1 Department of Ophthalmology, Muhimbili University of Health and Allied Sciences, Box 65001, Dar es Salaam,Tanzania.
Brain. 2013 Nov;136(Pt 11):3418-26. doi: 10.1093/brain/awt221. Epub 2013 Sep 9.
Bilateral optic neuropathy in Dar es Salaam is now considered endemic and is estimated to affect 0.3-2.4% of young adults. The condition is characterized by a subacute bilateral loss of central vision of unknown aetiology. Findings of spectral domain optical coherence tomography have not previously been reported for these patients. All patients diagnosed with endemic optic neuropathy over a 2-year period at the Muhimbili National Hospital underwent spectral domain optical coherence tomography macular imaging. Scans were graded qualitatively for severity of retinal nerve fibre layer loss as well as the presence of microcystic macular changes, which have not previously been described in this condition. Of the 128 patients included (54.7% male; median age 20 years), severe retinal nerve fibre layer loss was found in 185 eyes (74.0%). There was full concordance in retinal nerve fibre layer thickness between the two eyes in 113 (91.1%) patients. Microcystic macular spaces were found in 16 (12.5%) patients and were bilateral in nine (7.0%) individuals. These changes were typically more prominent in the nasal than the temporal macula, predominantly involving the inner nuclear layer, and often occurred in an annular configuration that was evident on en face infra-red imaging, though not discernible on colour fundus photography or clinically. All patients with microcystic macular changes had severe thinning of the retinal nerve fibre layer (P = 0.02). Four patients in whom cystic spaces were demonstrated had sequential scans, and there was no detectable alteration in the configuration of these changes over a period of up to 16 months. This is the first study to document optical coherence tomography findings in endemic optic neuropathy. We have observed symmetrical severe loss of the caeco-central projection (papillomacular bundle) with otherwise well-preserved macular architecture. Also, we have observed microcystic retinal changes in a significant proportion of patients, which were associated with severe retinal nerve fibre layer loss. Similar changes have recently been reported from optical coherence tomography images of patients with multiple sclerosis, relapsing isolated optic neuritis, dominant optic atrophy, Leber's hereditary optic neuropathy and a patient with a chronic compressive optic neuropathy, supporting the hypothesis that this may be a non-specific phenomenon secondary to ganglion cell death. The correspondence of the changes to an annulus discernible on infra-red en face imaging, but not using other conventional retinal imaging techniques highlights the potential usefulness of this modality.
达累斯萨拉姆的双侧视神经病变现在被认为是地方性的,据估计影响了 0.3-2.4%的年轻成年人。这种疾病的特征是亚急性双侧中央视力丧失,病因不明。以前尚未报道过这些患者的光谱域光相干断层扫描结果。在穆希比利国家医院,在两年期间被诊断为地方性视神经病变的所有患者都接受了光谱域光相干断层扫描黄斑成像。扫描结果根据视网膜神经纤维层丢失的严重程度和微囊样黄斑改变的存在情况进行定性分级,这些改变以前在这种情况下没有描述过。在纳入的 128 名患者中(54.7%为男性;中位年龄为 20 岁),185 只眼(74.0%)发现严重的视网膜神经纤维层丢失。在 113 名(91.1%)患者中,双眼的视网膜神经纤维层厚度完全一致。16 名(12.5%)患者有微囊样黄斑间隙,9 名(7.0%)患者为双侧。这些改变在鼻侧比颞侧更明显,主要累及内核层,常呈环形排列,在共面红外成像上可见,但在彩色眼底摄影或临床检查上不可见。所有有微囊样黄斑改变的患者均有严重的视网膜神经纤维层变薄(P=0.02)。在有囊性空间显示的 4 名患者中进行了连续扫描,在长达 16 个月的时间内,这些改变的形态没有可检测到的变化。这是第一项记录地方性视神经病变的光相干断层扫描结果的研究。我们观察到对称性的严重中心旁视野(乳头黄斑束)丧失,而其他黄斑结构完好。此外,我们还观察到相当一部分患者存在微囊样视网膜改变,这些改变与严重的视网膜神经纤维层丧失有关。最近在多发性硬化症、复发性孤立性视神经炎、显性视神经萎缩、Leber 遗传性视神经病变和一名慢性压迫性视神经病变患者的光相干断层扫描图像中也报告了类似的改变,支持了这可能是一种非特异性现象,继发于节细胞死亡的假说。这些改变与在共面红外成像上可辨别的环形物相对应,但使用其他常规视网膜成像技术无法识别,这突出了该模态的潜在有用性。