History of clinical identification of West syndrome--in quest after the classic.

摘要

The West syndrome (WS) is a distinct age-dependent global encephalopathy which encompasses manifold problems of developing brain, and because of this, WS stands out as a symbolic syndrome for child neurology as a whole. It is unanimously recognized that this syndrome was first described by Dr W.J. West of Tunbridge, UK in 1841. In the following 100 years, however, the disease remained in the dark of neglect and misconception. An extensive literature survey carried out by Gastaut et al. revealed that only a few articles followed after West; about one article per decade between 1840 and 1920, and 18 per decade between 1920-1950. Among those, most distinguished contributions were detailed clinical observations made by Asal and Moro (1925) and Zellweger (1948), according to the author's opinion. An explosion of scientific interest at the world level was triggered by the discovery of hypsarhythmia on EEG by Gibbs and Gibbs in 1952 and of dramatic therapeutic effect of ACTH by Sorel and Dusaucy-Bauloye in 1958. In Japan, Katsutaka Takagi first reported four cases of apparent WS in 1903. An extensive search for Japanese classic literatures conducted by the author revealed 13 highly probable WS cases scattered in eight papers by 1945. A great confusion in terms of a concept of the disease had been prevailed for 50 years after Takagi until 1957, when the author first reported clinical and EEG findings in 99 cases, together with a 16 mm film demonstration of typical spasms in three cases at the Japan Pediatric Society meeting. Needless to say, however, WS turned out to be one of the most popular targets for clinical investigation of child neurologists in Japan afterwards, and nowadays, about 30 to 40 reports continue to be either published or orally presented at the meeting each year.