Dravet syndrome is a rare and severe type of epilepsy. First appearing in early infancy, it is characterized by frequent and treatment-resistant seizures, which can be triggered by warm weather, fevers, or sunlight.
These seizures are usually long in duration (30 minutes or more) and highly resistant to many existing medications and treatments for epilepsy.
Seizure types in Dravet syndrome
Seizure types caused by Dravet syndrome are usually tonic-clonic (also called convulsive) involving muscle jerks or other body movements. They may be whole-body convulsive seizures, such as grand mal seizures, or can be hemiclonic seizures that affect only one side of the body.
In the second year of life, seizure types can also include absence seizures (staring spells) and myoclonic seizures (brief jerking or twitching of muscles), as well as other types of partial seizures.
Seizures lasting more than than five minutes are called status epilepticus. These types of seizures are life-threatening and require urgent medical treatment.
The sooner emergency care is given, which may include oxygen, breathing support, and intravenous fluid, the better the outcome.
How Dravet syndrome affects infants and children
Due to the frequency of the seizures in Dravet syndrome, some cognitive and physical problems are likely to occur.
Children often experience movement and balance problems and exhibit poor growth, and problems with bone structure and nutrition. Language and speech skills may be delayed, and troubled sleep is a frequent complaint. Many children also develop sensory processing problems, such as sensory integration disorder or symptoms that resemble autism.
Developmental delays are common, as are abnormal electroencephalogram (EEG) readings, especially in the child’s second year of life.
However, there is a spectrum of severity, and some children with Dravet syndrome have more easily controlled seizures and experience fewer developmental problems.
Dravet syndrome outcomes
Dravet syndrome has a 15-20 percent mortality rate in children younger than 18 due to SUDEP (sudden unexpected death in epilepsy). Accidents related to seizures, such as drowning, and infections due to a weakened immune system can also cause death.
However, most children with Dravet syndrome survive into adulthood with appropriate care and support, even though cognitive and physical impairment, as well as continued episodes of seizures, continue.
While there is no cure for Dravet syndrome, early recognition can help reduce some of the problems caused by the disorder, as well as limit the impact of other health concerns the child might have. In some cases, although rare, children stabilize and improve after age 6.
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