6533b7d3fe1ef96bd12613fc

RESEARCH PRODUCT

Infantile spasms—A multidisciplinary challenge

subject

description

Is it not surpnsmg that we are unaware of an earlier description of infantile spasms (IS) than that of Dr West from 1841 [1] , though this is hardly a new disease? May be, it needed an experienced observer to delineate these paroxysmal behavioral patterns against the background of abnormal psychomotor development. Up to now , we are by no means less puzzled by this manifestation of seizures than our professional forefather. It was only recently that we realized that the West syndrome consists not only of more or less typical serial seizures, usually a pathognomonic EEG pattern, and developmental arrest [2] , but has to be regarded as an encephalopathy [3-5] interfering with virtually all higher cerebral functions [6] . Pattern and course of this disease are rather monotonous in comparison to the variety of triggering conditions which seem to have in common an event resulting in arrest of brain maturation [7 , 8]; in most cases this may be hypoxia . We are on the verge of abandoning the concept of dichotomic etiology, symptomatic versus idiopathic, it seems more likely that the latter are just cryptogenic cases [9,10]. For obstetricians and neonatologists, IS have become somewhat a measure of the accuracy and efficacy of their perinatal care. Identification of provoking events and documentation of defects by brain imaging techniques have not contributed to the clarification of neither etiology nor pathogenesis [11]. We had to learn that IS belongs to the "proze~haften," deteriorating epilepsies, with a subclinical and clinical onset which may be considerably earlier than judged from the first full-blown Blitz-, Nickor Salaam spasms [6,12]. IS, like the related Lennox-Gastaut syndrome, should be considered in the context of age dependent early epilepsies [4]. In contrast to other epilepsies, they have scarcely profited from conventional and more recently introduced anticonvulsants. Despite worldwide