Review Article Infantile Spasms

Infantile spasms constitute both a distinctive seizure type and an age-specific epilepsy syndrome that have been extensively described for over a century. Stan-dardization of the classification of infantile spasms has evolved, culminating in recent recommendations for separately recognizing and distinguishing the seizure type (spasms or epileptic spasms) and the epilepsy syndrome of infantile spasms (West syndrome). More-detailed descriptions of the clinical and electrographic features of epileptic spasms and hypsarrhythmia have emerged. Advances in neuroimaging techniques have revealed clues about pathophysiology and increased the etiologic yield of the diagnostic evaluation of pa-tients with infantile spasms. Adrenocorticotrophic hor-mone remains the treatment of choice for many neu-rologists. Recent controlled studies support vigabatrin as first-line therapy, and open-label studies suggest that topiramate, lamotrigine, and zonisamide may be useful in treating spasms. Recent reports of visual-field constriction with vigabatrin may limit its use. Surgical treatment has been used successfully in a select sub-group of patients with secondarily generalized spasms from a single epileptogenic zone. Although the progno-sis for most patients with infantile spasms remains poor, further studies identifying predictors of favor-able prognosis and recent advances in understanding the pathophysiology of infantile spasms offer hope of safer and more-effective therapies that improve long