First appearing in early infancy, usually in the first year of life, Dravet is characterized by such seizures, which can be triggered by warm weather, fevers, or sunlight.
These seizures usually are long in duration — often more than 5 minutes — and highly resistant to many existing medications and treatments for epilepsy.
Seizure types in Dravet syndrome
Seizure types caused by Dravet syndrome usually are 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 hemiclonic, which affect only one side of the body.
Between the ages of 1.5 and 5, seizure types also can include absence seizures, which include staring spells, and myoclonic seizures, usually involving brief jerking or twitching of muscles. Other types of partial seizures also may occur.
When seizures last for more than five minutes, or when they occur so close together that the patient does not recover between them, they are called status epilepticus. These types of seizures are life-threatening and require urgent medical treatment, which may include oxygen, breathing support, and intravenous (into-the-vein) fluid.
The sooner emergency care is given, the better the outcome.
Due to the frequency of seizures in Dravet syndrome, some cognitive and physical problems are likely to occur.
Children often experience movement and balance problems and exhibit poor growth, oftentimes with 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 and third years of life.
Dravet syndrome outcomes
Due to seizures, some individuals may be at a higher risk for SUDEP, or sudden unexpected death in epilepsy. This may occur in up to 1% of people with Dravet each year, and more so in those with poorly controlled seizures.
Although it is not possible to prevent SUDEP, its risk can be reduced by improving seizure control and decreasing the frequency of generalized tonic-clonic or convulsive seizures, especially at night.
Other accidents related to seizures, such as drowning, also may occur. Infections due to a weakened immune system also are possible.
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 children, as they get older, the decrease of cognitive function stabilizes, or may improve slightly.
Last updated: June 4, 2021
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