摘要： 目的 初步总结视神经脊髓炎谱系疾病患者临床和影像学特点，回顾分析其血清和脑脊液自身抗体表达变化，为临床选择合理诊断方法提供参考。方法与结果 共10 例视神经脊髓炎谱系疾病患者，头部或脊髓MRI检查显示病灶分别位于脑干（3 例）、颈髓（3 例）、胸髓（6 例）或颈胸髓（1 例）。其中血清NMO-IgG 阳性3/8 例、抗核抗体阳性1/3 例、甲状腺球蛋白和甲状腺过氧化物酶抗体阳性2/3 例、甲状腺功能减退2 例和甲状腺功能亢进1 例、同型半胱氨酸升高2 例。结论 视神经脊髓炎谱系疾病好发于青壮年，女性多见，对高度怀疑视神经脊髓炎谱系疾病的患者应行血清和脑脊液自身抗体，以及头部和脊髓MRI 检查，为明确诊断和合理治疗提供临床依据。缓解期对自身抗体阳性病例进行实验室指标复查，能够明确自身抗体与视神经脊髓炎谱系疾病之间的关系。

关键词: 视神经脊髓炎, 磁共振成像, 水通道蛋白质4

Abstract: Objective  To observe the clinical and imaging characteristics and the changes of autoimmune antibodies in the serum and cerebrospinal fluid (CSF) of patients with neuromyelitis optica spectrum disorders (NMOSDs).  Methods  The data of 10 patients with NMOSDs in Aviation General Hospital of China Medical University from January 2011 to June 2014 were collected. The clinical and imaging features were retrospectively reviewed, and NMO-IgG in serum and CSF, anti-nuclear antibody (ANA), homocysteine (Hcy) and thyroid function were analyzed.  Results  Cranial and spinal MRI of these patients showed that brain stem was involved in 3 cases, cervical cord in 3 cases, thoracic cord in 6 cases, and cervical-thoracic cord in one case. Serum NMO-IgG were tested in 8 cases, among whom 3 patients were positive (3/8) and 5 were negative (5/8). ANA was positive in one case (1/3), and thyroglobulin (TG) antibody and thyroid peroxidase (TPO) antibody were positive in 2 cases (2/3). Hypothyroidism occured in 2 cases, hyperthyroidism occured in one case, and Hcy rised in 2 cases.  Conclusions  NMOSDs frequently occur in young and middle-aged women. Patients who were highly suspected with NMOSDs should receive tests of autoimmune antibodies in the serum and CSF, and cranial and spinal MRI examination, in order to make a definite diagnosis and receive appropriate treatment. Retesting the autoimmune antibodies should be done in catabasis, in order to identify the relationship between autoimmune antibodies and NMOSDs.

Key words: Neuromyelitis optica, Magnetic resonance imaging, Aquaporin 4