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. 2018 Oct 23;3(Suppl Suppl 2):143-154.
doi: 10.1002/epi4.12264. eCollection 2018 Dec.

Treatment of infantile spasms

Shaun A Hussain  1
Affiliations

Treatment of infantile spasms

Shaun A Hussain. Epilepsia Open. .

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.

Keywords: Epileptic encephalopathy; Epileptic spasms; Hypsarrhythmia; West syndrome.

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Figures

Figure 1
Figure 1
Example of vigabatrin‐associated MRI toxicity. These T2‐weighted MRI images were acquired in a patient with symptomatic vigabatrin‐associated toxicity (encephalopathy, respiratory compromise, and bradycardia) during vigabatrin exposure (panels A, B) and several months following vigabatrin discontinuation and symptomatic recovery (panels C, D). First published in Hussain et al.52
Figure 2
Figure 2
Key results of the International Collaborative Infantile Spasms Study. CT, combination therapy; HT, hormonal therapy. Adapted from O'Callaghan et al.56
Figure 3
Figure 3
A proposed protocol for treatment of infantile spasms. aPrednisolone dosage according to the UKISS/ICISS protocols is a reasonable weight‐independent alternative (i.e., 10 mg 4 times daily for 1 week, with further titration to 20 mg 3 times daily among initial nonresponders). bIf sACTH is used, UKISS/ICISS dosing is suggested (0.5 mg on alternate days for 1 week, with further titration to 0.75 mg on alternate days among initial non‐responders). cRelapse risk is associated with multifocal epileptiform discharges on follow‐up EEG 1 month after successful treatment (Hayashi et al.124). An abundance of epileptiform discharges may thus prompt escalation of treatment (especially VGB) to reduce relapse risk. ACTH, adrenocorticotropic hormone; BID, twice‐daily administration; CBD, cannabidiol; CLN, clonazepam; ES, epileptic spasms; FBM, felbamate; HYPS, hypsarrhythmia; KETO, ketogenic diet; PRED, prednisolone; aACTH, synthetic adrenocorticotropic hormone; TID, thrice‐daily administration; TPM, topiramate; VEEG, video‐electroencephalography; VGB, vigabatrin; VPA, valproic acid; ZNS, zonisamide.
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