title: The ketogenic diet can be used successfully in combination with corticosteroids for epileptic encephalopathies.
abstract: Hormonal therapy or ketogenic diet often permits overcoming the challenging periods of many epileptic encephalopathies (West and Lennox-Gastaut syndromes and encephalopathy with continuous spike-waves in slow sleep), but relapse affects over 20% of patients. We report here a monocenter pilot series of 42 consecutive patients in whom we combined oral steroids with the ketogenic diet for corticosteroid-resistant or -dependent epileptic encephalopathy. We retrospectively evaluated the effect on seizure frequency, interictal spike activity, neuropsychological course, and steroid treatment course. Twenty-three patients had West syndrome (WS), 13 had encephalopathy with continuous spike-waves in slow sleep (CSWS), and six others had miscellaneous epileptic encephalopathies. All patients succeeded to reach 0.8 to 1.6g/l ketone bodies in the urine following the usual KD regimen. For at least 6 months, 14/42 responded to the addition of the ketogenic diet: 4/23 with WS, 8/13 with CSWS, and 2/6 with miscellaneous epileptic encephalopathies. The addition of the KD allowed withdrawing steroids in all responders. Among them, 10/15 had been patients with steroid-dependent epileptic encephalopathy and 4/27 patients with steroid-resistant epileptic encephalopathy. Therefore, the ketogenic diet can be used successfully in combination with corticosteroids for epileptic encephalopathies. Patients presenting with steroid-dependent CSWS seem to be the best candidates.
authors.affiliation: Hospices Civils de Lyon, HFME, Paediatric Neurology Department and Centre of Reference for Tuberous Sclerosis and Rare Epileptic Disorders, F-69677 Bron, France. Electronic address: dorothee.ville@chu-lyon.fr.
authors.forename: Dorothée
authors.initials: D
authors.lastname: Ville
authors.affiliation: INSERM U1129 "Infantile Epilepsies and Brain Plasticity", Paris, France; University Paris Descartes, PRES Sorbonne Paris Cité, CEA, Gif sur Yvette, France; Neuropaediatrics Department, Necker Hospital, APHP, Paris, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Chiron
authors.affiliation: INSERM U1129 "Infantile Epilepsies and Brain Plasticity", Paris, France; University Paris Descartes, PRES Sorbonne Paris Cité, CEA, Gif sur Yvette, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: Laschet
authors.affiliation: INSERM U1129 "Infantile Epilepsies and Brain Plasticity", Paris, France; University Paris Descartes, PRES Sorbonne Paris Cité, CEA, Gif sur Yvette, France; Fondation Ophtalmologique Rothschild, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Dulac
mesh_terms: D000305:Adrenal Cortex Hormones / Q000494:pharmacology / Q000627:therapeutic use*; D000927:Anticonvulsants / Q000627:therapeutic use; D055423:Diet, Ketogenic; D004569:Electroencephalography / Q000187:drug effects; D004827:Epilepsy / Q000178:diet therapy* / Q000188:drug therapy*; D005260:Female; D006801:Humans; D007223:Infant; D065768:Lennox Gastaut Syndrome / Q000188:drug therapy; D008297:Male; D012189:Retrospective Studies; D012640:Seizures / Q000175:diagnosis / Q000188:drug therapy*; D012890:Sleep / Q000187:drug effects; D013036:Spasms, Infantile / Q000175:diagnosis / Q000188:drug therapy; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000927:Anticonvulsants
keywords: Encephalopathy with CSWS; Epileptic encephalopathy; Ketogenic diet; Steroid; West syndrome