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患者的临床特征

doi:10.3969/j.issn.1673-5765.2024.11.003

摘要/Abstract

摘要： 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）。

文章导读： 外侧膝状体及其后中枢视觉通路受损将继发出现逆行跨突触变性视神经病变，其特征性表现为光学相干断层扫描显示双眼均出现与视野缺损相一致的黄斑区视网膜神经节细胞层厚度薄变；逆行跨突触变性视神经病变发生与时间相关，多在发病5个月后出现。

关键词: 逆行跨突触; 变性; 视神经病变; 逆行跨突触变性视神经病变; 外侧膝状体; 中枢视觉通路

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.

Key words: Transsynaptic retrograde; Degeneration; Optic neuropathy; Transsynaptic retrograde degeneration of optic neuropathy; Lateral geniculate body; Central visual pathway

中图分类号:

霍燕, 蔺雪梅, 刘仲仲, 饶兰平, 宋沉生, 刘佩, 逯青丽, 王静, 吴松笛. 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患者的临床特征[J]. 中国卒中杂志, 2024, 19(11): 1268-1276.

HUO Yan, LIN Xuemei, LIU Zhongzhong, RAO Lanping, SONG Chensheng, LIU Pei, LU Qingli, WANG Jing, WU Songdi. Clinical Characteristics of Patients with Transsynaptic Retrograde Degeneration of Optic Neuropathy Secondary to Lateral Geniculate Body and Posterior Central Visual Pathway Injury[J]. Chinese Journal of Stroke, 2024, 19(11): 1268-1276.

参考文献

[1] DINKIN M. Trans-synaptic retrograde degeneration in the human visual system：slow，silent，and real[J/OL]. Curr Neurol Neurosci Rep，2017，17（2）：16[2024-01-02]. https://doi.org/10.1007/s11910-017-0725-2.
[2] BEATTY R M，SADUN A A，SMITH L，et al. Direct demonstration of transsynaptic degeneration in the human visual system：a comparison of retrograde and anterograde changes[J]. J Neurol Neurosurg Psychiatry，1982，45（2）：143-146.
[3] 霍燕，蔺雪梅，吴松笛. 逆行跨突触变性视神经病变的研究进展[J]. 中华眼底病杂志，2022，38（12）：1035-1038.
HUO Y，LIN X M，WU S D. Advances in transsynaptic retrograde degeneration of optic neuropathy[J]. Chin J Ocul Fundus Dis，2022，38（12）：1035-1038.
[4] MEIER P G，MAEDER P，KARDON R H，et al. Homonymous ganglion cell layer thinning after isolated occipital lesion：macular OCT demonstrates transsynaptic retrograde retinal degeneration[J]. J Neuroophthalmol，2015，35（2）：112-116.
[5] SCHWARTZ S G，MONROIG A，FLYNN H W，Jr. Progression of transsynaptic retinal degeneration with spectral-domain optical coherence tomography[J/OL]. Am J Ophthalmol Case Rep，2017，5：67-72[2024-01-02]. https://doi.org/10.1016/j.ajoc.2016.12.010.
[6] MITCHELL J R，OLIVEIRA C，TSIOURIS A J，et al. Corresponding ganglion cell atrophy in patients with postgeniculate homonymous visual field loss[J]. J Neuroophthalmol，2015，35（4）：353-359.
[7] VANBUREN J M. Trans-synaptic retrograde degeneration in the visual system of primates[J]. J Neurol Neurosurg Psychiatry，1963，26（5）：402-409.
[8] LENNARTSSON F，NILSSON M，FLODMARK O，et al. Injuries to the immature optic radiation show correlated thinning of the macular ganglion cell layer[J/OL]. Front Neurol，2018，9：321[2024-01-02]. https://doi.org/10.3389/fneur.2018.00321.
[9] BALK L J，STEENWIJK M D，TEWARIE P，et al. Bidirectional trans-synaptic axonal degeneration in the visual pathway in multiple sclerosis[J]. J Neurol Neurosurg Psychiatry，2015，86（4）：419-424.
[10] KELLER J，SÁNCHEZ-DALMAU B F，VILLOSLADA P. Lesions in the posterior visual pathway promote trans-synaptic degeneration of retinal ganglion cells[J/OL]. PLoS One，2014，9（5）：e97444[2024-01-01]. https://doi.org/10.1371/journal.pone.0097444.
[11] YAMASHITA T，MIKI A，GOTO K，et al. Evaluation of significance maps and the analysis of the longitudinal time course of the macular ganglion cell complex thicknesses in acquired occipital homonymous hemianopia using spectral-domain optical coherence tomography[J]. Neuroophthalmology，2020，44（4）：236-245.
[12] GOTO K，MIKI A，YAMASHITA T，et al. Sectoral analysis of the retinal nerve fiber layer thinning and its association with visual field loss in homonymous hemianopia caused by post-geniculate lesions using spectral-domain optical coherence tomography[J]. Graefes Arch Clin Exp Ophthalmol，2016，254（4）：745-756.
[13] MÜHLEMANN F，GRABE H，FOK A，et al. Homonymous hemiatrophy of ganglion cell layer from retrochiasmal lesions in the visual pathway[J/OL]. Neurology，2020，94（3）：e323-e329[2024-01-02]. https://doi.org/10.1212/WNL.0000000000008738.
[14] MEIER P，MAEDER P，BORRUAT F X. Transsynaptic retrograde degeneration：clinical evidence with homonymous RGCL loss on OCT[J]. Klin Monbl Augenheilkd，2016，233（4）：396-398.
[15] VIEN L，DALPORTO C，YANG D. Retrograde degeneration of retinal ganglion cells secondary to head trauma[J]. Optom Vis Sci，2017，94（1）：125-134.
[16] SCHWARTZ S G，PASOL J，LAM B L，et al. Spectral-domain optical coherence tomography documentation of transsynaptic retinal degeneration[J]. Ophthalmic Surg Lasers Imaging Retina，2016，47（8）：768-772.
[17] GUEDES M E，ALMEIDA A C，PATRICIO M S，et al. Acquired retrograde transsynaptic degeneration[J/OL]. BMJ Case Rep，2011，2011[2024-01-02]. https://doi.org/10.1136/bcr.08.2011.4653.
[18] PATEL K R，RAMSEY L E，METCALF N V，et al. Early diffusion evidence of retrograde transsynaptic degeneration in the human visual system[J]. Neurology，2016，87（2）：198-205.
[19] PELLEGRINI F，INTERLANDI E，PICHI F，et al. Retrogeniculate lesion of the visual pathways：retinal optical coherence tomography angiography shows evidence of transsynaptic retrograde degeneration[J]. Neuroophthalmology，2020，44（2）：114-117.
[20] JAUMANDREU L，SÁNCHEZ-GUTIÉRREZ V，MUÑOZ-NEGRETE F J，et al. Reduced peripapillary and macular vessel density in unilateral postgeniculate lesions with retrograde transsynaptic degeneration[J]. J Neuroophthalmol，2019，39（4）：462-469.
[21] CELIK BUYUKTEPE T，BINGÖL KıZıLTUNÇ P，BUYUKTEPE M，et al. Direct and transsynaptic retrograde degeneration and optic nerve head microvascular changes in patients with hemianopia[J]. Eur J Ophthalmol，2021，31（4）：1785-1794.