Infantile spasm: A review on the severity of epileptic encephalopathy

Abstract

Infantile spasm (IS) comprises of both an age dependent epileptic seizure and distinctive seizure Onset in infants. In the recent past the key observation in classification and standardization of infantile spasm has culminated many recommendations in distinguishing and recognizing the seizure type and the epileptic syndrome or the West syndrome. The Infantile spasm is an early onset epileptic encephalopathy which presents unique electrographic and clinical features, these features are found in children in the middle of the first year of their birth .However the pathophysiology and the heterogeneity of the infantile spasm remains partially or incompletely understood. In the neurobiological basis there are multiple aetiologies converge to form similar clinical interpretations. The description of the electroencephalographic features of the spasm and its hypsarrthythmia plays a pivot role in early diagnosis. The treatment options for infantile spasm are very limited and it is also called a “catastrophic” due to the poor developmental, cognitive and epileptic progress. In the recent past more detailed information about the electrographic and clinical features of the spasms and hypsarrhythmia in EEG has emerged. The advances in the neuro imaging techniques have revealed about the aetiology and the pathophysiology of infantile spasm to yield a prognosis in patients with infantile spasms. The pathophysiology of infantile spasm needs to be better clarified for any kind of novel treatments and a wide range of preclinical animal studies are essential for advancing the knowledge. Here, in this review paper we focus on the preclinical models of Infantile spasm, with information’s regarding the existing models and research findings, elaborate on some novel models and discuss on new data that can help in advancing the understanding of the cellular mechanisms underlying the specific EEG changes such as ictal electrodecrement and interictal hypsarrhythmia presented in Infantile spasm IS.