Aquaporin-4 astrocytopathy in Baló’s disease
Aquaporin-4 astrocytopathy in Baló’s disease
复制标题
Baló 病中的 Aquaporin-4 星形细胞病
作者：
T. Matsuoka;Satoshi O. Suzuki;T. Iwaki;T. Tabira;A. Ordinario;J. Kira
巴洛同心圆硬化症（BCS）被认为是多发性硬化症的一种罕见变体，其特征是脱髓鞘和保留髓鞘层的交替环。BCS的潜在机制仍有待阐明。最近，发生同心环的Baló被描述在脑干的患者与视神经肌萎缩症（NMO）。由于选择性的水通道蛋白-4（AQP 4）的丢失和补体和免疫球蛋白的血管中心性沉积是NMO的特征，我们的目的是评估BCS患者同心性脱髓鞘病变中AQP 4的表达。我们评估了AQP 4表达相对于另一个星形胶质细胞标志物（胶质细胞酸性蛋白）的表达，脱髓鞘的程度，病变分期和血管周围沉积的补体和免疫球蛋白在4例BCS和30个人与其他神经系统疾病。所有BCS病例在所有活动性脱髓鞘病变的脱髓鞘和有髓鞘层均表现出广泛的AQP 4缺失，伴有T细胞的血管周围淋巴细胞袖套，但血管周围无免疫球蛋白或补体沉积。这些发现表明，AQP 4丢失发生在异质性脱髓鞘条件下，即NMO和BCS。此外，急性BCS病变的特征是广泛的AQP 4损失，没有补体或免疫球蛋白的血管中心沉积。
Baló’s concentric sclerosis (BCS) is considered to be a rare variant of multiple sclerosis and characterized by alternating rings of demyelinated and preserved myelin layers. The mechanism underlying BCS remains to be elucidated. Recently, occurrence of concentric rings of Baló was described in the brainstem of a patient with neuromyelitis optica (NMO). Because selective loss of aquaporin-4 (AQP4) and vasculocentric deposition of complement and immunoglobulins are characteristic in NMO, we aimed to assess AQP4 expression in the concentric demyelinating lesions of BCS patients. We evaluated AQP4 expression relative to expression of another astrocytic marker (glial fibrillary acidic protein), the extent of demyelination, lesion staging and perivascular deposition of complement and immunoglobulin in four cases with BCS, and 30 individuals with other neurological diseases. All cases with BCS demonstrated extensive AQP4 loss in both demyelinated and myelinated layers of all actively demyelinating lesions, with perivascular lymphocytic cuffing of T cells, but no deposition of immunoglobulins or complement around vessels. These findings suggest that AQP4 loss occurs in heterogeneous demyelinating conditions, namely NMO and BCS. Furthermore, acute BCS lesions are characterized by extensive AQP4 loss without vasculocentric deposition of complement or immunoglobulin.
影响因子：
14.5
作者：
Saadoun, Samira;Waters, Patrick;Papadopoulos, Marios C.
通讯作者：
Papadopoulos, Marios C.
影响因子：
14.5
作者：
Lucchinetti, CF;Mandler, RN;Lassmann, H
通讯作者：
Lassmann, H
影响因子：
14.5
作者：
Roemer, Shanu F.;Parisi, Joseph E.;Lucchinetti, Claudia F.
通讯作者：
Lucchinetti, Claudia F.