两种具有不同视杆细胞敏感性的视网膜色素变性患者的独特特征。

Takahashi Hiroaki, Miyake Yozo, Horiguchi Masayuki, Tomida Naoki, Takakuwa Hideo

Nagaya Eye Clinic, Aichi, Japan.

Jpn J Ophthalmol. 2005 Mar-Apr;49(2):114-20. doi: 10.1007/s10384-004-0160-1.

PURPOSE

To determine the psychophysical differences between two types of retinitis pigmentosa (RP) patients with different rod sensitivities.

METHODS

Thirty-five RP patients with a visual acuity of > or =0.7 or better were classified by cone-rod perimetry into type 1, those with undetectable rod sensitivity, and type 2, those with measurable rod sensitivity. Their symptoms, age at onset of symptoms, cone and rod sensitivity, and full-field electroretinograms (ERGs) were compared.

RESULTS

The age when the symptoms of night blindness were first noticed was 13.1 +/- 3.3 years (mean +/- SD) for type 1 and 34.5 +/- 14.4 years for type 2 patients (P = 0.0001). One of nine type 1 patients (11%) and 10 of 26 type 2 patients (38%) did not have any symptoms of night blindness. The average rod sensitivity within the central 10 degrees was 43.7 +/- 12.0 dB for type 2 patients with night blindness, and 54.8 +/- 6.4 dB for type 2 patients without night blindness (P = 0.014). One of nine (11%) type 1 patients and 9 of 23 (39%) type 2 patients had recordable ERGs (P = 0.13).

CONCLUSIONS

These findings indicate that the two types of RP patients, distinguished by their rod sensitivity, have different psychophysical characteristics of the visual system. The course of the disease process and the long-term prognosis for these two types of patients are different.

目的

确定两种具有不同视杆敏感度的视网膜色素变性（RP）患者之间的心理物理学差异。

方法

对35名视力≥0.7或更好的RP患者，通过锥-杆视野计分为1型（视杆敏感度无法检测到的患者）和2型（视杆敏感度可测量的患者）。比较他们的症状、症状发作年龄、锥和视杆敏感度以及全视野视网膜电图（ERG）。

结果

1型患者首次注意到夜盲症状的年龄为13.1±3.3岁（平均值±标准差）；2型患者为34.5±14.4岁（P = 0.0001）。9名1型患者中有1名（11%）、26名2型患者中有10名（38%）没有任何夜盲症状。有夜盲症状的2型患者中央10度范围内的平均视杆敏感度为43.7±12.0 dB，无夜盲症状的2型患者为54.8±6.4 dB（P = 0.014）。9名1型患者中有1名（11%）、23名2型患者中有9名（39%）ERG可记录（P = 0.13）。