Use of the Atkins Diet for Children With Sturge Weber Syndrome - Full Text View

Sponsor:	Johns Hopkins University
Collaborator:	Vascular Birthmarks Foundation
Information provided by:	Johns Hopkins University
ClinicalTrials.gov Identifier:	NCT00639730

Purpose

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.

Condition	Intervention	Phase
Epilepsy Sturge Weber Syndrome	Dietary Supplement: modified Atkins diet	Phase I

Study Type:	Interventional
Study Design:	Allocation: Non-Randomized Endpoint Classification: Safety/Efficacy Study Intervention Model: Single Group Assignment Masking: Open Label Primary Purpose: Treatment
Official Title:	Use of the Atkins Diet for Children With Sturge Weber Syndrome

Resource links provided by NLM:

Genetics Home Reference related topics: ankyloblepharon-ectodermal defects-cleft lip/palate syndrome hypohidrotic ectodermal dysplasia Klippel-Trenaunay syndrome Parkes Weber syndrome pyridoxal 5'-phosphate-dependent epilepsy

MedlinePlus related topics: Epilepsy Seizures

U.S. FDA Resources

Further study details as provided by Johns Hopkins University:

Primary Outcome Measures:

Seizures [ Time Frame: 6 months ] [ Designated as safety issue: No ]
Seizure frequency will be compared to baseline (pre-Diet).

Secondary Outcome Measures:

Ketosis [ Time Frame: 6 months ] [ Designated as safety issue: No ]
Safety (stroke like events) [ Time Frame: 6 months ] [ Designated as safety issue: Yes ]

Estimated Enrollment:	5
Study Start Date:	May 2006
Study Completion Date:	May 2010
Primary Completion Date:	March 2010 (Final data collection date for primary outcome measure)

Arms	Assigned Interventions
Experimental: A This is open-label - all patients are placed on the diet. There is no control or placebo arm.	Dietary Supplement: modified Atkins diet High fat, low carbohydrate diet Other Name: Modified Atkins diet

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:

after being on the diet for 3 months and
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.

Eligibility

Ages Eligible for Study:	2 Years to 18 Years
Genders Eligible for Study:	Both
Accepts Healthy Volunteers:	No

Criteria

Inclusion Criteria:

2-18 years
Sturge Weber syndrome
Monthly seizures (at least)
Tried at least two anticonvulsants

Exclusion Criteria:

Prior use of the Atkins or ketogenic diets
Patients with non-epileptic seizures (pseudoseizures) will be excluded
Patients that are pregnant or become pregnant during the study will be excluded
High cholesterol

Contacts and Locations

Please refer to this study by its ClinicalTrials.gov identifier: NCT00639730

Locations

United States, Maryland
Johns Hopkins Hospital
Baltimore, Maryland, United States, 21287

Sponsors and Collaborators

Johns Hopkins University

Vascular Birthmarks Foundation

Investigators

Principal Investigator:

Eric H Kossoff, MD

Johns Hopkins School of Medicine

More Information

Publications:

Kossoff EH, Turner Z, Bluml RM, Pyzik PL, Vining EP. A randomized, crossover comparison of daily carbohydrate limits using the modified Atkins diet. Epilepsy Behav. 2007 May;10(3):432-6. Epub 2007 Feb 26.

Kossoff EH, McGrogan JR, Bluml RM, Pillas DJ, Rubenstein JE, Vining EP. A modified Atkins diet is effective for the treatment of intractable pediatric epilepsy. Epilepsia. 2006 Feb;47(2):421-4.

Responsible Party:	Eric Kossoff MD, Johns Hopkins School of Medicine
ClinicalTrials.gov Identifier:	NCT00639730 History of Changes
Other Study ID Numbers:	NA_00003359
Study First Received:	March 14, 2008
Last Updated:	May 26, 2010
Health Authority:	United States: Institutional Review Board

Keywords provided by Johns Hopkins University:

Epilepsy

Additional relevant MeSH terms:

Epilepsy
Klippel-Trenaunay-Weber Syndrome
Sturge-Weber Syndrome
Neurocutaneous Syndromes
Brain Stem Infarctions
Brain Diseases
Central Nervous System Diseases
Nervous System Diseases
Angiomatosis
Vascular Diseases
Cardiovascular Diseases
Hemangioma
Neoplasms, Vascular Tissue

Neoplasms by Histologic Type
Neoplasms
Ectodermal Dysplasia
Abnormalities, Multiple
Congenital Abnormalities
Skin Abnormalities
Skin Diseases, Genetic
Genetic Diseases, Inborn
Skin Diseases
Brain Infarction
Brain Ischemia
Cerebrovascular Disorders
Stroke

ClinicalTrials.gov processed this record on February 12, 2012