Infantile Epileptic Encephalopathy with Hypsarrhythmia (Infantile Spasms/West Syndrome)

Abstract

Infantile spasms is a unique disorder peculiar to infancy and early childhood. In this article, the clinical manifestations and electroencephalographic features of the disorder are described. The possible pathophysiologic mechanisms underlying infantile spasms and the relation of this disorder to other childhood encephalopathies are discussed. Finally, the treatment of patients with infantile spasms and their long-term outcome are briefly reviewed.

Getting rid of the catastrophe: frontier research in infantile spasms

“…On the other hand, structural/ metabolic (symptomatic) IS caused by preexisting brain disorders comprise the largest proportion of cases and are more frequently refractory to conventional therapies [1,25]. Thus, it is crucial to develop successful animal models of symptomatic IS to study mechanisms and design safer and more effective treatments.…”

“…Infantile spasms (IS) or West syndrome is an age-specific epileptic syndrome characterized by recurrent spasms, often accompanied by arrest or regression of psychomotor development as well as specific electroencephalographic (EEG) patterns showing interictal hypsarrhythmia and ictal electrodecremental responses [1]. IS typically begin in the first year of life and often transform into other intractable epilepsies in later life.…”

“…Depending on the etiology, IS have been classified into structural/metabolic, genetic, and etiologyunknown [2]. More than 200 possible causes have been described but the majority of IS are linked to pre-existing structural/metabolic brain pathologies such as cortical dysplasia, tuberous sclerosis complex, known metabolic disorders, and hypoxic/ischemic brain damage [1,3]. These structural/metabolic IS are frequently refractory to conventional therapies including adrenocorticotropic hormone (ACTH) and vigabatrin, although studies have suggested that successful initial treatment with these drugs results in better epilepsy and cognitive outcome in the etiologyunknown subgroup [4,5].…”

West syndrome and autism: a case report

“…Approximately 75% of the patients are in symptomatic WS group in which convulsions develop due to cortical malformations, prenatal events, neurocutaneous syndromes (tuberosclerosis, sturgeweber), chromosomal impairment and metabolic diseases. In criptogenic group, the underlying cause can not be clarified, while in idiopathic WS, the psychomotor development of the patients prior to convulsion attacks is normal [3].…”

Comparative Analysis of Therapy Outcomes in Patients With West Syndrome Receiving Tetraсosactidе and Other Antiepileptic Drugs

“…Одной из основных задач терапии этой злокачествен-ной эпилептической энцефалопатии раннего возраста является как можно более раннее и полное купиро-вание приступов, так как именно наличие приступов и гипсаритмии катастрофически влияет на развитие когнитивных и моторных функций [6]. Поэтому эф-фективным лечением при синдроме Веста рекомен-довано считать такую терапию, которая обеспечивает полный и максимально быстрый контроль инфан-тильных спазмов [9].…”