A 2-year-old boy with no chronic medical conditions is brought to the emergency department after he was found unresponsive with vomitus in his mouth. The patient has had muscle aches, cough, rhinorrhea, diarrhea, vomiting, and fever for the past 3 days. His parents gave him aspirin twice daily for symptom control. Vital signs are within normal limits. On examination, the patient is somnolent but withdraws from pain. Pupils are equal in size and reactive to light. Cardiopulmonary examination shows no abnormalities. Hepatomegaly is present. Extremities are well perfused. Laboratory results are as follows: Over the next day, the patient's condition deteriorates and he dies despite supportive care. Examination of the brain would most likely reveal which of the following findings?

Question

A 2-year-old boy with no chronic medical conditions is brought to the emergency department after he was found unresponsive with vomitus in his mouth.  The patient has had muscle aches, cough, rhinorrhea, diarrhea, vomiting, and fever for the past 3 days.  His parents gave him aspirin twice daily for symptom control.  Vital signs are within normal limits.  On examination, the patient is somnolent but withdraws from pain.  Pupils are equal in size and reactive to light.  Cardiopulmonary examination shows no abnormalities.  Hepatomegaly is present.  Extremities are well perfused.  Laboratory results are as follows:  Over the next day, the patient's condition deteriorates and he dies despite supportive care.  Examination of the brain would most likely reveal which of the following findings?

Quizplus · Accepted Answer

11ec160b_3b0e_f570_9249_53aad77a3c7b_MD0001_00 This patient had acute-onset encephalopathy and liver failure (eg, hepatomegaly, elevated transaminases) following the administration of aspirin for a viral infection (eg, influenza). These findings are characteristic of Reye syndrome, a rapidly progressive and potentially fatal toxic-metabolic encephalopathy. Most cases are related to salicylates (eg, aspirin), which can cause mitochondrial toxicity, particularly when given to young children for virally-induced fever. In the liver, impaired fatty acid oxidation by mitochondria leads to fat deposition in hepatocytes (ie, hepatic steatosis), hepatomegaly, and acute liver failure. In turn, excess ammonia accumulates due to hepatic dysfunction (eg, reduced urea cycle activity), causing diffuse astrocyte swelling and cerebral edema (eg, vomiting, confusion, lethargy). Treatment of Reye syndrome is supportive. Elevated intracranial pressure (ICP), which disrupts cerebral perfusion, is a major cause of death. (Choice B) Dural venous thrombosis most commonly presents with headache in young women with hypercoagulable risk factors (eg, oral contraception). Encephalopathy due to increased ICP can occur in severe cases, but liver function would not be affected. This patient's age, elevated aminotransferases, and hyperammonemia make this diagnosis unlikely. (Choices C and D) Hemorrhagic necrosis of the temporal lobe is characteristic of herpes simplex virus encephalitis, which can present with altered mental status and focal neurologic deficits similar to those of an inflammatory CNS vasculitis. However, hepatomegaly and acute liver failure would not be expected with either condition. (Choice E) Long-chain fatty acid deposition in the brain can be seen in patients with Alzheimer disease, which causes chronic dementia, not rapidly progressive encephalopathy. Although serum fatty acids can be elevated with Reye syndrome, cerebral deposition does not occur. (Choice F) Pontine myelinolysis (osmotic demyelination syndrome) can cause confusion, lethargy, and coma but typically occurs with rapid correction of hyponatremia due to shifting of water out of brain cells. Liver dysfunction is not associated with this condition. Educational objective: Reye syndrome should be suspected in a young child with acute liver failure and encephalopathy after receiving aspirin for a virus-induced fever. Aspirin in this setting is a mitochondrial toxin that leads to hepatic steatosis, hyperammonemia, and diffuse astrocyte swelling (ie, cerebral edema).

A 2-Year-Old Boy with No Chronic Medical Conditions Is Brought