Abstract

A CONGENITAL FORM OF AMAUROTIC FAMILY IDIOCY BY R. M. AND N. WOOD NORMAN From the Burden Mental Research Department, Stoke Park Colony, and the Pathological Bristol University Department, 11TH (RECEIVED NOVEMBER, 1941) SINCE the original in description of Tay-Sach's disease the eighties of the last century a group of allied nervous disorders has become recognized, the common pathological feature of which is a distention of ubiquitous the nerve cells with granules of a a lipoid nature. Apart from few anomalous forms it is customary to classify these amaurotic family idiocies according to the time of onset of the disease, infantile, late infantile, juvenile, late juvenile and adult types being described. The resemblance between the infantile type and Niemann-Pick's disease had been noted by Knox, et alia, in 1916, but it was not until Pick and Bielschowsky's (1927, 1928) demonstration of the closely similar cerebral pathologies of these two conditions that it became generally realized that disordered lipoid metabolism, not heredodegeneration, was responsible for the granular accumulations in the nerve cells. The consequent inclusion of the amaurotic family idiocies in the category of lipoidoses began a new chapter in the history of these much studied diseases and for this