Two cases of the juvenile form of amaurotic family idiocy

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 ...

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...

A case of clinical juvenile amaurotic idiocy with the histological picture of Alzheimer's disease.

The clinical picture of juvenile amaurotic idiocy was originally described in Norway by Stengel in 1826. He observed four siblings with normal development up to the age of 6 years when amaurosis and subsequent mental disorders developed. From the age of 14 years convulsive seizures were noted. The disease ran a progressive course and the patients died at the age of about 20 years. Vogt in Germa...