Symptomatic Infantile Spasms When First-Line Therapy Fails to Achieve Cessation of Spasms and Resolution of Hypsarrhythmia

Clinical Scenario

This protocol addresses infants with symptomatic infantile spasms — characterised by abnormal neurodevelopment and/or a clear underlying etiology — in whom tuberous sclerosis has been excluded as the cause.

Previous Treatment — Goals Not Met

An initial course of first-line therapy (ACTH, prednisolone, or vigabatrin) was completed but did not achieve the required endpoint: complete cessation of clinical spasms and resolution of hypsarrhythmia on video EEG, as assessed within 14 days of treatment.

Next Treatment Step

When the initial first-line agent has failed to achieve the required response, the recommended approach is to switch to a different first-line medication that was not used in the first treatment course. The choice of agent and full regimen are available in the complete protocol.

Treatment Goals

Complete cessation of spasms and resolution of hypsarrhythmia on video EEG — typically assessed within 14 days of initiating the new therapy.

Instant Access to Structured Evidence-Based Regimens

References

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.
It is recommended to try two first-line therapies before moving on to other options, as there is less data to support the second-line treatments.
Studies have reported patients that respond to ACTH after failing corticosteroids and vice-versa.
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.

DOI: 10.3978/j.issn.2224-4336.2015.09.01

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