Clinical Survey of 10 Cases of Leber's Optic atrephy Among 3 Families.

Kim, Sang Min; Yoo, Jin Hyoung

Original Article

Journal of the Korean Ophthalmological Society 1965;6(1):29-34.

Clinical Survey of 10 Cases of Leber's Optic atrephy Among 3 Families.

Sang Min Kim, Jin Hyoung Yoo

Eye Department, Catholic Medical College, Korea.

3가족에 (三家族) 나타난 ( LEBER ) 시신경위축 (視神經萎縮) 10명에 (名) 대한 (對) 임상적관찰 (臨床的觀察)

김상민(Sang Min Kim),유진형(Jin Hyung Yoo)

Abstract

Since it had not been possible to find any formal report on this disease in Korea, we had chance to examine and to make accurate diagnosis, so that we would like to report 10 calles among 3 families. Throughout the literature, the age of onset, the incidence, the mode of heredity, the etiology, the clinical picture, the prognosis, and the treatment were discussed. The results of this survey are as followings. 1. There were 5 males and 5 females in this material. This sex incidence showed rather similar with Japanese's than European's and American's. 2. As to the incidence of age of onset, Case I and III were between 15-22 years old, but Case II were between 6-8 years old. 3. As to the visual acuity, most patients had from H.M. to 0.1 (20/200), and most patients were who associated with the color disturbance. 4. The ophthalmoscopic finding revealed only total or temporal pallor on the disc, otherwise normal macula and retinal vessels. 5. Visual field changes showed central scotoma in all cases which were examined. 6. Various laboratory examinations, X-ray examinations for skull and orbit, neurological examination, etc., showed no significant pathological finding in cases which were examined. 7. As to the mode of heredity, those three families revealed not a typical sex-linked recessive form, as far as we could observe in two generations of the pedigree.