Children and Adults with Dravet syndrome experience multiple seizure types that are resistant to most anti-epileptic medications. Currently, the evidence supports the use of “rational polytherapy” which consists of a step-wise introduction of medications that have been shown to improve seizure control in patients with Dravet syndrome until the patient either responds favorably or experiences unacceptable side effects. It must be emphasized that significant differences exist between countries with regard to drug dose preferences and availability of anti-epileptic medications.

 

Most experts agree that the following medications have been shown to benefit patients with Dravet syndrome:

 

The following medications may aggravate seizures in Dravet syndrome:

        •  lamotrigine (Lamictal)

        •  phenytoin (Dilantin, Epanutin)

        •  fosphenytoin (Cerebyx, Prodilantin)

        •  carbamazepine (Tegretol, Calepsin, Cargagen, Barbatrol, Epitol, Finlepsin, Sirtal, Stazepine)

        •  oxcarbazepine (Trileptal)

        •  vigabatrin (Sabril, Sabrilan, Sabrilex)

 

Further study is needed on the following therapies before recommendations for use can be made:

 

Non-pharmacologic therapy with the ketogenic diet has been shown to improve seizure control in a significant percentage of children with Dravet syndrome.

 

Focal resective surgery is usually not helpful as SMEI is a systemic disorder without identifiable focal pathology.

 

 

WHOLE PATIENT CARE

 

Dravet syndrome is a systemic disorder affecting the whole person. A number of co-morbid conditions appear to be commonly associated with Dravet syndrome and are currently under further investigation. Preliminary data suggests that patients with Dravet syndrome may be at increased risk for:

        •  Orthopedic conditions, including but not limited to foot deformities, scoliosis and “crouch gait”.

        •  Impaired immune response with chronic upper respiratory infections and otitis media.

        •  Sensory integration disorders and other features consistent with the autism spectrum.

        •  Growth and nutritional issues.

        •  Autonomic dysfunction, including issues with temperature regulation, decreased sweating, intermittent tachycardia and slowed GI motility.

 

Virtually all patients with Dravet syndrome will experience some degree of developmental delay. Developmental assessments should begin as early as possible and should be repeated regularly. Early implementation of aggressive global therapies is essential to support optimal development. Patients with Dravet syndrome should receive physical, occupational, speech, and social/play therapies and an enriched environment is encouraged.