The EEG pattern of hypsarrhythmia may be associated with psychomotor developmental arrest. The prognosis, especially for modified hypsarrhythmia, is usually very unfavorable. Treatment for infantile seizures traditionally consists of adrenocorticotropic hormone (ACTH) and prednisone. However, these medications have variable side effects, and they can be used only for a limited period should spasms recur. Vigabatrin has received much attention for its efficacy in IS, but Inhibitors,research,lifescience,medical it can lead to some visual dysfunction. Other AEDs, such as topiramate, lamotrigine, zonisamide,

valproate, clonazepam, and ganaxolone, have limited efficacy in this setting. The KD offers an alternative therapeutic approach. The experience thus Inhibitors,research,lifescience,medical far, as summarized in recent studies by Kossoff et al.,23–25 is encouraging. Those authors reported that of 104 children with infantile spasm treated with the KD, 64% showed a >50% improvement after 6 months, including those who stopped the diet before then. One-third of the infants had prolonged periods Inhibitors,research,lifescience,medical free of spasms, and some became spasm-free permanently.25 Accordingly, treatment with a liquid KD in 12 infants as part of one study and 61 infants in another26 yielded a >90% reduction in

the number of seizures. In 2010, The Infantile Spasm Working Group released guidelines for use of the KD: “The ketogenic diet may be an option in drug-resistant epilepsy as an adjunct to pharmacologic therapy. The diet may work by enhancing γ-aminobutyric acid synthesis and improves energy utilization in the brain. Currently, there is insufficient class I evidence to recommend the ketogenic diet as a first-line intervention [for infantile spasm].”27 Inhibitors,research,lifescience,medical During the last 2 years, our Pediatric Neurology Inhibitors,research,lifescience,medical Service has treated more than 35 epileptic infants with the KD in the form of liquid formula, either orally or via a selleck nasogastric tube. About one-half of them were dropped from the treatment because of unresponsiveness or side effects, such as dyslipidemia, feeding difficulties, and weight loss. About 70% of the remaining patients have

shown a >90% reduction in the number of seizures, while 23% had no improvement. also Infants with severe epilepsy syndromes other than IS may also be expected to benefit from the KD, including those with Ohtahara syndrome, early-onset myoclonic epilepsies, migrating partial epilepsy of infancy, and Dravet syndrome.22 The most daunting challenge is the use of the diet in cases of infantile myoclonic encephalopathies, from infantile spasms through severe myoclonic encephalopathy of infancy to myoclonic-astatic epilepsy and others. The influence of the diet lies within the spectrum of marked improvement in seizure control and cognitive state, to failure of the diet and inability of the parents to withstand its difficulties. The diet may be beneficial in all types of epilepsy, but the rate of success is totally unpredictable.