Reyes Syndrome

Basic Information

R. Douglas Reye, M.D. an Australian pathologist, first recognized Reye’s syndrome as a distinct disease in 1963. The symptoms, however, had appeared as early as 1929. Reye’s (pronounced “rye”) syndrome is a disease believed to be triggered by the ingestion of medicines (such as aspirin) that contain salicylate (pronounced “sa liss a late”) that affects all the organs of the body but affects the liver and brain lethally. It is considered to be a two phase illness because it is almost always associated with a previous viral infection such as influenza, cold or chicken pox. It is not contagious and the cause is unknown.

In Reye’s syndrome, the level of ammonia and acidity rises while the sugar level drops. At the same time the liver may swell and develop fat deposits. Swelling can also develop in the brain and cause such emergency symptoms as seizures or convulsions and eventually lead to coma and brain death.

After Reyes Syndrome was identified in 1963, the number of recognized cases began to increase during the 1970’s. In 1980 the number of cases peeked in the U.S. at 555. After that the figure began a sharp decline. In 1984-94 there were no more than 36 cases of Reye’s syndrome per year, and since then only a few cases have been reported each year.

Research has shown that the ingestion of aspirin during and after a viral illness, such as chicken pox, flu or other respiratory tract illness, significantly increases the chance of Reye’s syndrome. One study found that 90 percent of patients with Reye’s syndrome had taken aspirin before or during a viral illness. People, especially children, with viral illnesses are encouraged not to use aspirin or any medications containing aspirin since it could trigger Reye’s syndrome.

Signs and Symptoms

There are two stages identified with Reye’s syndrome:

Stage I:

Persistent or continuous vomiting
Signs of brain dysfunction; that include listlessness, loss of pep and energy, drowsiness

Stage II:

Personality changes; that include irritability, aggressive behavior
Disorientation; including confusion, irrational behavior, combative behavior
Delirium, convulsions, coma

Reyes syndrome should be suspected in a person if this pattern or symptoms appears during or most commonly, after a viral illness. Not all of the symptoms have to be present or in this order. Fever is usually not present.

In infants, the symptoms may vary or follow no pattern. For example, diarrhea might replace vomiting.

Although cases of Reye’s syndrome have decreased dramatically over the years, due mainly to warning labels on medicines containing aspirin like substances, and also due to better tests used to diagnose metabolic conditions, it is imperative to contact your health care provider if you suspect Reye’s syndrome in a child. Even though Reye’s syndrome is not limited to children, the majority of the cases include children ages 2-16.

There are many over the counter medications that contain aspirin like substances, so it is also imperative that you contact your health care provider concerning treatment of any illness that concerns a child.

Some of the symptoms to be aware of in a child following viral infection would be:

Unusual sleepiness or lethargy
Sudden mental changes
Combative behavior

If your child looses consciousness or has convulsions or seizures, seek emergency medical assistance immediately.

Screening and Diagnosis

If your health care provider suspects Reye’s syndrome, he/she may want to check the liver function. In addition to blood and urine tests diagnostic procedures may include:

Spinal Tap (lumbar puncture): This procedure helps to rule out other diseases with similar symptoms such as meningitis or encephalitis. A local anesthesia is used to numb the puncture site. A needle is then inserted through the lower back into the space around the spinal cord to collect a sample of cerebrospinal fluid. (CSF) The CSF can be analyzed for protein, sugar, and red and white blood cells. Cultures made from these samples can also be used to identify bacterial and viral infections.
Liver Biopsy: In order to draw a tissue sample from the liver, a needle is inserted through the skin on the upper right of the abdomen. This tissue is then used for laboratory analysis to rule out other possible diseases that may be affecting the liver. Sometimes a liver biopsy is done as an “open liver biopsy”. In this case an incision is made in the abdomen, exposing the liver so that a piece of it may be taken for lab analysis.

Your health care provider may also want to evaluate the possibility that your child has one of the rare, inherited metabolic disorders that can mimic the symptoms of Reye’s Syndrome. Most of these may have already occurred with varying degrees of severity in other family members.

Reye' s would be suspected in an infant with:

Diarrhea, but not necessarily vomiting
Respiratory disturbances such as hyperventilation or apneic episodes,
Elevated SGOT-SGPT (SAT-ACT) [usually 200 or more units] in the absence of jaundice.
Unexpected vomiting following any viral illness such as a flu-like upper respiratory infection or chicken pox (usually no diarrhea)

Signs of disturbed brain function characterized by:

Lethargy
Staring
Stupor
Agitated delirium
Screaming
Drug reaction-like behavior
Extensor spasms
Decerebrate rigidity
Aspirin poisoning-like symptoms
Coma

In any case, your health care provider should be contacted and apprised of any Reye's type symptoms. Early diagnosis is vital. Unless the individual with Reye's syndrome is properly diagnosed and treated successfully, death is common and may occur in only a few days. The earlier the diagnosis and treatment, the better the chance of survival. According to the National Reye's Syndrome Foundation, 1996, the fatality rate among those with Reye's syndrome is 52 percent. Being informed of the warning signs is very important in order for the existence of the disease to be identified as soon as possible; for Reye's disease can be misdiagnosed as meningitis, diabetes, poisoning, mental illness or drug abuse, as the incidence of the syndrome is low, and many people are still unaware of it. Writing down the child's specific symptoms and behaviors and requesting the health care providers careful documentation of the symptoms in the child's chart is crucial in obtaining a timely and accurate diagnosis. Complications Neurological abnormalities resulting in mental retardation is the main complication; if the child survives the disease. Some children may have slight brain damage, while others may have none, and appear to recover completely. Psychological and neuropsychological testing should be conducted to determine the extent of impairment that was sustained. These effects would depend on the severity of the swelling of the brain. Treatment Treatment is usually provided in an intensive care unit in the hospital. The patient would require intensive monitoring of their fluids, electrolytes, blood gas status and nutrition. The amount of pressure of the fluids within the skull, as well as blood pressure, is closely monitored. Barbiturates and other drugs may be given to reduce this pressure and cool body temperature. Insulin is often given to increase glucose metabolism, corticosteroids to reduce brain swelling and diuretics to increase fluid loss. Patients are not given any food by mouth, but are fed intravenously. A breathing machine or respirator may be needed if the child begins having greater difficulty breathing. The outcome of each case depends, in large part, on the amount of swelling that occurred in the brain (Kids Health Organization, 1996). Two Things to Remember Never give aspirin or medicines containing aspirin to any children between the ages of 2 and 16. Always contact your health care provider immediately if you suspect reye's syndrome, the earlier the diagnosis, the better the chance of recovery. Other Resources Kids Health Organization National Reye's Syndrome Foundation at 1-800-233-7393 Always Your Choice Updated August 2001