Dravet Syndrome

Dravet syndrome is a rare and severe form of epilepsy that manifests before the age of five. The syndrome is characterized by fever-related seizures in a very young child, followed by various other types of seizures as the person ages. In many cases, Dravet syndrome is caused by a mutation of the gene that regulates proper development and function of the brain cells.

Children with Dravet syndrome usually have developmental and social delays, especially related to language and motor skills. They are also prone to hyperactivity, sleep disorders, growth and nutrition issues, and chronic upper respiratory infections. As these children get older, cognitive function often declines. During the second to fourth year of life, some children with Dravet syndrome will lose previously acquired skills. Many teenagers with Dravet syndrome requiring constant medical care. However, prognosis can vary. Many children with Dravet syndrome stabilize after age four.

Dravet syndrome sufferers are more likely to also have autism spectrum disorders, orthopedic disorders, and frequent ear infections. Though there is no cure for Dravet syndrome, the seizures it causes are treated with medication and in some cases, a special diet. Unfortunately, in the worst cases of Dravet syndrome the seizures are resistent to medication. Children with Dravet syndrome are also more likely to suffer from sudden unexplained death in epilepsy.

Dravet syndrome is rare, occuring in approximately 1 in 20,000 to 40,000 births. Though the cause is unknown, mutation of the SCN1A gene is thought to contribute to 50 to 80 percent of cases of Dravet syndrome.

 

 

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