Abstract
The treatment of infantile spasms is challenging, especially
in the context of the following: (1) a severe phenotype with high morbidity and
mortality; (2) the urgency of diagnosis and successful early response to
therapy; and (3) the paucity of effective, safe, and well-tolerated therapies.
Even after initially successful treatment, relapse risk is substantial and the
most effective therapies pose considerable risk with long-term administration.
In evaluating any treatment for infantile spasms, the key short-term outcome
measure is freedom from both epileptic spasms and hypsarrhythmia. In contrast,
the most important long-term outcomes are enduring seizure-freedom and measures
of intellectual performance in later childhood and adulthood. First-line
treatment options-namely hormonal therapy and vigabatrin-display moderate to
high efficacy but also exhibit substantial side-effect burdens. Data on
efficacy and safety of each class of therapy, as well as the combination of
these therapies, are reviewed in detail. Specific hormonal therapies
(adrenocorticotropic hormone and various corticosteroids) are contrasted. Those
etiologies that prompt specific therapies are reviewed briefly, as are an array
of second-line therapies supported by less-compelling data. The ketogenic diet
is discussed in greater detail, with a focus on the limitations of numerous
available studies that generally suggest that it is efficacious. Special
discussion is allocated to cannabidiol-the investigational therapy that has
received the most attention, and which is already in use in the form of various
artisanal cannabis extracts. Finally, a treatment algorithm reflecting the
concepts and controversies discussed in this review is presented.
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