Riley-Day syndrome

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Chromosomes and DNA

Familial dysautonomia; Hereditary sensory and autonomic neuropathy - type III (HSAN III)

Treatment may include:

• Anticonvulsant therapy if there are seizures
• Feeding in an upright position and giving textured formula to prevent gastroesophageal reflux
• Increased fluid and salt intake, caffeine, and elastic stockings to prevent low blood pressure when standing (postural hypotension)
• Medicines called antiemetics, to control vomiting
• Medicines, including liquid tears, to prevent dry eyes
• Physical therapy of the chest
• Protecting the person from injury
• Providing enough nutrition and fluids
• Surgery or spinal fusion
• Treating aspiration pneumonia

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

The following complications occur in about 40% of patients with this condition:

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

Call your doctor if symptoms change or get worse. A genetic counselor can help clarify information about the condition and tell you how to contact support groups in your area.

Review Date: 10/10/2010
Reviewed By: Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine. Also reviewed by Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org).