JUVENILE AMAUROTIC IDIOCY

Abstract

Clinicopathologic studies of the juvenile type of amaurotic family idiocy are infrequent. The present case offers an opportunity to correlate the neurologic, psychologic and electroencephalographic studies made shortly before the patient's death with the pathologic observations. REPORT OF A CASE A 16 year old youth entered the hospital Nov. 1, 1940 with the complaint of blindness for ten years, loss of memory and convulsions for one year. His development was said to have been normal until he was 6 years old. There was no family history of any neurologic or psychiatric disorder. His mother, father and 20 year old brother were alive and well. At the age of 6 years his mother noticed that he started to bump into objects, and soon after beginning school his first grade teacher noted difficulties in vision. An ophthalmologist told the parents that the condition would progress to complete blindness. During the following two