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Riley-Day syndrome - Treatment

Alternative Names

Familial dysautonomia; Hereditary sensory and autonomic neuropathy - type III

Treatment:

Treatment may include:

  • Protecting the person from injury
  • Treatment of aspiration pneumonia
  • Anticonvulsant therapy if seizures are present
  • Medicines, including liquid tears, to prevent dry eyes
  • Medicines called anti-emetics, to control vomiting

Postural hypotension (low blood pressure when standing) can be managed with increased fluid and salt intake, caffeine, and waist-high elastic stockings

Expectations (prognosis):

With advances in diagnosis and treatment, survival continues to improve. Currently, a newborn with Riley-Day has a 50% chance of reaching age 30.

Complications:

The following symptoms of "autonomic crises" occur in about 40% of patients:

  • Excessive sweating of the head and torso
  • Blotching of the face and torso
  • Mottling of the hands and feet
  • Hypertension (high blood pressure) and tachycardia (rapid heart rate)
  • Nausea/vomiting
  • Severe dysphagia/drooling
  • Irritability
  • Insomnia
  • Worsening of muscle tone

Calling your health care provider:

Call your doctor if symptoms change or worsen.

  • Reviewed last on: 12/11/2006
  • Brian Kirmse, MD, Mount Sinai School of Medicine, Department of Human Genetics, New York, NY. Review provided by VeriMed Healthcare Network.