This protocol addresses the first-line management of infantile spasms in the symptomatic setting — where abnormal neurodevelopment is present and/or a definable underlying cause has been identified — in patients without tuberous sclerosis.
Symptomatic infantile spasms are defined by the presence of abnormal development and/or a clear etiology for the spasms. This distinguishes them from cryptogenic presentations, which occur in infants with normal prior development and no identifiable cause. This protocol applies specifically to the symptomatic population, excluding tuberous sclerosis complex.
First-line management centers on hormonal therapy, which is the most widely accepted evidence-based approach for this population — the complete agent selection, dosing structure, and taper details are available in the full protocol.
Defines symptomatic infantile spasms as those patients with either abnormal development and/or a clear etiology for the infantile spasms, and cryptogenic spasms as those occurring in the context of normal development without a clear etiology.
ACTH is probably the most universally accepted first-line treatment of infantile spasms, stemming from the class 1 randomized controlled trials performed from 1983–1999 that show its efficacy.
The goals of therapy should include a complete cessation of the clinical events and resolution of hypsarrhythmia or modified hypsarrhythmia on video EEG. Response is typically seen within 14 days or sooner.
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