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New-onset refractory status epilepticus (NORSE) is a recently defined clinical entity which describes patients who present with severe generalized seizures of unclear etiology that occur in the context of a preceding febrile illness.1–3 These seizures are typically unresponsive to antiepileptic medications.
Case report.
A 26-year-old otherwise healthy right-handed man presented with generalized tonic-clonic seizures after a 1-week history of headache, fever, and myalgias. He was admitted to our intensive care unit for treatment and underwent continuous EEG monitoring for 77 days. On admission to our intensive care unit, the CSF contained 92 × 106 white blood cells (96% lymphocytes). Consistent with previous reports of NORSE, an exhaustive structural, infectious, toxic/metabolic, autoimmune, neoplastic, and paraneoplastic workup did not identify a clear underlying etiology (figure, A). IV infusions of propofol or thiopental were used to maintain the patient in either an electrographic burst suppression pattern or electrographic silence, while enteral antiepileptic medications were titrated to therapeutic doses (e.g., phenytoin, valproic acid, clobazam, topiramate, vigabatrin, phenobarbital, and levetiracetam) (figure, B). Attempts to wean IV antiepileptic drugs were met with recurrence of electrographic multifocal sharp transients, originating from either temporal lobe, which evolved into clinical and electrographic seizures.
Figure Laboratory investigations, imaging, and pathologic findings in a case of new-onset refractory status epilepticus
(A) Table of investigations performed to identify the underlying etiology of the …
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