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Epilepsy Behav. 2015 Jul;48:61-5. doi: 10.1016/j.yebeh.2015.03.003. Epub 2015 Jun 8.

The ketogenic diet can be used successfully in combination with corticosteroids for epileptic encephalopathies.

Author information

1
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.
2
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.
3
INSERM U1129 "Infantile Epilepsies and Brain Plasticity", Paris, France; University Paris Descartes, PRES Sorbonne Paris Cité, CEA, Gif sur Yvette, France.
4
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.

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.

KEYWORDS:

Encephalopathy with CSWS; Epileptic encephalopathy; Ketogenic diet; Steroid; West syndrome

PMID:
26057351
DOI:
10.1016/j.yebeh.2015.03.003
[Indexed for MEDLINE]

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