Dravet Syndrome

Many people have asked what Dravet syndrome is, so the long story is...

Dravet Syndrome, also known as Severe Myoclonic Epilepsy of Infancy (SMEI), is a progressive childhood neurodevelopmental disorder characterized by severe epilepsy that does not respond well to treatment. Estimates of the prevalence of this rare disorder have ranged from 1:20,000 to 1:40,000 births, though incidence may be far greater as new genetic evidence is discovered. It occurs more frequently in boys than in girls, but knows no geographic or ethnic boundaries.

The course of Dravet Syndrome is highly variable from child to child. It begins in the first year of life. Development is normal prior to the onset of seizures. In most cases the first seizures are correlated with fever. These seizures are often prolonged. In time seizures increase in frequency and become more likely to occur without fever. Additional seizure types may appear.

During the second year of life, progressive regression of aquired skills and developmental delays are usually observed to varying degrees and additional neurological symptoms. Additional features that are seen in most children with Dravet Syndrome are poor regulation of body temperature and increased susceptibility to infection. For a significant number of these children secondary problems can also include sleep disturbance, slowed physical growth, movement disorders, and orthopedic disorders.

At this time, the treatments available for Dravet Syndrome are to improve symptoms, primarily anticonvulsant medications to control seizures. The seizures are very resistant to therapy and the response to different medicines can be highly variable from child to child. Certain medicines have been found to be the most useful for most individuals with Dravet Syndrome, a few others have been quite consistently found to have an aggravating effect.

Social Development: One way that many parents first become aware of delays in social development is when their children do not demonstrate the expected stranger anxiety or seperation anxiety that most typical children experience between the ages of one and three. Parents note that their children with Dravet syndrome lack a sense of appropriate social boundaries. They do not fear strangers and, in fact may be excessively affectionate toward strangers. Children with Dravet syndrome may have difficulty learning to imitate others’ behavior. Many tend toward solitary play and may engage in parallel play, but cooperative play is difficult and many do not understand turn-taking. Their ability to understand the concepts of ownership, belonging and others’ points of view may be difficult to assess, but based on parent reports, difficulties in these areas are common. Individuals with Dravet syndrome are likely to remain dependent on others for assistance with self-care skills such as toileting, dressing, and eating, often into adulthood.

Behavioral Development: Ninety-five percent of the parents report that their children had some unusual behaviors. Those most frequently reported were perservation or repetitive behaviors, flapping or clapping of hands, obsessions, excessive stubbornness, and excessive activity. Many parents also express concern for the safety of their children because of their tendency to wander.

This is taken off of the IDEA league web site.

It is one of the most severe epilepsy syndromes.