Abstracts

Rationale: We report an 11 year old boy with glycine encephalopathy, diagnosed in the newborn period, who was initiated on Ketogenic diet for the treatment of medication refractory epilepsy. Methods: This report utilizes EEG data, metabolic testing, and parental reporting. Results: In addition to poor seizure control, he was also experiencing increased lethargy as a side effect from his antiepileptic medication. The patient was admitted to the pediatric epilepsy monitoring unit (EMU), connected to video electroencephalography (VEEG) and a nasogastric tube (NGT) was placed for diet initiation. The diet ratio was titrated slowly over the course of four days until moderate to large ketones in the urine were achieved. All antiepileptic medications were compounded to a sugar free formulation to comply with the diet. The patient showed a resolution of clinical seizures, a dramatic increase in alertness and wakefulness and a marked improvement in his serum amino acid profile following implementation of the Ketogenic Diet. Ketosis induced by a ketogenic diet caused improvement in the patient reported. Conclusions: Patients with glycine encephalopathy may benefit from Ketogenic Diet to be used as treatment for intractable seizures. Funding: None