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
