[A case of anti-AQP4 antibody-positive recurrent myelitis overlapped with autoimmune disorders including incomplete CREST syndrome revealed multiple discontinuous cord lesions].
[A case of anti-AQP4 antibody-positive recurrent myelitis overlapped with autoimmune disorders including incomplete CREST syndrome revealed multiple discontinuous cord lesions].
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[一例抗 AQP4 抗体阳性复发性脊髓炎与自身免疫性疾病重叠，包括不完全 CREST 综合征，显示多发不连续脊髓病变]。
DOI：
10.5692/clinicalneurol.49.115
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发表时间：
2009
期刊：
影响因子：
--
通讯作者：
S. Matsumoto
中科院分区：
文献类型：
--
作者：
Makio Takahashi;R. Nagata;A. Ozaki;S. Kaneko;H. Saiki;S. Matsumoto
据报道，一名 65 岁女性患有多种自身免疫性疾病，包括不完全 CREST 重叠与水通道蛋白 4 (AQP4) 抗体阳性复发性脊髓炎。她在临床上还患有干燥综合征和原发性胆汁性肝硬化（PBC）。 C4以下感觉迟钝，轻度运动无力，双下肢反射亢进，无病理反射。 T2 加权 MRI 显示 C1-5 和 T7/8 处有多处不连续的脊髓病变。视觉诱发电位研究揭示了双侧 P100 潜伏期延长。她临床上不仅表现为不完全CREST综合征（面部毛细血管扩张、双侧手指硬化和雷诺现象），还表现为干燥综合征（Sjögren）和PBC（黄疸）。免疫血清学研究显示，她的抗核、抗着丝粒和抗 AQP4（= NMO-IgG）抗体呈阳性。皮质类固醇和血浆置换的联合疗法对所有临床症状均有效。因此，本病例强调当临床诊断出复发性脊髓炎时，抗 AQP 4 抗体与其他重叠的自身免疫性疾病（例如 CREST 综合征）的相关性。
A 65-year-old woman presenting with multiple autoimmune disorders including incomplete CREST overlapping with aquaporin 4 (AQP4) antibody-positive recurrent myelitis was reported. She also clinically suffered from Sjogren syndrome and primary biliary cirrhosis (PBC). She had dysesthesia below C4 level, mild motor weakness and hyperreflexia without pathological reflexes on bilateral lower extremities. A T2-weighted MRI indicated multiple discontinuous spinal cord lesions at C1-5 and T7/8. A visual evoked potential study disclosed bilateral prolonged latency of P100. She clinically manifested not only incomplete CREST syndrome (facial teleangiectasia, sclerodactyly in bilateral fingers, and Raynaud's phenomenon), but also Sjögren (sicca syndrome) and PBC (jaundice). Immunoserological study showed that she was positive for anti-nuclear, anti-centromere, and anti-AQP4 (= NMO-IgG) antibodies. A combination therapy with corticosteroid and plasmapheresis was effective for all clinical symptoms. Therefore, this case stresses on the relevance of anti-AQP 4 antibody to the other overlapping autoimmune disorders, such as CREST syndrome, when recurrent myelitis is clinically diagnosed.
影响因子：
14.5
作者：
Matsuoka, Takeshi;Matsushita, Takuya;Kira, Jun-ichi
通讯作者：
Kira, Jun-ichi
影响因子：
14.5
作者：
Misu, T.;Fujihara, K.;Itoyama, Y.
通讯作者：
Itoyama, Y.