Prager, C; Cross, JH; (2017) Management of Dravet syndrome and emerging therapy options. Expert Opinion on Orphan Drugs , 5 (3) pp. 219-227. 10.1080/21678707.2017.1281124.

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Abstract

Introduction: Dravet syndrome is an early onset epileptic and genetic encephalopathy. Although a well-recognised electroclinical picture is key to diagnosis, a mutation in the SCN1A gene is found in excess of 80%. In general prognosis is poor overall for seizure control and neurodevelopment. With careful phenotyping of the condition and review of treatment response we have become aware of the antiepileptic drugs that are most likely to help, but more importantly drugs that may lead to deterioration and consequently to avoid. Despite this, a significant number of children continue to have seizures and there is little evidence to suggest that we have substantially changed the prognosis in these patients. Areas covered: This review explores current views on the diagnosis and treatment of Dravet syndrome. Consideration is given to the underlying genetics, as well as agents proving to be useful in the management of seizures, including those currently under trial. This has been prepared utilising a review of current literature, as well as expert opinion. Expert opinion: Considerable experience in the field has led to a knowledge of optimised treatment in this condition. This involves both acute and maintenance treatment. Despite this, there has been little impact on overall outcomes. Newer treatments now provide promise with regard to seizure control; whether this will have an impact on overall outcome remains to be seen.

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