The adjunctive application of transcranial direct current stimulation in the management of de novo refractory epilepsia partialis continua in adolescent‐onset POLG‐related mitochondrial disease

Abstract

SummaryFocal status epilepticus in POLG‐related mitochondrial disease is highly refractory to pharmacological agents, including general anesthesia. We report the challenges in managing a previously healthy teenager who presented with de novo epilepsia partialis continua and metabolic stroke resulting from the homozygous p.Ala467Thr POLG mutation, the most common pathogenic variant identified in the Caucasian population. We applied transcranial direct current stimulation (tDCS; 2 mA; 20 min) daily as an adjunctive therapy because her focal seizures failed to respond to five antiepileptic drugs at maximal doses. The electrical and clinical seizures stopped after 3 days of tDCS. The second course of tDCS was administered for 14 days when the focal seizures re‐emerged a month later. The patient tolerated the procedure well. Following 4 months of hospitalization and prolonged community rehabilitation, our patient has now returned to full‐time education with support, and there is no report of cognitive deficit. We have demonstrated the safety and efficacy of tDCS in treating refractory focal motor seizures caused by mitochondrial disease.

No effect of electrical transcranial direct current stimulation adjunct treatment for epilepsia partialis continua in POLG disease

“…We therefore conclude that – in this case study – tDCS did not have a beneficial treatment effect on treatment-resistant refractory epilepsia partialis continua in POLG-related mitochondrial disease. Hence, our results are inconsistent with those of Ng et al [6], who found that seizures stopped completely in a similar case study.…”

“…A study found mitochondrial dysfunction in one third of patients with epilepsy that underwent metabolic testing [5], emphasizing that drug-resistant seizures are a frequent problem in mitochondrial disease, and that new treatments need to be developed. In a previous case report, focal seizures in a patient with POLG-related mitochondrial disease ceased after two weeks of transcranial direct current stimulation (tDCS) [6]. Since these seizures are often refractory to medical treatment and the technique is non-invasive, we tested tDCS using similar parameters as in Ng et al [6] in a patient with POLG-related mitochondrial disease and drug-resistant multifocal epilepsy.…”

“…In a previous case report, focal seizures in a patient with POLG-related mitochondrial disease ceased after two weeks of transcranial direct current stimulation (tDCS) [6]. Since these seizures are often refractory to medical treatment and the technique is non-invasive, we tested tDCS using similar parameters as in Ng et al [6] in a patient with POLG-related mitochondrial disease and drug-resistant multifocal epilepsy.…”

Computational modelling of the long-term effects of brain stimulation on the local and global structural connectivity of epileptic patients

“…Moreover, this phenomenon may be able to explain some of the unexpected long-term effects of TCS that appear in parts of the brain that were not stimulated. An example of this phenomenon is presented in [67], where seizures reoccur starting from a different brain region a month after an initially successful application of TCS.…”

Polymerase gamma-relatert mitokondriesykdom

“…Epilepsia partialis continua er vanligvis behandlingsrefraktaer. Transkraniell likestrømsstimulering kan forsøkes (14), men vi har ikke kunnet bekrefte effekt. Mange forsøker ketogen diett, men det er ikke vist effektivt i denne sammenheng.…”