Historical details about Dr West, his son, James Edward West, the patient with infantile spasms, and the coining of the eponym ‘West syndrome’ are researched by pediatric neurologists and colleagues at the University of Nijmegen, the Netherlands; and University of California at Irvine. WJ West, a general practitioner at Tonbridge, UK, wrote a letter to the Lancet in 1841 (1:724-725), entitled ‘On a peculiar form of infantile convulsions.’ To paraphrase, the infant was well until 4 months of age, when he began slight bobbings of the head forward, later a complete heaving of the head forward toward his knees, and then immediately relaxing into the upright position, similar to attacks of emprosthotonus. The patient was born Feb 13, 1840 and died at 20 years of age on Sept 27, 1860, having spent the last 7 years of his life in an Asylum for the Feeble-Minded in Redhill, UK. He is buried with his father in the cemetery of the St Peter and Paul parochial church in Tonbridge. When Dr West’s treatments failed (leeches, calomel, phlogiston, hot baths, alterantia drugs, opium), he consulted Drs Clarke and Locock in London, who called the seizures by the term ‘Salaam convulsions,’ having seen two similar cases previously. Subsequent early publications include those of Newnham (1849), using the term ‘eclampsia nutans,’ Fere (1883) who described ‘tic de Salaam or salutation, and Zellweger (1948), who, like Fere, distinguished between symptomatic and idiopathic infantile spasms. Gibbs and Gibbs (1952) introduced the term ‘hypsarrhythmia’ and described the classical triad of diagnostic criteria, ‘spasms, hypsarrhythmia, and mental retardation.’ The term West syndrome was first suggested by Gastaut (1960) at the 9th Colloque de Marseille on infantile spasms, then called ‘infantile myoclonic encephalopathy with hypsarrhythmia’ (IMEH). [1]

COMMENT. The advances made in the riddle of West syndrome, since it was first described 160 year ago, are the descriptive electroencephalographic abnormalities (hypsarrhythmia) by the Gibbs’ (1952) and the introduction of ACTH (1958) by Sorel and Dusaucy-Bauloye, a largely empiric therapy. A study of etiologic factors in 61 patients with infantile West syndrome was published in 1962 (Millichap, Bickford, Klass, Backus, in Epilepsia 3:188), but the pathophysiology of the syndrome still eludes us (Baram, 1993). Incidentally, Tonbridge, the town in UK where West and his son are buried, is situated about 14 km north of Tunbridge Wells, the site of the Spastics Society and editorial office of the journal ‘Dev Med Child Neurol.’

Prognosis of West syndrome

A most current article on West syndrome out of Istanbul University, Turkey, concerns ‘The informative value of magnetic resonance imaging and EEG in the prognosis of infantile spasms.’ [2]. A total of 86 cases, 8 cryptogenic and 78 symptomatic, were followed clinically and by video-EEG and MRI for >1 year. A significant correlation was determined between clinical and EEG findings, especially with regard to psychosocial development, and less so for motor development. MRI findings were correlated only with motor development. EEG and MRI are complementary in regard to prognostic informative value.