Dominant Hereditary Optic Atrophy with Bitemporal Field Defects

Abstract

In April, 1946, a patient presented himself with the complaint of failing vision. He was found to have bilateral simple optic atrophy and bitemporal hemianopsia. The most likely cause of such a picture appeared to be intracranial disease involving the optic chiasm. The history of the patient's family was unique in that several other members also had optic atrophy and bitemporal hemianopsia. Furthermore, the condition did not conform to the usual characteristics of Leber's disease in its onset or in its clinical course. Analysis of Cases The family under consideration is affected by no hereditary trait other than that of optic atrophy. All the members of the family are of average intelligence and have no physical signs of pituitary disturbance. With the exception of the two oldest members described, the information concerning these patients has been obtained from direct examination. Facts relating to the two oldest members are anamnestic. In