A patient with recurrent episodes of complex partial status epilepticu
s and a distinctive pattern of periodic lateralized epileptiform disch
arges (PLEDs) is presented. The patient was subsequently shown to have
a mitochondrial disorder the MELAS type, a hitherto unreported associ
ation. The case illustrates that CPSE should be added to the list of p
ossible causes of acute neurological deterioration in MELAS patients.