Use of the Atkins Diet for Children With Sturge Weber Syndrome
Condition: Epilepsy; Sturge Weber Syndrome
MeSH terms: Sturge-Weber Syndrome; Epilepsy; Brain Stem Infarctions; Klippel-Trenaunay-Weber Syndrome; Syndrome
Study type: Interventional
Phase: Phase 1

Summary: 
      This research is being done to find out if the Atkins Diet can be used safely and effectively
      to reduce seizures in children who have Sturge Weber syndrome.

      The ketogenic diet is a proven therapy for epilepsy, but can be difficult to use in children.
      The Atkins Diet is a high fat, high protein, low carbohydrate diet introduced in the 1970s to
      help with weight loss. It is different from the ketogenic diet in four major ways: no calorie
      restriction, no fluid restriction, no protein restriction, and is easy to start outside the
      hospital. People on the Atkins Diet become ketotic (produce high levels of ketones, a certain
      substance in the body), like patients on the ketogenic diet, and the investigators believe
      this may lead to seizure control.

      Children aged 2-18 with Sturge Weber syndrome and seizures at least monthly, who have used at
      least two anti-seizure drugs may join. 5 children in total will be enrolled.
    

Detailed description: 
      If you agree to be in this study, we will ask you to do the following things:

      Before the diet is started:

        -  We will ask you to keep track of your child's seizures daily for 2 weeks before starting
           the Atkins diet and provide us with a 3-day food record as well.

        -  Baseline blood (about 2 teaspoons) and urine work will be obtained.

        -  We will give you a free copy of a carbohydrate-counting guide.

        -  You will be asked to fill out a 3-day food record.

      On the diet:

        -  Give your child a daily calcium supplement and multivitamin.

        -  Keep a daily seizure log.

        -  Check urine for ketones (to make sure the diet is working) and specific gravity (to make
           sure your child is well hydrated) twice a week and when you come to clinic.

        -  We will ask you to weigh your child at home every week, and we will weigh him or her
           when you come into clinic too.

        -  Do not fluid restrict your child; please give plenty of carbohydrate-free fluids.

        -  You will give 20 grams per day of carbohydrates. We will give you instructions on how to
           measure daily intake of carbohydrates.

        -  We will not change medications for the first three months. Do not change your child's
           seizure medicines without checking with our doctors.

        -  For the first three months, we ask you to avoid store-bought low-carbohydrate products
           (as they can have more carbohydrates than advertised).

      After being on the diet for one month, Dr. Kossoff will contact you by telephone to discuss
      how your child is doing. You can also speak with one of our dietitians. We will discuss the
      3-day food record with you

      You will be asked to come to the clinic (outpatient center, 5th floor) for two follow-up
      visits:

        1. after being on the diet for 3 months and

        2. after being on the diet for 6 months.

      During these visits you will meet with one of our doctors and a dietitian. We will review
      your child's seizure control and health. We may obtain blood (about 2 teaspoons). We will
      check your child's urine for ketones and specific gravity.

      If the diet is helping beyond 6 months, we will help your child continue on the diet with
      clinic visits and labs as necessary, at a carbohydrate amount that is most helpful.
    

Intervention name(s): modified Atkins diet
Intervention type(s): Dietary Supplement
Intervention description(s): High fat, low carbohydrate diet
Allocation: Non-Randomized
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Treatment
Keywords: Epilepsy