[Hereditary optic atrophy of the Kjer's type].

Kjer's disease is a benign form of hereditary optic nerve atrophy observed mainly in women. It starts in childhood and progresses slowly without leading to complete blindness. The inheritance is dominant. These features differentiate it from other known forms of hereditary optic nerve atrophy, that is Leber's and Behr's syndromes. The reported case was observed in a 21-year-old man whose mother, grandmother and great-grandmother had optic nerve atrophy. Since the age of 10 years the patient had visual acuity impairment progressing slowly. Examination demonstrated central scotoma, pallor of both optic discs particularly on the temporal side. After 10 years of the disease the visual acuity was 0.5 on the right and 0.3 on the left side. There was bilateral hearing impairment of high tones. The patient had no knee and ankle jerks. Differential diagnosis of Kjer's syndrome against other hereditary-degenerative diseases is discussed.