Infantile spasms: review of the literature and personal experience

The relative frequency among the epileptic syndromes is an another reason why not only neuropediatricians but also general pediatricians must be fully informed about diagnostic, clinical, imaging and genetic aspects.Early diagnosis is of paramount importance in order to obtain even complete results in patients with so called idiopathic situations. A number of problems are still to be solved. There is no agreement on the type and the schedule of treatment. A common denominator about this problem is not jet available even if some advances in this regard have been accomplished. Of paramount importance is an accurate clinical and laboratory examination as a prerequisite regarding prognosis and results of therapy in every single case.However, even if more than 170 years have elapsed since the first communication of dr. West on the peculiar syndrome that his child was suffering of, the interest of scientists on this subject has now been enriched and rewarded.This epileptic disorder of infancy or early childhood was first described by Dr. William James West in 1841 in his son [1].It is characterized by the triad of infantile spasms, hypsarrhythmia, and retardation. The EEG pattern (hypsarrhythmia) was illustrated by Gibbs [2]. The description of Dr. West is exhaustive: the spasms begun when his son was 4 months old and are described accurately. "The bowing and relaxing of the head and trunk would be repeated alternatively at intervals of few seconds and from ten to twenty or more times at each attack that would continue not more than two or three minutes ...sometimes two, three or more attacks in the day. The child appeared frightened and screamed.... he neither possessed the intellectual vivacity or the power of holding himself upright or using his limbs, and his head falls without support." Dr. West assumed that spasms were caused by an irritation of the nervous system, perhaps caused by teething. The used remedies were no avail. The patient died in an institution at the