Unusual Case of Acute Decompensated Maple Syrup Urine Disease in the Emergency Department.

A case report of an army soldier presenting to the emergency department with acute metabolic derangement resulting in encephalopathy, cerebral edema, and death is presented. The patient had no medical diagnoses before entering military service and was triaged in the emergency department with the common complaint of presyncope. However, as encephalopathy worsened, the patient experienced altered mental status, lethargy, emesis, and seizure. This patient ultimately died because of rapid decompensation. Maple syrup urine disease pathophysiology and treatment recommendations are reviewed.