Dravet syndrome is a disease characterized by prolonged and frequent seizures that begin in the first year of life and are difficult to control with medication. These seizures are harmful to the developing brain and can cause cognitive and behavioral impairment in patients.
As treatments improve, seizures are better controlled in new patients who may be spared some of the neurological damage seen in earlier generations of Dravet syndrome.
An estimated 10-20 percent of Dravet syndrome patients pass away before reaching adulthood. The majority of these deaths are due to SUDEP (sudden unexpected death in epilepsy patients) and status epilepticus (SE). SE is a single seizure lasting more than five minutes in length, or several seizures very close together with no recovery in between the episodes.
Dravet syndrome patients also are at risk of death caused by a seizure-related accident such as drowning or infections.
The management of seizures and medical interventions in emergencies can reduce the risk of death associated with Dravet syndrome.
Seizures also tend to become less frequent and severe once a Dravet syndrome patient reaches adulthood; many children diagnosed with the disease have a normal life expectancy.
The types and timing of seizures that children with Dravet syndrome experience may change over time. Around age 4, many patients experience fewer episodes of SE (though they remain a risk), but the frequency of seizures may increase. Patients may start getting a warning of an impending seizure called an “aura.” Seizures may happen less often during the day, but become more common during sleep.
Most children with Dravet syndrome show a slowing of their development beginning around age 2. The severity of developmental delays varies and there are reports of normal development in some children with Dravet syndrome. Patients also may experience movement and balance problems, growth or nutrition issues and, chronic infections.
There are many other conditions that often co-occur with Dravet syndrome. These include autism spectrum disorder (ASD), attention deficit hyperactivity disorder (ADHD), and dysautonomia — problems with autonomic nervous system functions such as regulation of body temperature, heart rate, and digestion.
A review of the long-term outcome for Dravet syndrome patients found that the majority of adults with the condition cannot live independently and require either partial or considerable support. However, as treatment methods improve along with the understanding of the disease, some researchers expect that long-term outcomes will improve.
While there is no cure for Dravet syndrome, the genetic cause of most cases of the disease is known, which provides hope that researchers may find a way to address this cause. In the meantime, several pharmaceutical companies and other research groups are developing new treatments for the symptoms of the disease, which can improve quality of life for patients.
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