J Korean Ophthalmol Soc.  1965 May;6(1):29-34.

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

• Kim SM
• Yoo JH

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.

MeSH Terms

Age of Onset
Diagnosis
Family Characteristics
Female
Heredity
Humans
Incidence
Korea
Male
Neurologic Examination
Orbit
Pallor
Pedigree
Prognosis
Retinal Vessels
Scotoma
Skull
Visual Acuity
Visual Fields