Physician-Family Panel Recommends Actions to Better Diagnose, Treat Dravet Syndrome

Physician-Family Panel Recommends Actions to Better Diagnose, Treat Dravet Syndrome

Thirteen specialists in epilepsy and Dravet syndrome, along with five family members of children with the disease, have jointly developed a set of recommendations for better diagnosis and management of the illness.

Their recommendations are contained in the report, “Optimizing the Diagnosis and Management of Dravet Syndrome: Recommendations From a North American Consensus Panel,” which appeared in the journal Pediatric neurology.

The experts and family members conducted the panel in three rounds of online questionnaires that aimed to identify consensual and contentious areas in diagnosing and managing Dravet – a type of childhood epilepsy characterized by prolonged seizures that may require emergency intervention.

Doctors usually manage Dravet with antiepileptic drugs, but these are not 100 percent effective at suppressing seizures completely. The syndrome is estimated to affect one in 40,000 to one in 20,000 children, according to an Epilepsy UK news story.

“We were able to identify areas where there was strong consensus that we hope will inform healthcare providers on optimal diagnosis and management of patients with Dravet syndrome, support reimbursement from insurance companies for genetic testing and Dravet syndrome-specific therapies, and improve quality of life for patients with Dravet syndrome and their families by avoidance of unnecessary testing and provision of an early accurate diagnosis allowing optimal selection of therapeutic strategies,” authors wrote.

The panel also reached consensus in the areas of clinical presentation of Dravet syndrome; range of electroencephalograms and magnetic resonance imaging (MRI) findings; genetic testing; priority for seizure control; seizure-triggering factors; requirement and indications for rescue therapy; and screening for diseases that may occur at the same time as Dravet, among other topics.

Consensus was not as strong regarding later therapies, including vagus nerve stimulation and callosotomy, and for specific therapies of related disease. And beyond the initial treatment with benzodiazepines and valproate, the panel could not agree on optimal management of convulsive status epilepticus in the hospital.

Linda Laux, MD, of Ann & Robert H. Lurie Children’s Hospital of Chicago, recently spoke about Dravet syndrome and current research in an educational video awareness series produced by the New Jersey-based Dravet Syndrome Foundation.

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