Ketogenic Diet in the Treatment of Refractory Epilepsy in Childhood Abeer M. Hassan, MD, DCH, Daniel L. Keene, BSc (MED), MD, MA, Sharon E. Whiting, MB, BS, Pierre J. Jacob, MD, Jocelyn R. Champagne, BSCN, RN, and Peter Humphreys, MD, CM There has been renewed interest in the ketogenic diet in the treatment of medically refractory seizure disor- ders in childhood. This article reports the results of a retrospective chart review of 52 patients who were treated with the ketogenic diet. The vast majority (49 of 52) were treated with the classic 4:1 diet. Seizure control improved in 67.3% of patients with complete abolition of seizures in six. Adverse reactions were uncommon and included the development of renal stones, gall bladder stones, and hypoproteinemia in one patient each. Routine biochemical screening during the diet did not identify or prevent adverse events. The authors’ experiences with the diet emphasize the need for close ongoing medical and dietary supervision. © 1999 by Elsevier Science Inc. All rights reserved. Hassan AM, Keene DL, Whiting SE, Jacob PJ, Cham- pagne JR, Humphreys P. Ketogenic diet in the treatment of refractory epilepsy in childhood. Pediatr Neurol 1999;21: 548-552. Introduction The ketogenic diet has been used in the treatment of refractory childhood epilepsy since the early 1920s. Re- cently it has received substantial media attention resulting in an increased number of inquiries from families with children who have refractory epilepsy. These parents are eager for their children to be seizure free and preferably off antiepileptic drugs (AEDs). The reported effectiveness of the ketogenic diet has varied. In at least 50% of patients, it has been reported to reduce seizure frequency by 50% and completely abolish seizures in half of these patients [1-4]. The exact mecha- nism by which the ketogenic diet controls seizures is unknown. Mechanisms of action that have been proposed include altered acid-base balance, change in water and electrolyte concentrations, elevated lipid levels, or direct action of ketone bodies themselves. The advantages of the ketogenic diet are that it may permit a decrease in AED dosages or discontinuance of AEDs altogether, and the patient may become more alert and exhibit improved behavior. There have been several complications attributed to the ketogenic diet [2,5-9]. The poor palatability of this dietary regimen has been a long-standing problem. In the older patient, noncompliance is a major concern. There have been reports of renal calculi in patients on the ketogenic diet; however, this could potentially be avoided thorugh adequate hydration [3,6]. Some patients have had elevated uric acid levels, the significance of which is not under- stood [3]. Disorders of vitamin and mineral metabolism have also been reported but can be avoided by using vitamin and mineral supplements [7]. There has been a case report of optic neuropathy presumed to be the result of a thiamine deficiency; this complication can probably be avoided by giving a thiamine supplement [8]. There have been reports of impaired neutrophil function in children on the ketogenic diet [9]. The purpose of this retrospective study was to review the clinical efficacy, adverse effects, and acceptability of the ketogenic diet. Patients and Methods A retrospective review of the charts of all children initiated on the ketogenic diet at the Children’s Hospital of Eastern Ontario was performed. Nutrition clinic notes were used to supplement physician and nursing clinic records. To be included in this review the patient had to have been less than 17 years of age at the time of initiation of the diet and had to have medically refractory epilepsy, and there had to have been no From the Division of Neurology; Department of Pediatrics; Children’s Hospital of Eastern Ontario; Ottawa, Ontario, Canada. Communications should be addressed to: Dr. Keene; Division of Neurology; Department of Pediatrics; Children’s Hospital of Eastern Ontario; 401 Smyth Road; Ottawa, Ontario K1H 8L1, Canada. Received December 4, 1998; accepted March 23, 1999. 548 PEDIATRIC NEUROLOGY Vol. 21 No. 2 © 1999 by Elsevier Science Inc. All rights reserved. PII S0887-8994(99)00045-4 ● 0887-8994/99/$20.00