Key takeaways:

• The number of seizures per day was lower from baseline to 3 months after diet initiation.
• The number of medications used by patients also decreased after diet initiation.

LOS ANGELES — Introduction of a ketogenic diet reduced seizure burden in children with Aicardi syndrome as soon as 3 months, with beneficial long-term effects, according to a poster at the American Epilepsy Society annual meeting.

“Aicardi syndrome often has high morbidity and mortality, and these patients often pass away from the seizure component of the disorder,” Samantha Katz, MD, from the Ann and Robert H. Lurie Children’s Hospital of Chicago, told Healio. “We’re looking at what sort of treatments can help decrease seizure burden in hopes to ultimately decrease morbidity and mortality.”

Prior research demonstrated that the ketogenic, or keto, diet, which reduces carbohydrates and increases fat intake from sources like meat, fish and eggs, lessens seizure burden in similar pediatric disorders. So, Katz and colleagues sought to examine the long-term efficacy and tolerability of the ketogenic diet in Aicardi syndrome.

They conducted a retrospective chart review of 10 children (mean age at first seizure, 2.7±0.5 months; mean age at diet initiation, 14.9±9.5 months) with clinical and radiological diagnosis of Aicardi syndrome who were treated at Lurie between 2004 and 2024.

The researchers followed each patient to track seizure-related events at baseline, 3 months, 1 year and 5 years.

According to the results, the average diet duration was 6.45±6.4 years. Five patients remained on the ketogenic diet, which was associated with long-term benefits of decreased seizure burden. Three patients discontinued the diet — often due to difficulties inherent in diet maintenance — and two individuals died from Aicardi syndrome, Katz said.

At baseline, patients recorded as many as 14 seizures per day, with four individuals experiencing double-digit seizures. The researchers noted that no patient experienced more than five seizures per day 3 months after starting the ketogenic diet.

At 1 year, two of seven patients with available data who were still on the diet were seizure free.

By year 5, one of four patients with available data who were still on the diet had up to six seizures a day, one patient had three seizures per day and two patients had a single seizure per day.

Medication use also decreased after diet initiation. Before the diet, patients used a total of 11 medications, including clonazepam, lamotrigine, levetiracetam, phenobarbital, topiramate, valproic acid and vigabatrin. Following initiation, only seven medications were recorded, among them cannabidiol.

“[Ketogenic diet therapy] is an effective and safe intervention with minimal long-term risks in the pediatric population for patients with Aicardi syndrome,” Katz and colleagues wrote. “Further studies are warranted to evaluate and establish efficacy, the effect on electroencephalogram and quality of life.”