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目的 探讨外侧膝状体及其后中枢视觉通路受损继发逆行跨突触变性视神经病变（transsynaptic retrograde degeneration of optic neuropathy，TRDON）患者的临床特征，观察视网膜神经纤维层（retinal nerve fiber layer，RNFL）、视网膜神经节细胞层（retinal ganglion cell layer，RGCL）厚度的变化。 方法 本研究为回顾性临床研究。收集2022年9月—2023年9月就诊于西安市第一医院神经内科和神经眼科，经相关检查确诊为外侧膝状体及其后中枢视觉通路受损且符合纳排标准的患者的临床资料。纳入患者均行最佳矫正视力、非接触间接眼压测定、裂隙灯显微镜、眼底照相、相对性传入性瞳孔障碍、视野、色觉、光学相干断层扫描（optical coherence tomography，OCT）和颅脑神经影像检查。对视盘RNFL和黄斑区RGCL进行“容易观察到的TRDON”评估，计算双眼RGCL的归一化不对称评分（normalized asymmetry score，NAS）。 结果 共纳入15例（30只眼）外侧膝状体及其后中枢视觉通路受损患者，年龄42～78岁，中位年龄为58（51～68）岁，发病时间（定义为从出现视觉症状到就诊的时间）为0.1～96.5个月，中位发病时间为12.0（0.5～36.0）个月。11例（73.3%）患者20只（66.7%）眼的黄斑区RGCL符合“容易观察到的TRDON”标准，其中10例患者发病时间均≥5个月，9例患者OCT显示双眼均出现与视野缺损区域相一致的黄斑区RGCL厚度薄变，1例患者OCT显示单眼出现与视野缺损区域相一致的黄斑区RGCL厚度薄变；且9例患者双眼的NAS均为正值，1例患者单眼的NAS为正值。7例（46.7%）患者10只（33.3%）眼的视盘RNFL符合“容易观察到的TRDON”标准，其中3例患者OCT表现为双眼视盘RNFL厚度薄变，4例患者OCT表现为单眼视盘RNFL厚度薄变，但薄变区域与视野缺损区域一致性差。 结论 外侧膝状体及其后中枢视觉通路受损将继发出现TRDON，其特征性表现为OCT显示双眼均出现与视野缺损相一致的黄斑区RGCL厚度薄变，其发生与时间相关（发病5个月后出现）。 Abstract: Objective To investigate the clinical characteristics of patients with transsynaptic retrograde degeneration of optic neuropathy (TRDON) secondary to lateral geniculate body and posterior central visual pathway injury. The thickness changes of the retinal nerve fiber layer (RNFL) and the retinal ganglion cell layer (RGCL) were observed. Methods This study was designed as a retrospective clinical study. From September 2022 to September 2023, clinical data of patients who were diagnosed with the lateral geniculate body and posterior central visual pathway injury through relevant examinations and met the inclusion and exclusion criteria in the Department of Neurology and Neuro-Ophthalmology of the First Hospital of Xi’an were collected. All included patients underwent best corrected visual acuity, non-contact indirect intraocular pressure measurement, slit lamp microscope, fundus photography, relative afferent pupillary defect, visual field, color vision, optical coherence tomography (OCT), and head imaging examination. The “easily observed TRDON” assessment was performed in the optic disc RNFL and macular lutea RGCL, and the normalized asymmetry score (NAS) of the binocular RGCL was calculated. Results A total of 15 cases (30 eyes) with the lateral geniculate body and posterior central visual pathway injury were included. The included patients were aged between 42 and 78 years, with a median age of 58 (51-68) years. The time of onset (defined as the time from the appearance of visual symptoms to the time of seeing a doctor) ranged from 0.1 to 96.5 months, and the median time of onset was 12.0 (0.5-36.0) months. In 11 cases (73.3%) of patients [20 eyes (66.7%)], the macular lutea RGCL met the criteria of “easily observed TRDON”. Among them, the onset time for 10 cases was≥5 months. Nine cases of OCT showed thinning of the macular lutea RGCL in both eyes, consistent with the areas of visual field defects. One case of OCT showed thinning of the macular lutea RGCL in single eye, consistent with the areas of visual field defects. Moreover, both eyes of the nine cases had positive NAS values, and one eye of the one case had a positive NAS value. Seven patients (46.7%) [10 eyes (33.3%)] met the criteria of “easily observed TRDON” in the optic disc RNFL. Among them, three patients showed thinning of the optic disc RNFL in both eyes on OCT, while four patients showed thinning of the optic disc RNFL in only one eye. However, the correlation between the areas of thinning and the areas of visual field defects was poor. Conclusions TRDON was secondary to the lateral geniculate body and posterior central visual pathway injury, which is characterized by thin thickness of RGCL in the macular lutea in both eyes, consistent with the visual field defects. The occurrence of TRDON is time-related, typically manifesting after 5 months from the disease onset. |
