West's Syndrome (infantile Spasms)--a Polymyographic Study.

A syndrome of brief repetitive massive spasms involving the muscles of the neck, trunk, and limbs, accompanied by either regression or loss of motor and mental skills, was described by Dr. W. J. West, a practitioner in Tonbridge, in his own son (1841). Dr. West had taken the child to London for a consultation with Sir Charles Clarke who had already seen four cases of what he called the 'salaam convulsions'. Sir Astley Cooper, who also saw Dr. West's child, apparently had never seen or heard of such a case: in his opinion, 'it either arose from disease of the brain, and the child would not recover, or it proceeds merely from teething, and when the child cuts all his teeth, may probably get well'. In more recent times the original observations of Vazquez and Turner (1951) and those of Gibbs and Gibbs (1952), that a gross electroencephalographic (EEG) abnormality is common in this syndrome, have been confirmed by many workers. As the sudden, brief, massive spasms are the most dramatic feature, this syndrome (including also mental and motor regression and gross EEG abnormality) has been described under various names (lightning fits, infantile myoclonus, infantile spasms, salaam attacks, jack-knife attacks, massive spasms, etc.); even the term 'hypsarrhythmia', at first introduced by Gibbs to describe the grossly irregular appearance of the EEG, has often been misapplied by other authors to describe the clinical syndrome. In order to avoid further confusion it is proposed that the condition be named 'West's syndrome', at least until more is known about either the aetiological factors or the underlying pathological processes. It would be difficult to improve upon this author's original description of the clinical features and early course of the illness. Patients with West's syndrome have been referred to the Neurophysiological Department at Great Ormond Street at the rate of about 20 to 40 a year from 1957 to 1963 inclusive, representing a figure of 571 3 the order of nearly 200 of all new referrals. Though in the published material both clinical and EEG studies in this syndrome are numerous, little attention has been paid to the study of the motor phenomena occurring during the brief attacks. This neglect may be explained by the following considerations: (1) that in the majority of patients the factors precipitating the attacks are unknown; (2) that the series of brief spasms may last only a few minutes and recur perhaps only once or twice a day; and (3) that a variety of stimuli may stop the series of brief spasms, sometimes for hours. For example, attempts to film the attacks are often wasted because the floodlights may inhibit the beginning ofa series of attacks, or occasionally even interrupt them once they have started. In a study of the co-ordination of voluntary and involuntary movements of the four limbs (Pampiglione, 1959, 1961) a polymyographic method was developed which could be applied to record simultaneously the electrical activity of extensor and flexor muscles of arms and legs in babies even during violent movements. In the present paper the timing, duration, and sequence of the electrical events in muscles, which underlie the motor phenomena, were documented during one or more series of spasms in 25 patients. Often the EEG was recorded together with the activity of the groups of muscles with mostly antagonistic action (biceps and triceps, quadriceps and hamstring).