Abstracts

Rationale: Infantile spasms is one of the malignant epilepsy syndromes associated with both an arrest in psychomotor development and the electroencephalographic pattern of hypsarrhythmia or one of its variants. It has an unfavorable prognosis. The usual treatments for infantile spasms include ACTH and Vigabatrin. These medications have serious potential side effects. Other anti-epileptic medications (AEDs) like sodium valproate and topiramate have limited success rates. We reviewed the efficacy of the ketogenic diet in the treatment of intractable infantile spasms at Children’s Hospital of Pittsburgh. Methods: From May 2008 to May 2009 we started 8 infants with intractable infantile spasms on the ketogenic diet. Diagnoses included HIE in 2, pyruvate dehydrogenase deficiency in one, prematurity with intracranial hemorrhage in 1 and cryptogenic in 4. EEGs showed hypsarrhythmia or modified hypsarrhythmia in 6 children. The EEG in 2 infants showed multifocal sharp waves and a disorganized background. All patients had the diagnosis of infantile spasms confirmed with EEG monitoring. MRI showed cystic encephalomalacia in 3 infants, hemosiderin deposits in 1 and was non-contributory in 4. Infants were treated with 2 to 5 AEDs before the ketogenic diet was started initially at a 3:1 ratio and then increased to 4:1. Results: All 8 infants showed improvement on the diet. Three infants were spasm free after 4-6 weeks on the diet. One of these infants relapsed after one month but still maintained a > 50% reduction in spasms. Two infants had > 75% reduction in the spasms frequency and 3 infants had a >50% reduction. EEG’s also showed improvement in 3 infants attributable to dietary therapy. Conclusions: This study shows that the ketogenic diet can be efficacious in the treatment of intractable infantile spasms. All 8 infants showed improvement with both a decrease in frequency of spasms and an improvement in the EEG. Two infants had complete and sustained resolution of spasms. The ketogenic diet should be considered as an option for infantile spasms that fail to respond to standard treatments.