Ketogenic Diet Controls Seizures Within Two Weeks In Infant With Rare Metabolic Disorders
A classic 3:1 ketogenic diet controlled seizures within two weeks in a female infant carrying two rare inherited metabolic disorders that had resisted five anti-seizure medications, according to a case report published Sept. 18, 2026, in the European Journal of Clinical Nutrition.
Researchers at Tanta University in Egypt describe one female infant diagnosed by whole exome sequencing and enzyme testing with both non-ketotic hyperglycinemia, a genetic disorder that lets the amino acid glycine build up and injure the brain, and biotinidase deficiency, an inherited inability to recycle the B vitamin biotin. She developed focal seizures and worsening brain function at birth that did not respond to five different anti-seizure medications. At 18 months old, during a pediatric intensive care admission, a multidisciplinary team started a classic 3:1 ketogenic diet, three grams of fat for every one gram of combined protein and carbohydrate. The team reported meaningful improvement within two weeks. Over the following year seizures were controlled, three of the five medications were withdrawn, encephalopathy episodes stopped, a feeding tube was removed, and the infant reached developmental milestones the report did not further quantify.
Anti-Seizure Medications Before and After Ketogenic Diet In One Infant: 5 To 2
The authors say the diet may work by interrupting a two-pronged toxic cycle: glycine that keeps accumulating because of the genetic mutation, and a biotin-dependent metabolic pathway that is also disrupted. Shifting the body's main fuel from glucose to ketones is thought to lower circulating glycine and ease strain on the affected pathways, calming the seizures those toxins were driving, according to the authors.
This is a single infant treated in one hospital, not a controlled trial, so there is no comparison group and no way to know how much of the improvement came from the diet versus other changes made during her intensive care admission. The authors reported no competing interests; funding was not stated in the available record.
Data Panel
- Who
- One female infant with confirmed non-ketotic hyperglycinemia and biotinidase deficiency, diagnosed by whole exome sequencing and enzyme assay; seizures began at birth and resisted 5 anti-seizure medications.
- Design
- Single-patient case report; classic 3:1 ketogenic diet (3 grams fat per 1 gram combined protein and carbohydrate) started during a pediatric ICU admission at 18 months of age, supervised by a multidisciplinary team, followed for 1 year.
- Dose
- Classic 3:1 ketogenic diet ratio; 5 anti-seizure medications had failed before the diet started.
- Primary result
- Improvement reported within 2 weeks of starting the diet; over the following year seizures were controlled and 3 of the 5 prior anti-seizure medications were withdrawn, leaving 2.
- Secondary
- Encephalopathy episodes stopped, nasogastric tube feeding was discontinued, and the infant reached developmental milestones not further quantified in the report.
- Funding / conflicts
- Authors reported no financial or non-financial competing interests; funding source not stated in the available record.
Dr. Axe's Take
I read this as evidence that fuel-switching diets belong in the conversation for genetic epilepsies, not only classic refractory epilepsy. Moving this infant's brain from glucose to ketones appears to have eased the glycine buildup driving her seizures, letting the medical team withdraw three of five anti-seizure drugs within a year. That outcome came from one infant inside a hospital with a full metabolic team tracking labs daily, not a home experiment. If your child has a diagnosed metabolic disorder and drug-resistant seizures, ask the neurology and genetics team whether a supervised ketogenic diet fits their specific mutation, and keep every current medication as prescribed unless that team changes the plan.
— Dr. Axe