Infantile spasms

Infantile spasms constitute both a distinctive seizure type and an age-spec ific epilepsy syndrome that have been extensively described for over a cent ury. Standardization of the classification of infantile spasms has evolved, culminating in recent recommendations for separately recognizing and disti nguishing the seizure type (spasms or epileptic spasms) and the epilepsy sy ndrome of infantile spasms (West syndrome). More detailed descriptions of t he clinical and electrographic features of epileptic spasms and hypsarrhyth mia have emerged, Advances in neuroimaging techniques have revealed clues a bout pathophysiology and increased the etiologic yield of the diagnostic ev aluation of patients with infantile spasms. Adrenocorticotrophic hormone re mains the treatment of choice for many neurologists, Recent controlled stud ies support vigabatrin as first-line therapy, and open-label studies sugges t that topiramate, lamotrigine, and zonisamide may be useful in treating sp asms. Recent reports of visual-field constriction with vigabatrin mag limit its use. Surgical treatment has been used successfully in a select subgrou p of patients with secondarily generalized spasms from a single epileptogen ic zone. Although the prognosis for most patients with infantile spasms rem ains poor, further studies identifying predictors of favorable prognosis an d recent advances in understanding the pathophysiology of infantile spasms offer hope of safer and more-effective therapies that improve longterm outc ome. (C) 2001 by Elsevier Science Inc. All rights reserved.