New-Onset Refractory Status Epilepticus (NORSE)
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report New-Onset Refractory Status Epilepticus (NORSE) is not the name you expected.
New-onset refractory status epilepticus (NORSE) is defined as refractory status epilepticus without an obvious cause after initial investigations; "initial" typically refers to 1-2 days, which is adequate time to rule out strokes, brain masses, drug overdoses, and herpes encephalitis. Refractory status epilepticus (SE) is a condition in which patients suddenly experience continuous seizures or a flurry of very frequent seizures (the definition of "SE") that do not respond to standard anticonvulsant medications (the definition of "refractory"). Seizures are thought to be due to an excess of pro-inflammatory molecules in the brain, perhaps triggered by a simple viral infection, although no clear cause has ever been demonstrated. Affected individuals are most often treated for weeks in an intensive care unit because they require prolonged anesthesia with coma-inducing drugs to control their seizures. NORSE carries a high rate of complications and mortality, but a significant proportion of patients do eventually recover. Epilepsy (a life-long predisposition to unprovoked seizures) and cognitive issues are common among survivors although a small minority of them eventually return to a normal lifestyle.
Refractory status epilepticus most commonly complicates an acute brain injury (stroke, trauma, infection, etc.) or a serious medical illness. It may also occur in people with a chronic seizure disorder. In a significant minority of cases, however, refractory SE strikes out of the blue and no cause can be identified. These cases are known as cryptogenic new-onset refractory SE, or NORSE.
NORSE has been reported under different names, including:
•cryptogenic new-onset refractory status epilepticus (cryptogenic NORSE)
•presumed encephalitis with refractory status epilepticus
In addition, several epilepsy syndromes in infants and children share many similarities with NORSE. It is not clear whether they are different age-related manifestations of the same disease or separate entities, but all these syndromes have in common an acute onset, a prolonged course of refractory SE, and inflammatory features in the CSF. These related disorders include:
•febrile illness-related epilepsy syndrome (FIRES; see separate NORD report)
•idiopathic hemiconvulsion-hemiplegia and epilepsy syndrome (IHHES)
•devastating epilepsy in school-aged children (DESC)
•acute encephalitis with refractory, repetitive partial seizures (AERRPS).
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Last Updated: 4/11/2016
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