Progressive Myoclonic Epilepsy: A Typical Case of MERRF and the Lessons Learnt

Mitochondria are referred to as the energy centers of cells, and mitochondrial DNA are known to alter the mitochondrial respiratory chain, which reduces cellular energy and causes ion-channel malfunction and neuronal cell death. As majority of the cells contain mitochondria, these disorders involve multiple systems. Since the energy requirements of different tissues vary, their vulnerability to mitochondrial dysfunction also varies in terms of threshold. This causes these disorders to appear clinically in a variety of ways and with significant phenotypic overlap. The genotypic and phenotypic definitions of some specific symptoms are, nevertheless, well established. Potentially deadly mitochondrial toxins are present in some epileptic medications and early diagnosis of the condition can help prevent serious morbidities. We describe a typical case of sodium valproate and lamotrigine-induced fast multiaxial worsening in a patient with myoclonic epilepsy with red ragged fibers (MERRF) as these drugs act as mitochondrial poisons and could prove life threatening for patients with mitochondrial diseases. When mitochondrial cocktail was introduced and the problematic medicines were removed, the symptoms were somewhat reversed. MERRF in progeny of non- consanguineous parents is a rare occurrence resulting in poor prognosis of the disease. Epileptic drugs lamotrigine and sodium valproate should be avoided as these drugs worsen myoclonus and cause mitochondrial toxicities. With knowledge of the information in this scenario, further harm to the patient can be prevented.