However, the clinical picture was not typical for this abnormality. A second possibility was that these abnormalities were secondary to valproate-induced inhibition of fatty acid oxidation. The valproic acid was discontinued, and all parameters normalized after 1 week. At that point, we felt that it was safe to initiate the KD, which led to some decrease in seizure frequency for several months, making it possible for us to at least taper the vigabatrin dose. The obvious lessons learned from this child are: always rule out the rare contraindications before initiating the diet, even when

the clinical presentation does not MK-8776 purchase support the presence of a contraindication. Biochemical Inhibitors,research,lifescience,medical changes induced by intake of valproic acid can mimic those of a mitochondrial disorder,13 thus, awareness of potential effects of it as well as of other AEDs that are already in use is critical. In this case, Inhibitors,research,lifescience,medical although the metabolic abnormalities were valproic-acid-related, they did not allow for the use of the KD before they had been excluded by withdrawal Inhibitors,research,lifescience,medical of the medication. SPECIFIC CONDITIONS TREATABLE WITH THE KD

The KD has been found to be the most appropriate treatment for glucose transporter 1 deficiency and pyruvate dehydrogenase deficiency.1,11 Other epileptic conditions, including tuberous sclerosis complex, Rett syndrome, severe myoclonic epilepsy of infancy (Dravet syndrome), and specific mitochondrial disorders, also respond to the diet.14 One study noted a 40%–50% seizure-free response Inhibitors,research,lifescience,medical rate in patients with myoclonic-astatic epilepsy (Doose syndrome), which is higher than values reported for AEDs.15 Another report suggested that the KD may be Inhibitors,research,lifescience,medical more effective than AEDs for Lennox–Gastaut syndrome, and the authors recommended that it be considered for

early use in affected patients.16 In terms of seizure type, success appears to be lower in patients with complex partial seizures1,12 or epileptiform discharges in the temporal region.12 Neal et al.17 reported that there was no significant difference in the efficacy of the treatment between symptomatic generalized or symptomatic focal syndromes. In their study, the mean percentage of baseline seizures was significantly lower in the diet however group than in the controls after 3 months (P < 0.0001). Specifically, 38% of the subjects in the diet group had a >50% seizure reduction compared with 6% of the controls (P < 0.0001), and 7% in the diet group had a >90% seizure reduction compared with 0% of the controls (P = 0.0582).17 The conclusion of Keene’s review was that, overall, the estimated rate for obtaining complete seizure control was 15.6% and that one-third of the studies reported a >50% reduction in the number of seizures.