{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T13:27:03Z","timestamp":1790861223940,"version":"4.1.0"},"reference-count":44,"publisher":"Georg Thieme Verlag KG","issue":"06","license":[{"start":{"date-parts":[[2022,7,26]],"date-time":"2022-07-26T00:00:00Z","timestamp":1658793600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropediatrics"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n          Objectives\u2003The 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.<\/jats:p><jats:p>\n          Methods\u2003A 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.<\/jats:p><jats:p>\n          Recommendations\u2003If 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.<\/jats:p>","DOI":"10.1055\/a-1909-2977","type":"journal-article","created":{"date-parts":[[2022,7,26]],"date-time":"2022-07-26T23:14:03Z","timestamp":1658877243000},"page":"389-401","source":"Crossref","is-referenced-by-count":40,"title":["Treatment of Infantile Spasm Syndrome: Update from the Interdisciplinary Guideline Committee Coordinated by the German-Speaking Society of Neuropediatrics"],"prefix":"10.1055","volume":"53","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7931-2327","authenticated-orcid":false,"given":"Georgia","family":"Ramantani","sequence":"additional","affiliation":[{"name":"Division of Clinical Neurophysiology and Epilepsy, University Children's Hospital, Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0008-3339","authenticated-orcid":false,"given":"Bigna K.","family":"B\u00f6lsterli","sequence":"additional","affiliation":[{"name":"Division of Clinical Neurophysiology and Epilepsy, University Children's Hospital, Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Alber","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology and Developmental Medicine, University Children's Hospital, Tubingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joerg","family":"Klepper","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Klinikum Aschaffenburg-Alzenau, Aschaffenburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4638-3460","authenticated-orcid":false,"given":"Rudolf","family":"Korinthenberg","sequence":"additional","affiliation":[{"name":"Department of Neuropediatrics and Muscular Diseases, Centre of Pediatrics and Adolescent Medicine, University Medical Centre, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerhard","family":"Kurlemann","sequence":"additional","affiliation":[{"name":"St. Bonifatius Hospital Lingen, Children's Hospital, Lingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Tibussek","sequence":"additional","affiliation":[{"name":"Center for Pediatric and Teenage Health Care, Child Neurology, Sankt Augustin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Wolff","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology, Vivantes Hospital Neuk\u00f6lln, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernhard","family":"Schmitt","sequence":"additional","affiliation":[{"name":"Division of Clinical Neurophysiology and Epilepsy, University Children's Hospital, Zurich, 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