Infantile Spasms: Opportunities to Improve Care

Abstract

Infantile spasm (IS) is a distinct epilepsy syndrome characterized by epileptic spasms (the clinical seizure type) and hypsarrhythmia (the electrographic abnormality). IS is frequently accompanied by impaired neurodevelopment and is often associated with structural, genetic, or metabolic etiologies. Prompt treatment of this severe epileptic encephalopathy improves long-term outcomes but remains elusive in many situations. Despite common misconceptions, even patients with identified etiologies or preexisting developmental delay benefit from proven standard therapies, including adrenocorticotropic hormone (ACTH), oral corticosteroids, or vigabatrin. Treatment efficacy should be assessed with electroencephalography at 2 weeks, and an alternative therapy is indicated if epileptic spasms or hypsarrhythmia have not resolved. Collaboration with primary care providers is critical to mitigate the potentially serious adverse effects of standard treatments and also to provide developmental interventions. Although new approaches are on the horizon, addressing current challenges and opportunities now can dramatically improve patient outcomes.

Vigabatrin — new data on indications and safety in paediatric epilepsy

“…VGB is an antiepileptic drug used as first-line therapy in IS, a severe epileptic encephalopathy leading to developmental delay which frequently is a precursor of other forms of epilepsy (e.g. Lennox-Gastaut syndrome) [5,13,22]. In IS treatment, VGB's therapeutic effectiveness is weaker than other first line options such as ACTH and corticosteroids [22].…”

“…Lennox-Gastaut syndrome) [5,13,22]. In IS treatment, VGB's therapeutic effectiveness is weaker than other first line options such as ACTH and corticosteroids [22]. However, combined therapy of VGB and ACTH is significantly more effective and faster in achieving clinical and electroclinical responses in children with IS [32].…”

“…Historically, the classic West syndrome triad has included the characteristic clinical seizures -epileptic spasms (ES) -sudden contraction of the trunk and extremities lasting less than one second, most frequently occurring in series, an abnormal interictal electroencephalographic (EEG) pattern (hypsarrhythmia), and developmental abnormalities. However, developmental delay and hypsarrhythmia do not always occur, especially at the beginning, which has led to the updating and broadening of the IS definition by the International League Against Epilepsy (ILAE) and the West Delphi Consensus Statement, according to which IS is recognised when characteristic ES are present, with or without the presence of hypsarrhythmia in EEG [21,22].…”

“…Several randomised, multicentre trials have been conducted to compare different IS therapeutic options. According to ILAE, short-term VGB response rates range from 35% to 54%, much less than with hormonal treatment (76-87%) [22]. In the UKISS study, Lux et al showed that infants assigned to hormonal therapy had a greater chance of not having ES on days 13 and 14 of treatment compared to those treated with VGB at the minimum dose of 100 mg/kg/day (73% compared to 54%) [27].…”

COVID-19: A Review for the Pediatric Neurologist

“…However, if patients fail vigabatrin, ACTH or equivalents should be considered given the risk to the infant of delaying appropriate treatment. 58,59 Febrile Seizures…”