Infantile spasms Richard

Infantile spasms represent a seizure disorder with unique clinical and electroencephalographic (hypsarrhythmia) features and a poor prognosis including chronic intractable epilepsy and psychomotor retardation. The association of spasms and hypsarrhythmia, with or without mental retardation, defines West's syndrome. West's syndrome is not uncommon; the incidence is considered to be 0 16-0-42 per 1000 live births.' In 1991, over 760 000 live births occurred in England, Wales, and Scotland (Office of Population Censuses and Surveys, personal communication) giving an estimated number of 122 to 319 new cases ofWest's syndrome each year. In the 150 years since the original description, there has been little progress in the understanding of the pathophysiology of the spasms, although some advance has been made in their classification and aetiology. Treatment has remained essentially empirical, but there is increasing evidence that the newer antiepileptic drugs and even surgery may be of benefit. This paper reviews the current 'understanding' and areas of future development in this seizure disorder.