Hyperammonemia is a rare complication of lung transplant with a high mortality rate. It presents as encephalopathy and progresses to seizures, status epilepticus, coma, cerebral edema, and brain death. Multiple treatments have been documented including administration of medications, gut decontamination, and dialysis. However, no definitive treatments exist and mortality remains between 67% and 75%. We present the case of a 65-year-old male with idiopathic pulmonary fibrosis who developed refractory status epilepticus secondary to hyperammonemia following lung transplant. The patient presented on postoperative day 7 with super-refractory status epilepticus and normal computed tomography scan of the head. Hyperammonemia was suspected due to refractory seizures and confirmed with peak ammonia level >1000 μmol/L. Despite aggressive treatment, the patient developed global cerebral edema and died. Postmortem investigations revealed that the patient was positive for Ureaplasma parvum. Additional studies are needed to elucidate the exact mechanism of disease and investigate successful treatment options.
Keywords: Cerebral edema; hyperammonemia; lung transplantation; seizures; status epilepticus.