POLG-related mitochondrial disorders
Sep. 12, 2024
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Postanoxic myoclonic status epilepticus
This 86-year-old woman with a history of atrial fibrillation suffered a cardiac arrest of unclear etiology and duration. On examination 18 hours after resuscitation, the patient was in coma with intact brainstem reflexes. She demonstrated frequent rhythmic movements of her facial muscles, including eyelid twitching tongue and jaw protrusion. EEG background is diffusely slow. Myoclonic movements recur every 3 to 5 seconds corresponding to 4 to 7 Hz centrally predominant, generalized spike-wave activity lasting 3 to 5 seconds. The movements did not respond to levetiracetam but abated to some degree with benzodiazepines. Epileptiform discharges and myoclonus resolved within 72 hours. SSEPs performed 3 weeks after resuscitation demonstrated intact cortical responses. The patient died 6 weeks after presentation due to a combination of progressive neurologic decline and renal failure. (Video-EEG courtesy of Dr. Sara Inati.)