Current and future treatment of infantile spasms

Abstract

Introduction: Infantile spasms (IS) are an age-related epileptic encephalopathy associated with spasms in clusters, hypsarrhythmia and psychomotor delay. Duration of the disease negatively impacts both psychomotor development and pharmacosensitivity. Therefore, rapid and efficient management is crucial for long-term prognosis, and a precise treatment algorithm needs to be promoted.

Areas covered: This review is based on the combination of literature data drawn from a PubMed review from 1968 to 2015 and own experience.

Expert opinion: The algorithm combines several treatment strategies in order to optimize the benefit/risk ratio. We propose a stepwise therapeutic strategy, starting with vigabatrin for at least 2 weeks and prolonged in the case of success for a period depending on the etiology of IS. In case of failure, a switch to oral steroids is advised for at least for 2 weeks, and in case of success continuing with slowly decreasing dose for a total time of 1 to 6 months depending on age in order to prevent relapse. Adrenocorticotropic hormone is therefore restricted to failures of oral steroids. The forth step is the ketogenic diet (KD). Topiramate (under close clinical control to recognize nephrocalcinosis), then zonisamide and valproate should come after, eventually in addition to the KD.

Keywords:

• encephalopathy
• spasms
• treatment
• vigabatrin

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Notes

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