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CC BY-NC-ND 4.0 · Neuropediatrics 2022; 53(06): 389-401
DOI: 10.1055/a-1909-2977
Review Article

Treatment of Infantile Spasm Syndrome: Update from the Interdisciplinary Guideline Committee Coordinated by the German-Speaking Society of Neuropediatrics

Georgia Ramantani    *
1   Division of Clinical Neurophysiology and Epilepsy, University Children's Hospital, Zurich, Switzerland
,
Bigna K. Bölsterli    *
1   Division of Clinical Neurophysiology and Epilepsy, University Children's Hospital, Zurich, Switzerland
,
Michael Alber
2   Department of Pediatric Neurology and Developmental Medicine, University Children's Hospital, Tubingen, Germany
,
Joerg Klepper
3   Department of Pediatrics, Klinikum Aschaffenburg-Alzenau, Aschaffenburg, Germany
,
Rudolf Korinthenberg 
4   Department of Neuropediatrics and Muscular Diseases, Centre of Pediatrics and Adolescent Medicine, University Medical Centre, Freiburg, Germany
,
Gerhard Kurlemann
5   St. Bonifatius Hospital Lingen, Children's Hospital, Lingen, Germany
,
Daniel Tibussek
6   Center for Pediatric and Teenage Health Care, Child Neurology, Sankt Augustin, Germany
,
Markus Wolff
7   Department of Pediatric Neurology, Vivantes Hospital Neukölln, Berlin, Germany
,
Bernhard Schmitt
1   Division of Clinical Neurophysiology and Epilepsy, University Children's Hospital, Zurich, Switzerland
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Abstract

Objectives The manuscript serves as an update on the current management practices for infantile spasm syndrome (ISS). It includes a detailed summary of the level of current evidence of different treatment options for ISS and gives recommendations for the treatment and care of patients with ISS.

Methods A literature search was performed using the Cochrane and Medline Databases (2014 to July 2020). All studies were objectively rated using the Scottish Intercollegiate Guidelines Network. For recommendations, the evidence from these studies was combined with the evidence from studies used in the 2014 guideline.

Recommendations If ISS is suspected, electroencephalography (EEG) should be performed within a few days and, if confirmed, treatment should be initiated immediately. Response to first-line treatment should be evaluated clinically and electroencephalographically after 14 days. The preferred first-line treatment for ISS consists of either hormone-based monotherapy (AdrenoCorticoTropic Hormone [ACTH] or prednisolone) or a combination of hormone and vigabatrin. Children with tuberous sclerosis complex and those with contraindications against hormone treatment should be treated with vigabatrin. If first-line drugs are ineffective, second-line treatment options such as ketogenic dietary therapies, sulthiame, topiramate, valproate, zonisamide, or benzodiazepines should be considered. Children refractory to drug therapy should be evaluated early for epilepsy surgery, especially if focal brain lesions are present. Parents should be informed about the disease, the efficacy and adverse effects of the medication, and support options for the family. Regular follow-up controls are recommended.

Keywords

epilepsy - infantile spasms - infantile spasm syndrome - West syndrome - guideline

Author's Contributions

G.R. contributed toward drafting the initial manuscript, revising the manuscript, analysis and interpretation of data, and acquisition of data. She approved the final manuscript as submitted. B.K.B., M.A., J.K., R.K., G.K., D.T., and M.W. revised the manuscript, did the analysis or interpretation of data, and acquisition of data. They approved the final manuscript as submitted. B.S. drafted the initial manuscript, revised the manuscript, contributed to the study concept, analysis and interpretation of data, acquisition of data, and study supervision. He approved the final manuscript as submitted.


* These authors contributed equally.


Supplementary Material



Publication History

Received: 12 March 2022

Accepted: 05 May 2022

Accepted Manuscript online:
26 July 2022

Article published online:
22 September 2022

© 2022. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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