REYES SYNDROME

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REYES SYNDROME

• BY JENNIFER DELANEY

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OBJECTIVES

• HISTORY
• ETIOLOGY
• WHAT IT DOES
• SIGN SYMPTOMS
• STAGES
• WHAT WE WILL BE CALLED FOR
• DIAGNOSIS
• DIFFERNTIAL DIAGNOSIS
• TREATMENT
• RECOVERY RATE

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HISTORY

• Was named after Dr. R. Douglas Reye.
• Also discovered with fellow Australians Dr.
  Graeme Morgan Dr. Jim Baral.
• Study was first published in 1963 in the British
  Medical Journal.
• The first occurrence may have occurred prior in
  1929.

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HISTORY CONT.

• In 1963 Dr. George Johnson with colleagues
  published an investigation of an outbreak in 16
  children with influenza B that developed
  neurological problems after the virus. 4 of the
  children had symptoms that were very similar to
  those of Reyes.
• For this it is also know as Reyes-Johnson
  Syndrome.

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ETIOLOGY

• Precise reason why it occurs is unknown.
• Its called a syndrome because the clinical
  features that are used to diagnosis it are very
  broad.
• Major presentation that occurs in the United
  States occurs after viral-flu like illness or
  after varicella.

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ETIOLOGY CONT.

• The involvement of salicylates and Reyes was
  first suggested in 1962, which was before Reyes
  was a clinical entity.
• Reports showed that in 4 cases of infants that
  had varicella infections who had received ASA, 2
  of the infants developed symptoms compatible with
  Reyes

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ETIOLOGY CONT.

• Another study done in 1975 reviewed 56 patients
  with pathologically confirmed Reyes. 53 of whom
  had some sort sacilcylate ingestion.
• In 1977 yet another study was done with 175
  confirmed Reyes syndrome which showed that 78
  of the patients had received ASA.

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ETIOLOGY CONT.

• These plus many other reports have led to a great
  deal of talk about ASA and children.
• This lead to a significant decrease in the
  occurrence after 1970.
• It is now not recommended to give ASA to kids 18
  and under without doctors approval.
• Progresses through 5 stages

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WHAT IT DOES

• Disease causes fatty deposits to develop in the
  liver and the brain.

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SIGNS SYMPTOMS

• Usually show up 3-4 days after a viral illness
  such as influenza B and varicella.
• Usually occurs during the Fall Winter months.
• Predominantly occurs in children.

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S/S CONT.

• Sluggish respiratory rate
• Dilated pupils
• Enlarged liver-with no jaundice present
• Encephalitis
• Increased ICP
• Posturing-decerebrate or decorticate

• Continuous N/V
• Sleepless/lethargy
• Confusion
• Irritability
• Aggressive or irrational behavior
• Weakness or hemi-paresis
• Seizures
• Loss of consciousness

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STAGES

• Stage I
• Persistent, heavy N/V
• Generalized lethargy
• Confusion
• Nightmares

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STAGES CONT.

• Stage II
• Stupor (caused by minor brain swelling)
• Hyperventilation
• Fatty liver (biopsy)
• Hyperactive reflexes

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STAGES CONT.

• Stage III
• Continuation of stages I II
• Coma (possible)
• Cerebral edema (possible)
• Respiratory arrest (rarely)

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STAGES CONT.

• Stage IV
• Deepening coma
• Large pupils with minimal response
• Hepatic dysfunction (minimal)

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STAGES CONT.

• Stage V (very rapid onset)
• Deep coma
• Seizures
• Respiratory failure
• Flaccidity
• Extremely high blood amonia
• Death

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911 CALL

• Seizure activity
• Loss of consciousness
• General malaise
• N/V

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DIAGNOSIS

• There is no test for Reyes syndrome.
• Testing for Reyes usually starts with blood and
  urine tests.
• Spinal tap- rule out other diseases with similar
  S/S.
• Liver biopsy- rule out other liver disorders.
• CT or MRI- rule out other causes of the
  behavioral changes.

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DIFFERNTIAL DIAGNOSIS

• Inborn metabolic disorders
• Viral encephalitis
• Drug overdose or poisoning
• Head trauma
• Hepatic failure
• Meningitis
• Renal failure

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TREATMANT

• Usually treated in the hospital setting.
• Care is mainly supportive.
• IV fluids- glucose electrolyte solution with
  sodium, potassium, and chloride.
• Insulin- increase sugar metabolism
• Corticosteroids- to reduce brain swelling.
• Diuretics- increase fluid loss through urination.
• Ventilator- to assist with breathing.

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RECOVERY/PROGNOSIS

• Directly related to the duration of cerebral
  dysfunction.
• Severity and rate of the progression of the coma.
• Severity of increased ICP.
• Fatality rates average 21 percent but can range
  from lt2 in patients in stage I, to gt80 in
  patients in stage IV or V.

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RECOVERY/PROGNOSIS

• If they recover the prognosis is good, and
  reoccurance is rare.
• However incidence of neurologic sequela, ie
  mental retardation, seizure disorders, is as high
  as 30 among those who developed seizures or
  decerebrate posturing during their illness.

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RESOURCES

• Merch Manual, 18th edition
• Mayo Clinic, www.mayoclinic.com/print/reyes-syndro
  me
• Wikipedia, www.wikipedia.org/wiki/Reyes_syndrome
• Kids Health, Reyes Syndrome
• American Liver Foundation, www.liverfoundation.org
  /education/info/reye
• The Western Journal of Medicine, Reyes Syndrome
• Tabers Cyclopedic Medical Dictionary
• All sources where used throughout the
  presentation.