Treatment of Infantile Spasms: Report of the Interdisciplinary Guideline Committee Coordinated by the German-Speaking Society for Neuropediatrics

Abstract

Objectives This report aims to define treatment goals, to summarize the evidence level (EL) of different treatment options for infantile spasms (IS), both in terms of efficacy and adverse effect, and to give recommendations for the management of IS. Methods The Cochrane and Medline (1966-July 2014) databases were searched. Literature known to the guideline working group and identified through citations was also considered. The results of previously published guidelines were taken into account in our analysis. Rating the level of evidence followed the Scottish Intercollegiate Guidelines Network. Recommendations If IS are suspected, electroencephalogram (EEG) should be performed within a few days and, if confirmed, treatment should be initiated immediately. Response to first-line treatments should be evaluated clinically and electroencephalographically after 14 days.Adrenocorticotropic hormone, corticosteroids, and vigabatrin are the first-line drugs for the treatment of IS. In children with tuberous sclerosis complex, vigabatrin is the treatment of first choice. Ketogenic diet, sulthiame, topiramate, valproate, zonisamide, and benzodiazepines can be used when first-line drugs have proved ineffective. Children refractory to drug therapy should be evaluated for epilepsy surgery, especially if focal brain lesions are present.Regular follow-up controls, including EEG (preferably sleep EEG) and standardized developmental assessment are recommended.

Response to “Is ACTH therapy loaded with severe side‐effects? Do not use synthetic ACTH with the same dosages as ‘natural’ ACTH”

“…Consequently, treatment protocols have been adapted in many centers and also by us. 8 We therefore agree with the concerns of Professor Riikonen and we cannot recommend the high-dose protocol used in our study. However, it remains still unclear which low dose is to be used.…”

West syndrome: a comprehensive review

“…Cannabinoid treatment for epileptic seizures has received careful interest [131]. At the moment no clear results about the efficacy and long-term safety in ISs patients have been reported [132].…”

Treatment of infantile spasms in Saudi Arabia

“…A more recent report by a European interdisciplinary guideline committee expanded the first line drugs to include ACTH, other corticosteroids, and vigabatrin. 7 Although most pediatric neurologists (71%) recognize epilepsy surgery as an option in selected patients, they rarely referred their resistant cases for surgical evaluation. However, many pediatric neurologists considered vitamin B6 and the ketogenic diet for resistant cases which is consistent with the recent literature.…”