Long-term outcome of the ketogenic diet for intractable childhood epilepsy with focal malformation of cortical development.
OBJECTIVE
We evaluated the efficacy and long-term outcome of the ketogenic diet in patients with intractable childhood epilepsy as a result of focal malformation of cortical development.


METHODS
A retrospective analysis evaluated seizure outcomes of 47 patients who had intractable epilepsy from (and) surgically remediable focal malformation of cortical development and were first treated with the classic ketogenic diet, involving the 4:1 lipid/nonlipid ratio. The long-term prognosis of 21 patients, who became seizure-free 3 months after the ketogenic diet, was followed up with that of 22 patients who eventually underwent epilepsy surgery.


RESULTS
Three months after diet initiation, 29 (61.7%) patients showed a reduction in seizure frequency of >50%, including 21 (44.7%) who became seizure-free. Of the 21 patients with complete seizure control at 3 months, 16 (76.2%) successfully completed the diet for 2 years without relapse, and 10 (47.6%) have remained seizure-free after cessation of the diet (mean follow-up for 3 years and 10 months), including 1 patient who remained seizure-free with additional medication after a relapse. Of the 22 patients who underwent epilepsy surgery, a seizure-free outcome was obtained for 13 (59.1%).


CONCLUSIONS
The ketogenic diet should be considered to be an additional option even in patients with focal malformation of cortical development, and long-term seizure-free outcome can be expected for patients who become seizure-free 3 months after the diet.
Jung
Da Eun
DE
Department of Pediatrics, Pediatric Epilepsy Clinic, Severance Children's Hospital, Brain Research Institute, Yonsei University College of Medicine, Seoul, Korea.
Kang
Hoon Chul
HC
Kim
Heung Dong
HD
D002540:Cerebral Cortex / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D050528:Diet, Carbohydrate-Restricted; D004041:Dietary Fats / Q000008:administration & dosage*; D004044:Dietary Proteins / Q000008:administration & dosage; D004569:Electroencephalography; D004829:Epilepsy, Generalized / Q000175:diagnosis / Q000178:diet therapy* / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007657:Ketone Bodies / Q000096:biosynthesis; D007662:Ketosis / Q000378:metabolism*; D008279:Magnetic Resonance Imaging; D008297:Male; D019635:Neurosurgical Procedures / Q000379:methods*; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome
D004041:Dietary Fats; D004044:Dietary Proteins; D007657:Ketone Bodies