Riley-Day syndrome

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Chromosomes and DNA

Overview Symptoms Treatment Prevention

Riley-Day syndrome

Treatment may include:

• protection from injury
• treatment of aspiration pneumonia
• anticonvulsant therapy if seizures are present
• liquid tears and bethanechol to prevent drying of eyes
• anti-emetics may be used 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

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 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

Call your doctor if symptoms change or worsen.

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