Causes and Risks: The exact cause of Reye’s syndrome is unknown. However, it has been associated with viral agents such as influenza or varicella, toxins, aspirin and other salicylates (see salicylates - oral), and metabolic defects. It affects children of all ages. The illness has a rapid onset and symptoms may vary greatly. Changes in mental status occur including delerium, combative behavior, and coma.

Typically Reye syndrome follows an upper respiratory infection or chickenpox by about a week. It frequently begins with vomiting which is persistent over many hours. The vomiting is rapidly followed by irritable and combative behavior. As the condition progresses the child may become semi conscious or stuporous. Ultimately seizures and coma develop followed shortly after by death.

Prevention: In general, parents are advised to not give aspirin to their children during a viral illness because of its association with Reye’s syndrome.

Symptoms:

• a history of preceding URI or chickenpox (about 1 week previously)
• nausea
• vomiting (possible)
• mental status changes
• lethargy
• confusion
• combative behavior
• loss of consciousness or coma may develop
• seizures
• unusual posturing (decerebrate posture; the arms are extended straight and turned toward the body, the legs are held straight and the toes are pointed downward)

• weakness in arms or legs
• muscle function loss or paralysis of the arms or legs
• double vision
• speech difficulties
• hearing loss

Signs and Tests:

• blood chemistry tests shows low glucose (hypoglycemia)
• elevated liver enzymes (liver function tests)
• elevated serum ammonia test
• a liver biopsy
• a spinal tap for cerebral spinal fluid analysis
• a neurological examination
• a head CT or head MRI scan that rules out other causes of mental status changes
• a variety of other tests that may be indicated

Treatment: Intensive supportive care is needed, although specific treatment measures have not been determined.

Intravenous fluids to provide electrolytes and glucose are given. Dexamethasone is given to reduce swelling in the brain. Intensive monitoring of the pressure within the brain, blood gases, and blood pH are needed. Support of the respiratory system during a deep coma may be needed.

The stress of illness can often be helped by joining support groups where members share common experiences and problems. See Reye’s syndrome - support group.

Prognosis: The outcome is related to the severity of the coma as well as other factors. The average death rate is about 20%. The outcome for those who survive the acute episode is good. The incidence of permanent neurological complications is not known but is thought to be related to the severity of the coma.

Complications:

• permanent brain damage
• coma
• death

Go to the emergency room or call the local emergency number (such as 911) if your child has confusion, lethargy, or other mental changes.