Researchers at Johns Hopkins Hospital, Baltimore, MD have evaluated the efficacy of the ketogenic diet in the treatment of 104 consecutive infants with infantile spasms (IS) and hypsarrhythmia on EEG. The cohort included 23 of initial patients seen, 13 new-onset IS, and 68 additional patients. The etiology was symptomatic in 74 (71%), and previous therapy had included a mean of 3.6 anticonvulsants, with corticosteroids or vigabatrin in 71%. Diet efficacy was assessed through patient clinic visits at 3, 6, 9, 12, and 24 months, and by telephone and e-mail contact. Developmental progress was based on parental reports and clinical examination. Follow-up EEG was obtained after 6-12 months in children with intractable IS and 2-4 weeks in those with new-onset IS. Mean age at diet onset was 1.2 years, and diet duration was a mean of 1.3 years. An initial 3.1:1 or 3.5:1 ratio of fat to carbohydrate and protein was used in 68 (65%) patients; a 4:1 ratio was started in 16 of older age patients. All patients fasted prior to diet initiation. Parents kept patient records of daily seizure frequency, weekly weight, and biweekly urine ketones. Intent-to-treat analysis showed >50% control of spasms in 64% at 6 months and 77% after 1-2 years. Complete control for at least 6 months within a median of 2.4 months was achieved in 38 (37%). Improvement in development occurred in 62%, EEG improvement in 35%, and reduction in current anticonvulsants in 29%. Adverse effects developed in 33%, with diminished linear growth in 6%. Older age at onset of IS and fewer prior anticonvulsants were associated with >90% spasm reduction at 6 months. In this prospective trial, two thirds of patients with IS treated with the ketogenic diet were benefited, and the diet is strongly recommended for patients who have failed to respond to corticosteroids and vigabatrin. 
COMMENT. Nordli DR Jr and associates at Children's Memorial Hospital, Chicago, have previously demonstrated the efficacy and safety of the ketogenic diet in the treatment of 32 infants with intractable seizures, particularly for infantile spasms/myoclonic seizures.  Fifty five percent of infants had >50% reduction in seizure frequency (19% were seizure-free). Improvements in behavior, attention/alertness, activity level and socialization were also reported. The diet was well tolerated, and 96% maintained appropriate growth. Adverse events were all reversible.