Intravenous immunoglobulin and febrile status epilepticus in children with Dravet syndrome: A retrospective multicentre study.

AIM: To assess the efficacy and tolerability of intravenous immunoglobulin (IVIG) in reducing febrile status epilepticus in children with Dravet syndrome.

METHOD: We conducted a retrospective multicentre study across seven French university hospitals (2005-2022). Children with genetically confirmed Dravet syndrome who received sequential IVIG were included. Clinical data were collected over two 6-month periods: before and after IVIG initiation.

RESULTS

Fourteen individuals (six males, eight females) were included. At IVIG initiation, all were in the stormy phase, aged 10 to 92 months, and receiving a median of four antiseizure medications. IVIG was administered every 1 to 6 weeks (0.3-0.5 g/kg per infusion). Hospitalizations for status epilepticus significantly decreased, from a median of 4 (range 0-16) at baseline to 1 (range 0-6) after treatment (p = 0.002). Twelve individuals improved, two remained stable. Adverse events occurred in 6 out of 14 individuals, including infusion-related fever or seizures. Central venous access was required in six cases. IVIG was continued beyond 6 months in 11 out of 14 individuals.

INTERPRETATION: These series suggest a potential benefit of IVIG in reducing status epilepticus in selected children with Dravet syndrome. However, tolerability and feasibility issues were identified. A prospective controlled trial is warranted to further define the role of IVIG in this population.