Clinicopathological features in anterior visual pathway in neuromyelitis optica
Clinicopathological features in anterior visual pathway in neuromyelitis optica
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DOI：
10.1002/ana.24608
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发表时间：
2016-04-01
影响因子：
11.2
通讯作者：
Kawachi, Izumi
中科院分区：
文献类型：
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作者：
Hokari, Mariko;Yokoseki, Akiko;Kawachi, Izumi
视神经肌萎缩症谱系障碍（NMOsd）是以水通道蛋白-4（AQP 4）自身抗体为特征的中枢神经系统自身免疫性疾病。本研究的目的是阐明参与的前部视觉通路（AVP）和神经退行性变通过胶质细胞-神经元的相互作用在NMOsd.MethodsThirty日本患者血清学验证NMOsd进行了评估与神经眼科研究的特点。使用27个组织块从13个其他情况下的NMOsd，我们进行了神经病理学分析的胶质细胞和神经轴突参与的AVP。结果AVP参与NMOsd的特点是，相比多发性硬化症：（1）纵向广泛的视神经炎（ON），（2）更严重的视力损害和预后差的ON;（3）独特的AQP 4动力学，包括在ON病变中星形胶质细胞上AQP 4免疫反应性的丧失与补体激活，在视网膜中Muller细胞上AQP 4免疫反应性的丧失与无补体沉积，在视神经和视网膜神经纤维层（RNFL）继发性顺/逆行变性的胶质增生中，星形胶质细胞上的AQP 4免疫反应密集;和（4）更严重的神经变性，包括在ON病变中具有补体依赖性星形胶质细胞病理学的变性线粒体和瞬时受体电位melastatin 4通道的轴突积累，水平细胞的轻度损失，以及RNFL变薄和具有大量AQP 4（+）星形胶质细胞的神经节细胞的损失，说明ON后继发性退行性变性。解释严重和广泛的神经轴突损伤和星形胶质细胞/Muller细胞的独特动力学与AQP 4的改变在AVP中是突出的，并且可能与NMOsd中的不良视觉功能和预后相关。神经学年鉴2016;79：605-624
ObjectiveNeuromyelitis optica spectrum disorder (NMOsd) is an autoimmune disorder of the central nervous system characterized by aquaporin-4 (AQP4) autoantibodies. The aim of this study was to elucidate the characteristics of involvement of the anterior visual pathway (AVP) and neurodegeneration via glia-neuron interaction in NMOsd.MethodsThirty Japanese patients with serologically verified NMOsd were assessed with a neuro-ophthalmological study. Using 27 tissue blocks from 13 other cases of NMOsd, we performed neuropathological analysis of glial and neuroaxonal involvement in the AVP.ResultsThe AVP involvement in NMOsd was characterized by the following, compared to multiple sclerosis: (1) longitudinally extensive optic neuritis (ON); (2) more severe visual impairment and worse prognosis for ON; (3) unique AQP4 dynamics, including loss of AQP4 immunoreactivity on astrocytes with complement activation in ON lesions, loss of AQP4 immunoreactivity on Muller cells with no deposition of complement in the retinas, and densely packed AQP4 immunoreactivity on astrocytes in gliosis of secondary anterograde/retrograde degeneration in the optic nerves and retinal nerve fiber layer (RNFL); and (4) more severe neurodegeneration, including axonal accumulation of degenerative mitochondria and transient receptor potential melastatin 4 channel with complement-dependent astrocyte pathology in ON lesions, mild loss of horizontal cells, and RNFL thinning and loss of ganglion cells with abundance of AQP4(+) astrocytes, indicating secondary retrograde degeneration after ON.InterpretationSevere and widespread neuroaxonal damage and unique dynamics of astrocytes/Muller cells with alterations of AQP4 were prominent in the AVP and may be associated with poor visual function and prognosis in NMOsd. Ann Neurol 2016;79:605-624