Study on the correlation between rate of visual field loss and gene abnormality in retinitis pigmentosa

视网膜色素变性是由多种基因异常引起的。由于该病的基本治疗方法尚未确立，有关疾病进展和视力预后的信息对患者可能很重要。本研究提出了一种利用自动静态视野检查定量评估RP病情进展的方法，对RP患者的视野检查结果进行了研究，并利用HFA文件版本对Humphrey视野检查程序30-2和10-2的平均偏差（MD）进展率进行了分析。5（BeeLine Office Co. Tokyo）.在接受Humphrey视野检查5年或更长时间的65例RP患者的126只眼中，68只眼（54%）在随访期间显示MD显著减少。30-2方案中26眼MD进展率为0.11 ~ 3.1dB/年，平均0.93dB/年。10 ~（-2）程序组51眼的平均值为0.64dB/年，范围在0.19 ~ 2.05之间。Humphrey视野计中心10-2程序的MD为-15dB时，大部分患者的视力仍保持良好状态，超过此值后，部分患者视力开始下降。MD为-15 dB的患眼视力较差，视力与矫正模式标准差（CPSD）呈良好的相关性。CPSD值高的眼睛，这意味着视野不均匀受损，在视野中心显示几乎正常的敏感度，而在10度中心视野的外围显示绝对暗点。

Retinitis pigmentosa is caused by various gene abnormality. Because the fundamental treatment for the disease has not been established, the information on the disease progression and visual prognosis may be important for the patients. In the present study, we proposed the method of quantitative evaluation for the disease progression using the automated static perimetry.We studied the perimetry results of patients with RP and analyzed the progression rate of the mean deviation (MD) of Humphrey perimetry program 30-2 and 10-2 using HFA files ver. 5 (BeeLine Office Co. Tokyo). Out of 126 eyes of 65 patients with RP who had been examined using Humphrey perimetry for 5 years or more, 68 eyes (54%) demonstrated statistically significant reduction of MD during the follow-up period. The progression rate of MD in 30-2 program ranged between 0.11 and 3.1 dB/year with the average of 0.93 dB/year in 26 eyes. That for 10-2 program ranged between 0.19 and 2.05 with the average of 0.64 dB/year in 51 eyes.Then we studied the relationship between visual loss and the perimetric progression. Most eyes maintain good visual acuity until the MD of Humphrey perimetry central 10-2 program is -15dB, while after that, some cases start to show decreased visual acuity. Eyes with MD of -15 dB of worse, the visual acuity and corrected pattern standard deviation (CPSD) showed good correlation. Eyes with high value of CPSD, which means unevenly damaged visual field showing almost normal sensitivity at the canter of the field and absolute scotoma in the periphery of the 10-degree central field.