Jacobson D M, Stone E M, Miller N R, Pollock S C, Fletcher W A, McNussen P J, Martin T J
Department of Neurology, Marshfield Clinic, Wisconsin 54449, USA.
Am J Ophthalmol. 1998 Aug;126(2):291-5. doi: 10.1016/s0002-9394(98)00152-4.
It has been suggested that the pupillary light reaction is relatively preserved in the affected eyes of patients with Leber hereditary optic neuropathy (LHON). To test the hypothesis that visual-pupillomotor dissociation exists in LHON, we performed a retrospective study to evaluate the magnitude of the relative afferent pupillary defect (RAPD) in patients who had experienced monocular visual loss. We also compared the size of the measured RAPD with the size of the RAPD that would be expected on the basis of documented visual field loss.
We identified a cohort of patients with LHON and monocular visual loss, whose pupillary reactions had been quantified using neutral density filters. From a review of the case records, we determined whether an RAPD was present, as well as the magnitude of the documented RAPDs. We also calculated the expected size of the RAPD for each patient, using previously established templates that correlated the size of the RAPD with the degree of visual field loss.
An RAPD was identified in all 10 patients in this study. There was no significant difference between the size of the measured and predicted RAPD, nor did the size of the RAPD correlate with visual acuity or the time interval between the onset of visual loss and evaluation.
The results of this study do not support the hypothesis that visual-pupillomotor dissociation is a common feature of LHON.
有人提出，在Leber遗传性视神经病变（LHON）患者的患眼中，瞳孔对光反应相对保留。为了验证LHON中存在视-瞳孔运动分离这一假说，我们进行了一项回顾性研究，以评估单眼视力丧失患者相对传入性瞳孔障碍（RAPD）的程度。我们还将测得的RAPD大小与根据记录的视野缺损预期的RAPD大小进行了比较。
我们确定了一组LHON和单眼视力丧失的患者，他们的瞳孔反应已使用中性密度滤光片进行了量化。通过查阅病例记录，我们确定是否存在RAPD以及记录的RAPD的程度。我们还使用先前建立的将RAPD大小与视野缺损程度相关联的模板，计算了每位患者预期的RAPD大小。
本研究中的所有10例患者均发现存在RAPD。测得的RAPD大小与预测的RAPD大小之间无显著差异，RAPD大小也与视力或视力丧失发作与评估之间的时间间隔无关。
本研究结果不支持视-瞳孔运动分离是LHON常见特征这一假说。
