1,18 This is particularly important in epileptic disorders that mostly affect cognition and alertness, such as electrical status epilepticus during slow-wave sleep (ESES). Indeed, the risk of psychomotor retardation in certain epileptic disorders, such as modified hypsarrhythmia, is a major consideration in favor of using the KD. Prolonged

status epilepticus, both convulsive and non-convulsive, is also of special interest. Non-convulsive status epilepticus poses a unique problem because it is Inhibitors,research,lifescience,medical not associated with overt seizures but rather with a significant decline in linguistic ability, leading to a severe, often irreversible, deterioration in scholastic performance.19–21 Teaching case II An 8-year-old girl was referred to our center with a history of seizures of about 2.5 years’ duration. The initial seizures were generalized tonic-clonic type, and occurred only during sleep. The EEG study at the time was suggestive of idiopathic photosensitive occipital Inhibitors,research,lifescience,medical epilepsy of childhood. Treatment consisted of valproic acid with the later addition of sulthiame. This led to an improvement in seizure severity but not frequency. A learning

Inhibitors,research,lifescience,medical disability and an attention deficit disorder were first manifested during the course of treatment. By age 7 years, the EEG tracing was compatible with ESES. The administration of clobazam led to the disappearance of both the seizures and the ESES pattern. However, shortly thereafter, while the patient was fully seizure-free, the EEG pattern switched to non-convulsive status epilepticus (Figure 1). The child’s alertness decreased, her scholastic problems continued, and she started exhibiting behavioral difficulties. Levetiracetam was of no benefit, and administration of intravenous immunoglobulin exacerbated Inhibitors,research,lifescience,medical the EEG findings. Intravenous pulse methylprednisolone successfully normalized the EEG but not the patient’s cognitive/behavioral state, and the non-convulsive

status epilepticus recurred during a trial to taper Inhibitors,research,lifescience,medical the steroids. The patient was started on the KD. Prompt improvement was noted in the EEG findings (Figure 2) as well as in cognition, alertness, and behavior. Interestingly, ketosis was maintained during steroid treatment. DNA ligase With time, it was necessary to increase the dose of steroids, but the parents felt that given its clear beneficial effects, the diet should be continued despite some resistance from the child. Figure 1 Electroencephalographic recording showing non-convulsive status epilepticus. Figure 2 click here Marked improvement of the electroencephalographic recording (EEG) 1 month after initiation of the diet in a patient on maintenance with prednisone, which had failed to normalize the EEG. USE OF THE KD IN INFANTILE EPILEPSY The KD was found applicable for use in infantile epilepsies.22 The most prominent types of infantile epilepsies are IS, ranging from West syndrome to modified hypsarrhythmia.