Abstracts

To evaluate efficacy and safety of the ketogenic diet (KD) in infantile spasms (IS) by reviewing our experiences. We undertook a retrospective analysis of 34 patients with intractable IS treated with the KD from June 1995 to January 2003 at two epilepsy centers. The efficacy and safety of the classic 4:1 ketogenic diet (KD) as an add-on treatment in refractory IS were evaluated. In 9 patients, ketone milk (Ketonia, brand name) constituted mainly with medium chain triglyceride was introduced. Outcome measures included seizure frequency, EEG findings, and adverse effects during the ketogenic diet. Outcomes for a reduction of seizure frequencies are as followings; at 3 months after initiating the diet, all patients remained on the diet, 61.8% (21/34) showed a reduction of seizure frequency with over 50% including 35.3 % (12/34) with seizure free; at 6 months, 67.6% (23/34) patients remained on the diet, 58.8% (20/34) with over 50% including 50% (17/34) with seizure free; at 9 months, 44.1% (15/34) patients remained on the diet, 42.2% (14/34) with over 50% including 38.2% (13/34) with seizure free; at 12 and 15 months, respectively, 35.3% (12/34) patients and 32.4% (11/34) remained on the diet with seizure free. Supplementarily introduced Ketonia was well tolerated. During the KD, 29.4% (10/34) patients should discontinue the diet due to complications in 11.8% (4/34) and/or intolerance in 17.6% (6/34). The complications included gastrointestinal troubles in 2 patients, serious infectious disease in 1 patient and lipoid aspiration pneumonia in 1 patient. By modifying the protocol to omit the initial period of fasting in 17 patients, especially in infants and early childhood period as an usual onset age of IS, we could prevent acute dehydration in most patients, with no difference in the time to ketosis or in the efficacy of the diet. EEG background rhythms with hypsarrythmia before the KD were significantly improved in 43.3% (13/30) at 3 to 6 months after starting the diet, even showing normal EEG in 1 patient. Many of patients with intractable IS could be completely controlled by the KD without serious complications. The KD should be considered as a next step treatment in patients with IS refractory to other treatment modalities with expecting high efficacy and tolerability, and early trial prior to prolonged high dose anti-epileptic drugs could be considered.