What is Dravet Syndrome, and how is it managed in South Korea?

Dravet Syndrome is a rare, severe form of epilepsy that typically begins in infancy. It is characterized by prolonged seizures, developmental delays, and increased risk of sudden unexplained death in epilepsy (SUDEP). The condition is most often caused by mutations in the SCN1A gene, which affects the function of sodium channels in the brain, leading to hyperexcitable neurons and recurrent seizures.

In South Korea, awareness and management of Dravet Syndrome have been improving over recent years. Early diagnosis is critical, as timely intervention can reduce seizure frequency and improve long-term outcomes. Diagnostic approaches include genetic testing to identify SCN1A mutations, EEG monitoring to evaluate seizure patterns, and comprehensive clinical assessment.

Treatment in South Korea primarily focuses on seizure control and supportive care. Anti-seizure medications such as valproate, clobazam, and stiripentol are commonly used, while sodium channel blockers like carbamazepine are typically avoided because they may worsen symptoms. In addition to medication, dietary therapies such as the ketogenic diet, and adjunctive therapies including physical, occupational, and speech therapy, are employed to support development and quality of life.

South Korea has also seen increased access to specialized epilepsy centers and patient support networks, providing families with educational resources, genetic counseling, and psychosocial support. Research initiatives are ongoing to explore novel therapies, including gene-targeted treatments and new anti-seizure drugs.

Overall, the management of Dravet Syndrome in South Korea combines early diagnosis, careful selection of treatments, and multidisciplinary support to improve patient outcomes and quality of life.