原発性定型網膜色素変性症の予後に関する検討—14施設調査

Visual loss in typical retinitis pigmentosa:a collaborative study in 14 hospitals

　1989年の2か月間に14施設を受診した定型網膜色素変性症253例，男性：女性＝122：131，平均年齢48歳，平均自覚症状発現年齢28歳を対象に，視力予後について分析した。視力と年齢，経過年数との間に有意な相関関係が認められた。視力0.1以下の眼の頻度は，年齢を20歳区切りにすると，若い年齢群から4，23，38，40％の頻度であり，経過年数を20年区切りにすると，25，44，47，67％であった。10°以下の高度視野障害眼の頻度は，40歳以上及び経過年数20年以上で，視力0.1以下の眼の頻度を上まわっていた。遺伝形式別では，優性が孤発や劣性より障害が軽い傾向が認められた。

We evaluated the state of visual acuity in typical retinitis pigmentosa in a collaborative study. A total of 253 cases, 122 males and 131 females, were seen during a 2-month period. The average age was 48 years. Subjective symptoms had appeared, on average, at age 28. The visual acuity correlated with the age and the duration of the disease. The prevalence of eyes with visual acuity of 0.1 or less was 4% under 20 years of age, and 23%, 38%, and 40% for each consecutive 20-year age group. The prevalence of visual acuity of 0.1 or less was 25% under 20 years of duration, and 44%, 47%, and 67% for each cosecutive 20-year duration group.

Advanced visual field constriction to 10 degrees or less was more common than visual acuity of 0.1 or worse in eyes over 40 years of age and/or eyes with duration of 20 years or more. Visual loss tended to milder in dominantly inherited than in recessive and simplex cases.