2013 年 6 巻 1 号 p. 19-29
Infantile spasms (IS) are an age-specific epileptic syndrome with overall poor outcome and are recognized as one of the 'catastrophic epilepsies'. Current conventional therapies including adrenocorticotropic hormone and vigabatrin often fail to control the spasms and improve the long-term outcome, especially in cases with structural/metabolic etiologies. To improve this situation, new treatments with a disease modifying potential must be identified. Recent translational studies have led to the development of several animal models of IS that reflect their multiple etiologies. Among these, the multiple hit model has been used to screen new promising therapies that, in the future, may be explored in clinical trials.