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This is open-label - all patients are placed on the diet. There is no control or placebo arm.

modified Atkins diet

Eric H Kossoff, MD

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.

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.

Use of the Atkins Diet for Children With Sturge Weber Syndrome

Seizure frequency will be compared to baseline (pre-Diet).

Vascular Birthmarks Foundation

Johns Hopkins University

Eric Kossoff MD

Recurrence

Glioblastoma

Brain Neoplasms

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Antineoplastic Agents

All Drugs and Chemicals

Bevacizumab

Antineoplastic Agents, Immunological

Angiogenesis Inhibitors

Growth Inhibitors

Patients and caregivers will be educated by a nutritionist skilled in the MAD. Patients will also be receiving Bevacizumab as standard of care.

Subjects receive Bevacizumab as standard of care

The modified Atkins diet (MAD) includes high fat, unlimited protein, and restricted carbohydrates (\< 20gm/day).

Modified Atkins Diet

Lisa Rogers, DO

Patients may participate in this research study if they have glioblastoma. (a brain tumor) that has come back after being treated. Standard treatment for this cancer is a chemotherapy drug called bevacizumab. This research study involves bevacizumab in combination with a special diet called the Modified Atkins Diet (MAD).

The purpose of this study is to research if patients can stay on the MAD when it is added to the standard bevacizumab treatment.

Primary Objective

- Determine compliance to treatment at 6 and 12 weeks. Compliance is assessed by review of the daily food diary and the urine and serum ketone levels. Diet compliance is defined as following the diet guidelines 80% of the time with resultant ketosis 80% of the time. If 60% of the patients are compliant with the diet, as defined above, that is a considered a positive result.

Secondary Objective(s)

* Determine patient compliance in monitoring of blood glucose and urine levels of ketosis.
* Determine obstacles to compliance.
* Determine the frequency of achieving ketosis, as measured by urine ketones checked daily and serum BHB checked every two weeks.
* Determine quality of life (FACT-BR) and adverse events.
* Determine response, progression free survival at 6 months (PFS 6), and overall survival (OS).

Exploratory Objective

* To correlate levels of ketosis and blood sugar with treatment outcome.
* To correlate the level of MCT4 expression and IDH1 mutation status with treatment outcome.

Feasibility Trial of the Modified Atkins Diet and Bevacizumab for Recurrent Glioblastoma

A Feasibility Trial of the Modified Atkins Diet and Bevacizumab for Recurrent Glioblastoma

The number of patients following a Modified Atkins Diet for at least 80% of the time as a measure of feasibility

The number of patients with documented ketosis (Serum beta-hydroxybutyrate (BHB) above 4mg/L) for at least 80% of the time as a measure of feasibility

Correlate the relationship between blood sugar levels and measured level of compliance

Correlate the relationship between ketosis values and measured level of compliance

Correlate the relationship between ketosis values and measured level of tumor response as a measure of treatment outcomes

Correlate the relationship between blood sugar levels and measured level of tumor response as a measure of treatment outcomes

University of Cincinnati

OhioHealth Research Institute

Case Comprehensive Cancer Center

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Use of the Atkins Diet for Children With Sturge Weber Syndrome	NCT00639730	Epilepsy Sturge Weber Sy...	modified Atkins...	2 Years - 18 Years	Johns Hopkins University
Feasibility Trial of the Modified Atkins Diet and Bevacizumab for Recurrent Glioblastoma	NCT02768389	Glioblastoma Brain Tumor	Bevacizumab Modified Atkins...	18 Years -	Case Comprehensive Cancer Center
Feasibility Trial of the Modified Atkins Diet and Bevacizumab for Recurrent Glioblastoma	NCT02768389	Glioblastoma Brain Tumor	Bevacizumab Modified Atkins...	18 Years -	Case Comprehensive Cancer Center

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