诊疗及预后

CSF electrophoresis demonstrated oligoclonal bands with a high IgG index. AQP4-Ab was positive (1:10) both in blood and CSF. Thoracic spine MRI studies did not reveal any abnormality. A diagnosis of neuromyelitis optica spectrum disorder was made based on Wingerchuk’s criteria (myelitis associated with brain lesions typical of neuromyelitis optica).

 Intravenous of methylprednisolone (1 g/day) was initiated and then converted to oral after 5 days. Azathioprine was then added. She gradually recovered 3 days after treatment and could walk by herself 20 days later.

脑脊液电泳显示寡克隆条带并IgG指数增高的。AQP4-抗体在血和脑脊液中均呈阳性(1:10)。胸椎MRI检查未发现任何异常。按照Wingerchuk标准明确诊断为视神经脊髓炎谱系障碍(脊髓炎合并典型的视神经脊髓炎脑部病变)。开始静脉滴注甲基强的松龙1g/d，5d后转为口服。然后加硫唑嘌呤。治疗3天后逐渐恢复，20天后可自主行走。

Cerebral MRI performed 7 months later did not show any new lesions, and the dorsolateral medullary lesion had significantly reduced in size.  She has not experienced any clinical relapses during the follow-up period.

7个月后进行的脑部MRI检查没有发现任何新的病灶，而且髓质背外侧病灶的大小明显缩小。在随访期间，她没有出现任何临床复发。

NMOSD is a rare autoimmune disease marked by inflammation in the spinal cord and the optic nerve — the nerve that transmits signals between the eyes and brain. In some patients, the disease also may affect the brain, including the brainstem, the bottom portion of the brain that connects it to the spinal cord.

However, NMOSD symptoms can have similar features to those of other neurological diseases, which may lead to a misdiagnosis for some patients. Lab tests are a standard procedure to help distinguish the disorder, given that most patients are positive for antibodies that target the aquaporin-4 (AQP4) protein.

NMOSD 是一种罕见的自身免疫性疾病，其特征是脊髓和视神经出现炎症。在某些患者中，这种疾病还可能影响大脑，包括脑干。

然而，NMOSD 的症状可能与其他神经系统疾病的症状有相似之处（包括但不限于卒中），这可能会导致一些患者被误诊。鉴于大多数患者针对水通道蛋白-4（AQP4）的抗体呈阳性，因此实验室检测是帮助鉴别该疾病的标准操作。

7预后复查MRI如下图