Our results emphasize screening of the common POLG mutations and POLG sequencing in any child or adolescent who presents with intractable seizures and at least one raised CSF lactate (or brain magnetic resonance spectroscopy lactate) or suggestive brain MRI changes (with thalamic predominance) with or without status epilepticus, epilepsia partialis continua or liver manifestations typical for Alpers disease, especially when the disease course is progressive. The gene discussed is POLG; the disease is status epilepticus.