The impact of induced burst suppression on outcomes in patients with POLG-related status epilepticus.

Purpose: Management of status epilepticus in patients with POLG-related disease is challenging as many patients with POLG-related status epilepticus progress to refractory or super-refractory status epilepticus, often with fatal outcome. We aimed to study the impact of induced burst suppression on seizure control and survival in patients with POLG-related refractory status epilepticus, describe the extent of anaesthetic agents needed to induce burst suppression, and the potential side-effects of long-term anaesthesia.

Methods: A total of 15 trials of induced burst suppression in six patients with genetically confirmed POLG-related disease were analysed. Time points of status epilepticus start, intubation, induction of burst suppression, extubation and death, together with the total length of burst suppression, and details on anaesthetic agents and anti-seizure medications used during burst suppression induction were systematically collected.

Results: Rapid induction of burst suppression (< 24 h) from the onset of status epilepticus showed a mean survival time of 171.3 days, versus 140.7 days when burst suppression was induced after 24 h. Patients with burst suppression lasting <48 h had a mean survival of 238 days, whereas those with burst suppression lasting >48 h had a mean survival time of 161.7 days. The induction of burst suppression required multiple anaesthetic agents. We did not find any evidence of propofol-infusion syndrome.

Conclusion: Treatment of POLG-related status epilepticus is extremely challenging and has a major impact on survival. The role of early and prolonged burst suppression in treatment of refractory status epilepticus in patients with POLG-related disease should be further studied.