Reye's Syndrome

Reye's syndrome is an extremely rare but serious illness a potentially fatal disease that causes numerous detrimental effects to many organs, especially the brain and liver, as well as causing a lower than usual level of blood sugar (hypoglycemia). The classic features are liver damage, aspirin use and a viral infection. The exact cause is unknown, and while it has been associated with aspirin consumption by children with viral illness, it also occurs in the absence of aspirin use. The disease causes fatty liver with minimal inflammation and severe encephalopathy (with swelling of the brain). The liver may become slightly enlarged and firm, and there is a change in the appearance of the kidneys. Jaundice is not usually present.

Early diagnosis is vital; while most children recover with supportive therapy, severe brain injury or death are potential complications. Reye syndrome often begins with vomiting, which lasts for many hours. The vomiting is quickly followed by irritable and aggressive behavior. As the condition gets worse, the child may be unable to stay awake and alert. Other symptoms of Reye syndrome are Confusion, Lethargy, Loss of consciousness or coma, Mental changes, Nausea and vomiting, Seizures, Unusual placement of arms and legs (decerebrate posture) -- the arms are extended straight and turned toward the body, the legs are held straight, and the toes are pointed downward.
Other symptoms that can occur with this disorder includes Double vision, Hearing loss, Muscle function loss or paralysis of the arms or legs, Speech difficulties, Weakness in the arms or legs.

Most diagnosed with Reye's syndrome have a history of a recent viral infection. Chickenpox and influenza are identified most often, though rotavirus (a cause of bowel inflammation or gastroenteritis) has also been described. In addition to the recent viral infection, most have a history of taking aspirin to control fever. Some researchers have suggested that children with undiagnosed metabolic disorders may also be at risk, though this is not completely clear.

Primarily the treatment is aimed at decreasing the effects of the metabolic dysfunction. Patients with Reye's syndrome are admitted to an intensive-care unit and monitored for a worsening neurologic and metabolic condition. The primary goal is to manage electrolyte imbalances and brain swelling. It is difficult to predict which patients will have a progressive illness. Unfortunately, there is no absolutely effective treatment for Reye's syndrome.