Our case highlights the progressive nature of POLG-related mitochondrial disease, the overlap of clinical syndromes and difficulty of predicting the trajectory of disease progression, and the management challenge of refractory mitochondrial epilepsy.4 The presence of focal onset motor status, together with the acute stroke-like lesions, is likely related to the neuronal energy failure6 of which inhibitory interneurons have been shown to be particularly vulnerable to mitochondrial dysfunction.7 The gene discussed is POLG; the disease is mitochondrial disease.