進行性錐・杆体変性症の2例

The two cases of cone-rod dystrophy (progressive cone-rod degeneration)

　9歳で初診した22歳の進行性錐・杆体変性症の1例を報告した。あわせて逆網膜色素変性症と診断された1例を報告し，色覚，暗順応，螢光眼底所見，ERG,ERP,EOGなどの精神物理学的，電気生理学的検索の結果を比較した。

　その結果，いずれも進行性の錐体，杆体の変性疾患で，錐体の障害が杆体の障害よりもより優位であり，cone-rod dystrophy （progressive cone-rod degeneration）と考えられた。

We observed cone-rod dystrophy (progressive cone-rod degeneration) in two females aged 22 and 26 years each. Progressive visual disturbance, photophobia and night blindness were present aschief complaints. Psychophysical and electrophys-iological examinations showed following features.

Fluorescein angiography showed diffuse and mottled hyperfluorescence indicating defects in the pigment epithelial layer. There were reduced or subnormal potentials in the early receptor poten-tial (ERP), photopic and scopic electroretinograms and LP/DT ratio of the electrooculogram.