Journal of the Korean Ophthalmological Society 1964;5(2):55-62.
Clinical Survey of 43 Cases of Pigmentary Degeneration of the Retina Including 3 Cases of Laurens-Moon-Biedl Syndrome.
Sang Min Kim, Byung Joo Yoon
Eye Department, National Medical Cencter, Korea.
Laurence - Moon - Biedl Syndrome 3예를 (例) 포함한 (包含) 강막색소변성증 (綱膜色素變性症) 43예에 (例) 대한 (對) 임상적 (臨床的) 관찰 (觀察)
김상민(Sang Min Kim),윤병주(Byung Joo Yoon)
Abstract
Summary: Forty three cases of the pigmentary degeneration of the retina (P.D.R.) visited N.M.C. from Nov. 1958 to Sept. 1964 were analysed clinically in regard to various factors which were also based on a review of litrature. The results of the study include following findings. 1. Among 43 cases, retinitis pigmentosa binocular were the largest (25), Laurens-Moon-Biedl Syndrome (3), Macular Involvement (2), Retinitis Pigmentosa sine pigment (2), Retinitis Pigmentosa monocular (1). 2. 6-15 years group is exceedingly largest in regard to the age of the onset. 3. Male is exceedingly prevalent (32) than female(11). 4. 51-60 years old group is the largest in regard to the duration of illness. 5. The known cases through the family history was only 10. The mode of heredity seems to be rather prevalent as recessive than dominant but the group is too small to make a definite decision. 6. Pigmentary deposits in the retina was the largest in regard to the ophthalmoscopic findings. Coneen tric contraction is the the largest as to the visual field changes. 7. Cataract is the largest (24 cases), myopia (12 cases) in the next, as to the complications. 8. As to the symptoms of the Laurens-Moon-Biedl Syndrome, Obecity, Retinitis Pigmentosa, and Mental retardation is found in all cases, but Polydactly in 2 cases, and Genital dystrophy in 1 case. None of cases were specifically related to this disease through the family history.


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