Reye syndrome is a very rare but life-threatening disorder that causes inflammation and swelling of the brain and impairment and loss of function of the liver.
The cause of Reye syndrome is unknown, although it typically occurs after infection with certain viruses, such as influenza (flu) or varicella (chickenpox), particularly in children who take aspirin during these infections. Because of this increased risk of triggering Reye syndrome, aspirin is not recommended for children, except for the treatment of a few specific diseases (such as juvenile idiopathic arthritis and Kawasaki disease [see Kawasaki Disease]). Now that aspirin use has declined, about 2 children a year develop this syndrome. The syndrome occurs mainly in children younger than 18. In the United States, most cases occur in late fall and winter.
Reye syndrome varies greatly in severity. The syndrome begins with the symptoms of a viral infection, such as an upper respiratory tract infection, influenza, or sometimes chickenpox. After 5 to 7 days, the child suddenly develops very severe nausea and vomiting. Within a day, the child becomes sluggish (lethargic), confused, disoriented, and agitated. These changes in the child's mental condition are sometimes followed by seizures, coma, and death. Impairment and loss of function (degeneration) of the liver may lead to blood-clotting problems and bleeding and buildup of ammonia in the blood.
Doctors suspect Reye syndrome in children who suddenly develop symptoms of brain dysfunction (encephalopathy) and vomiting. To confirm the diagnosis and rule out other diseases, such as certain hereditary metabolic disorders, doctors do blood tests and often remove a piece of liver tissue using a small needle (liver biopsy). Doctors do computed tomography (CT) or magnetic resonance imaging (MRI) of the head and may also do a spinal tap to obtain cerebrospinal fluid for further testing. Once diagnosis is confirmed, the level of severity is staged from I (lowest) to V (highest) based on the symptoms and test results.
The child's prognosis depends on how long the brain has been dysfunctional, the severity and rate of progression of coma, the severity of increased pressure on the brain, and the level of ammonia in the blood. The overall chances that the child will die are about 21% but range from less than 2% among children with mild disease (stage I) to more than 80% among children in a deep coma (stage IV or V). Many children who survive the acute phase of the illness recover fully. However, children who had more severe symptoms may later have signs of brain damage, such as intellectual disability, a seizure disorder, or muscle weakness. Abnormal muscle movement or damage to specific nerves may also occur. Reye syndrome rarely affects a child twice.
There is no specific treatment for Reye syndrome. Children are placed in intensive care. Children with blood-clotting problems are given vitamin K or fresh frozen plasma. To reduce swelling and pressure on the brain, doctors may place a tube into the windpipe (endotracheal intubation) and provide a higher than normal breathing rate (hyperventilation), restrict fluids, elevate the head of the bed, and give drugs (such as mannitol) that force the body to get rid of water. Doctors also give dextrose to maintain a normal level of sugar (glucose) in the blood. Occasionally, doctors place a pressure-measuring device in the head to monitor the increased pressure.
Last full review/revision October 2012 by Elizabeth J. Palumbo, MD