Adjunctive transcranial DC stimulation in the management of refractory drug-resistant focal epilepsy in POLG mitochondrial disease: A case series.

BACKGROUND

Pathogenic POLG variants produce an age-related progressive multi-system mitochondrial disorder, with neurologic manifestations that include focal onset seizures, stroke-like episodes, cerebellar ataxia, sensory neuronopathy, complex ophthalmoplegia and myopathy. Focal onset status epilepticus or epilepsia partialis continua (EPC) frequently occurs in patients with paediatric and early adulthood onset disease.

METHODS

Between 2014 and 2020, we treated 15 episodes of drug-refractory focal epileptic status (epilepsia partialis continua) in 5 patients with POLG-related mitochondrial disease with cathodal transcranial direct current stimulation (tDCS). Here we describe the management of each episode of EPC, including the application of tDCS.

RESULTS

Transcranial DCS, delivered for 20 min at 2 mA, once daily (minimum 3 days; maximum 14 days), as an adjunct to optimal standard medical care, was associated with a significant reduction (93% of episodes after 4.3 sessions) or a cessation of seizures (63% of episodes after 8.4 sessions). No major side effects of tDCS were noted.

CONCLUSION

Our findings support further exploration of tDCS as a potential adjunctive therapy for complex neurological challenges in mitochondrial disorders.