Nerve-Cell Swelling of the Juvenile Amaurotic Family Idiocy Type: Associated with Tuberose Sclerosis in an Infant Aged Twelve Months

Nerve-Cell Swelling of the Juvenile Amaurotic Family Idiocy Type: Associated with Tuberose Sclerosis in an Infant Aged Twelve Months.

This paper records the occurrence in the brain of an infant aged twelve months of tuberose sclerosis together with nerve cell changes of a type which, until recently, has been considered peculiar to the juvenile or Spielmeyer-Vogt variety of amaurotic idiocy. Pathological coincidences of this sort in the nervous system are rare and with the exception of Ostertag's (1925) description of infantil...

Juvenile and late forms of amaurotic idiocy in one family.

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...

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...

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...