEDCT_US]