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What is Dravet syndrome?

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Dravet syndrome is a rare genetic disease of the brain that causes seizures in young children, explains the National Organization for Rare Disorders. Mutations in a single gene cause about 80 percent of all cases of Dravet syndrome.

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Dravet syndrome is a severe disorder, and the seizures it causes are very resistant to treatment, states the National Organization for Rare Disorders. Multiple anti-seizure drugs must be used to control symptoms, and some epilepsy medications make the seizures from Dravet syndrome worse. Seizures related to Dravet syndrome often last more than five minutes, and sometimes last more than 30 minutes. Initially, the seizures are associated with fevers, but they can occur without fever as the condition develops. Surgery is usually ineffective for treating Dravet syndrome.

Most children with Dravet syndrome suffer developmental delays that are evident by 2 years of age, according to the National Organization for Rare Disorders. Additionally, children with the disorder often walk abnormally or suffer tremors. Acquired skills may be lost as Dravet syndrome progresses. Over time, the effects of the seizures can cause bone problems due to abnormal posture and movement. About 20 percent of people with Dravet syndrome die before the age of 20 due to complications from the disease.

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