Dravet syndrome is a severe form of epilepsy that is part of a group of diseases known as SCN1A-related seizure disorders. The condition appears during the first year of life as frequent fever-related (febrile) seizures. As the condition progresses, other types of seizures typically occur, including myoclonus and status epilepticus. A family history of either epilepsy or febrile seizures exists in 15 percent to 25 percent of cases. Intellectual development begins to deteriorate around age 2, and affected individuals often have a lack of coordination, poor development of language, hyperactivity, and difficulty relating to others. Around 85% of Dravet syndrome cases are due to a mutation in the SCN1Agene, which is required for the proper function of brain cells. In about 10% of cases the cause is unknown but other genes are likely the cause. The main goal of treatment is to reduce seizures frequency and prevent status epilepticus.Moderate to severe cognitive impairment and intractable epilepsy into adulthood is common.
Last updated: 12/29/2016
What is the long-term outlook for people with Dravet syndrome?
People with Dravet syndrome require constant care, and the condition can severely impact the patient’s and family’s quality of life. About 10-20% of people with this condition are estimated to pass away before adulthood, with most premature deaths occurring before 10 years of age. The average age of death is about 8 years and ranges from infancy to 18 years of age. The most common cause of death is sudden unexpected death in epilepsy (SUDEP).
Last updated: 7/10/2017
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