Dravet syndrome is a severe type of epilepsy characterized by prolonged seizures, often triggered by fever, that begin in the first year of life.
Prolonged seizures, or a series of shorter epileptic episodes close together, is associated with a state known as status epilepticus, which can be life-threatening.
What is status epilepticus?
Status epilepticus is a term that describes epileptic episodes in which either an individual seizure lasts longer than five minutes, or many seizures occur close together without the patient recovering between them. Status epilepticus is common in Dravet syndrome.
There are two types of status epilepticus: nonconvulsive and convulsive.
Patients with nonconvulsive status epilepticus may appear confused or look as though they are daydreaming. They may be unable to respond to external stimuli, or they may behave irrationally.
Convulsive status epilepticus is more likely to lead to long-term injury due to the prolonged nature of the convulsions. Convulsive status epilepticus requires emergency treatment by medical professionals.
Prevention
Activities that may raise body temperature should be avoided; these can stimulate seizures. Anticonvulsant treatments can help reduce the incidence and severity of seizures, although complete seizure control is unlikely.
A small study published in the journal Seizure reported that a ketogenic diet may improve overall seizure incidence in Dravet syndrome patients.
For this study, 20 patients were placed on a ketogenic diet for six months. Seventeen experienced at least a 50 percent reduction in seizure frequency and, among these 17 responders, no generalized convulsions lasting five or more minutes or episodes of  status epilepticus were reported during the six months.
Treatment
Emergency treatment for status epilepticus is essential, and typically consists of anticonvulsant therapy. In extreme cases, anesthetics may be used to chemically induce a comatose state to stop the seizure or seizures. Continuous EEG (electroencephalogram) monitoring may be needed to monitor seizures and response to treatment.
It is important to have the patient’s emergency seizure management plan at hand when speaking to emergency responders, so that they know which medications the patient is taking and those that should not be used.
Prognosis
Status epilepticus is the second highest cause of mortality in Dravet syndrome patients, following sudden unexpected death in epilepsy (SUDEP).
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