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Reye Syndrome

(Reye's Syndrome)

By

Christopher P. Raab

, MD, Sidney Kimmel Medical College at Thomas Jefferson University

Reviewed/Revised Feb 2023
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Topic Resources

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 but it may be triggered by a viral infection and the use of aspirin.

  • Children typically have symptoms of a viral infection and then severe nausea, vomiting, confusion, and sluggishness, sometimes followed by coma.

  • The diagnosis is based on sudden changes in the child's mental condition and on the results of blood tests and a liver biopsy.

  • The prognosis depends on how long and how severely the brain is affected.

  • Treatment involves measures to lower pressure on the brain.

Now that aspirin use has declined and chickenpox is less common because of vaccination, only about 2 children a year in the United States develop Reye syndrome.

The syndrome occurs mainly in children younger than 18.

In the United States, most cases occur in late fall and winter.

Symptoms of Reye Syndrome

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 caused by increased pressure within the skull (intracranial pressure) and are sometimes followed by seizures Seizures in Children Seizures are a periodic disturbance of the brain’s electrical activity, resulting in some degree of temporary brain dysfunction. When older infants or young children have seizures, they often... read more , coma Stupor and Coma Stupor is unresponsiveness from which a person can be aroused only by vigorous, physical stimulation. Coma is unresponsiveness from which a person cannot be aroused and in which the person's... read more , and death.

In some children, the liver does not function correctly, which may lead to blood-clotting problems and bleeding and a buildup of ammonia in the blood.

Diagnosis of Reye Syndrome

  • Laboratory and imaging tests

  • Biopsy of the liver

Doctors suspect Reye syndrome in children who suddenly develop changes in mental condition and vomiting.

To confirm the diagnosis of Reye syndrome 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 Biopsy of the Liver Doctors can obtain a sample of liver tissue during exploratory surgery, but more often they obtain a sample by inserting a hollow needle through the person's skin and into the liver. This type... read more ).

Once the diagnosis is confirmed, the level of severity is staged from I (lowest) to V (highest) based on the symptoms and test results.

Treatment of Reye Syndrome

  • Measures to lower pressure on the brain

There is no specific treatment for Reye syndrome.

Children are hospitalized and placed in an intensive care unit Types of units People who need specific types of care while hospitalized may be put in special care units. Intensive care units (ICUs) are for people who are seriously ill. These people include those who have... read more . 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 medications (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 inside the head to monitor the increased pressure on the brain.

Prognosis for Reye Syndrome

The child’s prognosis depends on how long and how severely the brain was affected, whether the illness progressed to 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.

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