A CONGENITAL FORM OF AMAUROTIC FAMILY IDIOCY

A Congenital Form of Amaurotic Family Idiocy.

SINCE the original description of Tay-Sach's disease in the eighties of the last century a group of allied nervous disorders has become recognized, the common pathological feature of which is a ubiquitous distention of the nerve cells with granules of a lipoid nature. Apart from a few anomalous forms it is customary to classify these amaurotic family idiocies according to the time of onset of t...

Juvenile and late forms of amaurotic idiocy in one family.

The Thalamic Pathology of Amaurotic Famiijy Idiocy

Most of our knowledge of the human thalamus has evolved from experimental work on animals and from the study of focal lesions in the human brain. Strangely enough, not much progress has been made in the investigation of the pathology of the thalamus. While the anatomist and the physiologist, in studying the thalamus, are aware of the fact that they are dealing with a combination of different nu...

Retinal Neuropathology of Late Infantile Amaurotic Idiocy.

THE retinal histopathology of amaurotic idiocies, as seen in sections stained with routine technique, as well as some of the histochemical changes are relatively well known (Holmes and Paton, 1925; Givner and Roisin, 1944; MacMillan, 1948). Good knowledge of the neuropathology, however, in this group of diseases is largely limited to the brain (Spielmeyer, 1906). To demonstrate the changes of t...

A report of two cases of the juvenile form of amaurotic familial idiocy (cerebromacular degeneration).

The cerebral lipidoses are rare diseases, which have few certain links in common beyond the shared facts of disturbed lipid metabolism, progressive mental deterioration coming on early in life, and a heredofamilial pattern of occurrence. In NiemannPick's disease, Gaucher's disease, and xanthomatosis, the lesions of the nervous system are part of a disorder which affects many tissues, though in ...