Developmental Malformations
Aicardi-Goutieres syndrome
Mar. 22, 2024
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Myoclonic status epilepticus in the setting of renal failure
This 65-year-old man had a history of bilateral kidney transplants complicated by autoimmune rejection. He presented on 2 occasions with obtunded mental status and frequent myoclonic jerks lasting more than 24 hours in the setting of acute or chronic renal failure. On exam, the patient had both synchronous and asynchronous myoclonic jerks predominantly affecting the upper extremities. Video-EEG demonstrates moderate diffuse background slowing and frequent central sharp waves. The patient’s myoclonus and mental status improved following dialysis. (Video-EEG courtesy of Dr. Sara Inati.)