Reappraisal of Aquaporin-4 Astrocytopathy in Asian Neuromyelitis Optica and Multiple Sclerosis Patients
Reappraisal of Aquaporin-4 Astrocytopathy in Asian Neuromyelitis Optica and Multiple Sclerosis Patients
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DOI：
10.1111/j.1750-3639.2011.00475.x
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发表时间：
2011-09-01
期刊：
影响因子：
6.4
通讯作者：
Kira, Jun-ichi
中科院分区：
文献类型：
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作者：
Matsuoka, Takeshi;Suzuki, Satoshi O.;Kira, Jun-ichi
选择性水通道蛋白-4（AQP 4）缺失和血管中心性补体及免疫球蛋白沉积是视神经肌萎缩症（NMO）的特征。我们最近报道了Balo病损的脱髓鞘和有髓鞘层中广泛的AQP 4丢失，而无血管周围免疫球蛋白和补体沉积。我们的目的是重新评估水通道蛋白4在NMO和多发性硬化（MS）的表达模式。我们评估了11例NMO和NMO谱系疾病（NMOSD）、5例MS和30例其他神经系统疾病中AQP 4表达与胶质细胞酸性蛋白、脱髓鞘程度、病变分期（巨噬细胞CD 68染色）和补体和免疫球蛋白血管周围沉积的关系。病变分为活动性脱髓鞘（n = 66），慢性活动性（n = 86），慢性非活动性（n = 48）和未分类（n = 12）。6例NMO/NMOSD和2例MS病例在脱髓鞘区以外显示优先AQP 4丢失，与病变分期无关。5例NMO和3例MS病例显示，即使在活动性脱髓鞘病变中，尽管有严重的组织破坏，也存在AQP 4保留。补体和免疫球蛋白的血管中心性沉积仅在NMO/NMOSD患者中检测到，少于30%的活动性脱髓鞘病变显示AQP 4丢失。我们目前和以前的研究结果表明，抗体非依赖性AQP 4丢失可以发生在异质性脱髓鞘疾病，包括NMO，Balo病和MS。
Selective aquaporin-4 (AQP4) loss and vasculocentric complement and immunoglobulin deposition are characteristic of neuromyelitis optica (NMO). We recently reported extensive AQP4 loss in demyelinated and myelinated layers of Balo's lesions without perivascular immunoglobulin and complement deposition. We aimed to reappraise AQP4 expression patterns in NMO and multiple sclerosis (MS). We evaluated AQP4 expression relative to glial fibrillary acidic protein, extent of demyelination, lesion staging (CD68 staining for macrophages), and perivascular deposition of complement and immunoglobulin in 11 cases with NMO and NMO spectrum disorders (NMOSD), five with MS and 30 with other neurological diseases. The lesions were classified as actively demyelinating (n = 66), chronic active (n = 86), chronic inactive (n = 48) and unclassified (n = 12). Six NMO/NMOSD and two MS cases showed preferential AQP4 loss beyond the demyelinated areas, irrespective of lesion staging. Five NMO and three MS cases showed AQP4 preservation even in actively demyelinating lesions, despite grave tissue destruction. Vasculocentric deposition of complement and immunoglobulin was detected only in NMO/NMOSD patients, with less than 30% of actively demyelinating lesions showing AQP4 loss. Our present and previous findings suggest that antibody-independent AQP4 loss can occur in heterogeneous demyelinating conditions, including NMO, Balo's disease and MS.