Batten Disease

How Is Batten Disease Diagnosed?

• Genetic testing
• Blood test
• Physical and eye exam

What Are the Signs and Symptoms of Batten Disease?

• Seizures/epilepsy
• Dementia
• Vision loss
• Language delay or loss
• Walking less (decreasing)
• Personality and behavior changes
• Motor decline like balance, coordination, and muscle weakness

How Is Batten Disease Treated?

Your child will have an Ommaya^® or Rickham^® reservoir implanted under their scalp. Their medicine, called Brineura^® will be given through their scalp or chest port every 2 weeks. This medicine helps prolong your child’s ability to walk. These reservoirs are only used for Brineura and should only be accessed by a brain doctor (neurologist) or brain surgeon (neurosurgeon).

The neurologist will insert the needle into your child’s port. Lidocaine cream is used to numb the skin before they insert the needle. The nurse will pull cerebrospinal fluid from the port. The sample will be sent to the lab. During the infusion, your child will be monitored. The infusion may last 4 hours. When it’s done, the needle will be removed, and your child will go home.

When Should I Call the Doctor?

Call your child’s doctor or health care provider right away if they:

• Have seizures.
• Have a headache.
• Feel shaky.
• Have low blood pressure (hypotension).
• Feel grumpy (irritable).
• Are vomiting.
• Have a fever of 100.4° Fahrenheit (F) or 38° Celsius (C) or higher.

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