What Should be the Next Choice After Failure of Hormonal and Vigabatrin Therapy in Infantile Epileptic Spasms Syndrome?

Excerpt

The epidemiology of infantile epileptic spasms syndrome (IESS) in South Asia is peculiar because of a long treatment lag, preponderance of structural etiology, availability of synthetic adrenocorticotropic hormone therapy (ACTH), and limited availability of centers with expertise in ketogenic diet therapy and epilepsy surgery [1]. Many children with IESS fail to respond to hormonal therapy (oral steroids or intramuscular ACTH) and vigabatrin, whether tried sequentially or in combination. Then, the question arises of the suitable next choice and available options—another form of hormonal therapy, antiseizure medications (nitrazepam, topiramate, etc.), ketogenic diet, or epilepsy surgery. If oral steroids have been tried as hormonal therapy and failed, it may be worth giving a trial of ACTH—synthetic or natural, depending on availability and feasibility. However, if an option of ACTH therapy has been initially exhausted, there is no quality evidence on the effectiveness of subsequent trial of oral steroids therapy. However, it might be effective in some children with IESS based on the corresponding author’s experience. The option of epilepsy surgery should be explored in children with IESS having lateralized pathologies. However, in children with non-lateralized pathologies where curative epilepsy surgery is not possible, there remain limited options — trial of the ketogenic diet or other antiseizure medications. …