Ketogenic Diet in Infantile Spasms: Time for New Perspectives

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Abstract and Introduction

Abstract

The ketogenic diet is a nonpharmacologic treatment for epilepsy. Infantile spasm is a particular form of epilepsy in infants characterized by a high level of resistance to antiepileptic drugs and a poor outcome. In recent years, small studies have suggested that the ketogenic diet may be an interesting option for refractory infantile spasms. The presently evaluated prospective study reports the use of the ketogenic diet in 104 patients with infantile spasms. The ketogenic diet is an interesting treatment in infantile spasms and data strongly suggest that it should be used as a second- or third-line treatment in infantile spasms. It may also be used as first-line treatment in selected patients. The authors present new perspectives, suggesting that now is the time to cautiously evaluate all epileptic conditions that may be treated early by the ketogenic diet, such as myoclonic-astatic syndrome, Dravet syndrome, Lennox–Gastaut syndrome, worsening epilepsy and status epilepticus.

Introduction

A large prospective study on the use of the ketogenic diet (KD) in cases of infantile spasm (IS) has been recently published.[1] The KD is a high-fat, low-carbohydrate, nonpharmacologic treatment for epilepsy that has been in continuous use since 1921. This new study provides valuable data on the efficacy of the KD in IS, an epilepsy syndrome that usually occurs between the ages of 3 and 8 months. IS is classified as an epileptic encephalopathy.[2] Prognosis is mostly unfavorable, even in cryptogenic cases, and the majority of patients exhibit profound developmental regression. Hormonal therapies (adrenocorticotropic hormone [ACTH] or prednisolone) and vigabatrin are the usual first-line treatments. These treatments are effective, especially corticosteroids (ACTH and high-dose oral prednisolone), demonstrating approximately 60–70% spasm-free rates, and vigabatrin, with 40–60% spasm-free rates.[3] Other antiepileptic drugs (AEDs) have demonstrated limited efficacy. No guidelines to manage refractory IS are currently available. It has been suggested by several studies that the KD can be an efficacious nonpharmacologic therapy for intractable IS.[4–6] More recently, Kossoff et al. have also started to use the KD in nonrefractory IS with promising results.[7]

Future Neurology. 2010;5(5):653-656. © 2010 Future Medicine Ltd.

Cite this: Ketogenic Diet in Infantile Spasms: Time for New Perspectives – Medscape – Sep 01, 2010.