Pearlman J T, Flood T P, Seiff S R
Am J Ophthalmol. 1976 Apr;81(4):417-9. doi: 10.1016/0002-9394(76)90296-8.
Sixty-eight consecutive patients with retinitis pigmentosa were studied to determine the frequency of the nonpigmented form of the disease. There was an overall incidence of 22%. Fifty percent of all cases had no characteristic pigmentation, if the duration of night vision difficulty was three years or less. The nonpigmented form of retinitis pigmentosa was frequently an early stage of the disease and not an unusual or atypical variant. Patients without the pigmentary changes characteristic of the disorder also showed less functional impairment: the electroretinographic b wave was more apt to be recordable, although impaired, and the rod threshold, as determined by dark adaptation measurement, was less elevated. The clinician should suspect retinitis pigmentosa, even in the absence of pigmentary changes, if there is a family history of the disorder, night blindness, peripheral field loss, and an impaired or nonrecordable electroretinographic response.
对68例连续性视网膜色素变性患者进行了研究，以确定该病无色素型的发生频率。总体发病率为22%。如果夜视力困难的持续时间为3年或更短，所有病例中有50%没有特征性色素沉着。视网膜色素变性的无色素型通常是该病的早期阶段，而非异常或非典型变体。没有该疾病特征性色素变化的患者功能损害也较小：视网膜电图b波虽然受损，但更易于记录，并且通过暗适应测量确定的视杆阈值升高程度较小。如果有该疾病的家族史、夜盲、周边视野缺损以及视网膜电图反应受损或无法记录，即使没有色素变化，临床医生也应怀疑患有视网膜色素变性。
