Neurology Today Conference Reporter: CNS Annual Meeting

Monday, October 9, 2017

A Ketogenic Diet Reduces Intractable Seizures in Infants

BY JAMIE TALAN

KANSAS CITY, MO—A high-fat, low-protein ketogenic diet used to reduce seizures in children and adults is safe and effective for infants, as well, according to a new retrospective study by a team of investigators at the Mayo Clinic in Rochester, MN, who presented their findings here last week at the Child Neurology Society annual meeting.

Elaine C, Wirrell, MD, FAAN, professor of neurology and director of pediatric epilepsy, and her colleagues reviewed medical files from 2007 to 2016, identifying 27 infants who began having seizures at two months old. More than 90 percent were having seizures every day that could not be controlled by at least two different medications.

"Early-life intractable epilepsy has a significant developmental cost," said Dr. Wirrell. "If we can better control seizures, we can hopefully prevent or ameliorate future disability."

Her team reviewed the medical records of the infants for details on their seizures, when the diet was initiated, and how the infants fared. Nineteen of 27 infants had a serious epilepsy syndrome, including West syndrome, epilepsy of infancy with migrating focal seizures, early myoclonic encephalopathy, Ohtahara syndrome, and Dravet syndrome.

All but one baby started the ketogenic formula in the hospital, and within two days, 88 percent of them developed urinary ketosis. Their families were counselled on delivering the diet. Roughly two-thirds of the infants continued the ketogenic diet for at least a year. 

The researchers reported that 68 percent of the infants maintained on the diet achieved a greater than 50 percent reduction in seizures at one month, 82 percent at six months, and 91 percent at one year. By one month, 20 percent had complete seizure freedom, 29 percent at six months, and 27 percent at one year. By contrast, Dr. Wirrell said that a child who has failed two antiepileptic therapies has less than a 10 percent chance of responding to a third or fourth medication.

By 12 months, two infants had to be taken off the diet for adverse effects. They developed increased triglycerides, dehydration, or severe ketoacidosis. Five of the infants continued to have seizures and they were taken off the diet for lack of efficacy. Six were lost to follow-up and two died from unrelated causes, said Dr. Wirrell. She recommends that the diet should be started in the hospital to identify any early side effects such as hypoglycemia with emesis or reduced intake of the formula.

Among theories about the mechanism behind the ketogenic diet is that the mix of high-fat, low carbohydrates, and reduced protein intake may reduce firing of excitatory brain cells that can make the brain vulnerable to seizures. The brain burns fat for energy, instead of carbohydrates.

Implementing the diet is a team effort that is often managed by a dietitian who understands the diet. "We start having discussions with families early on," said Mayo dietitian Susan Eckert.

Commenting on the findings, Eric H. Kossoff, MD, professor of neurology and pediatrics and medical director of the Ketogenic Diet Center at Johns Hopkins Medicine, who was not involved with the study, said: "This is a very important study, adding to the growing recognition that infants can be safely and effectively treated with the ketogenic diet. Only a few years ago this idea would have been discouraged; it's now becoming more widely accepted."​

LINK UP FOR RELATED INFORMATION:

Wirrell, EC. Ketogenic ratio, calories and fluids: Do they matter? Epilepsia 2008; 49:17-19.

Kosseff EH, Zupec-Kania BA, Amark PE, et al. Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group. Epilepsia 2009;50(2):304-317.