视神经脊髓炎的临床及磁共振成像研究

Clinical and brain MRI appearances in neuromyelitis optica

目的探讨视神经脊髓炎临床与MRI表现特点。方法回顾分析20例经临床或实验室检查明确诊断的视神经脊髓炎患者临床资料,总结其发病特点。结果视神经脊髓炎多以急性脊髓炎(13例)或视神经炎(7例)为首发症状,复发病程(18例)多见。临床主要表现为急性视力减退、视野呈向心性缩小或复视(7例),以及病灶平面以下运动、感觉和自主神经功能紊乱(13例)。部分患者眼底呈急性视神经炎、视乳头水肿(3例),球后视神经炎(5例)或视神经萎缩(4例)变化;视觉诱发电位呈现潜伏期延长、波幅下降和波形分化不良(7例)。脑脊液和血清免疫球蛋白IgG阳性检出率分别为44.44%(8/18)和50.00%(6/12),寡克隆区带阳性率为27.78%(5/1 8)。8例患者头部MRI检查显示存在颅内病灶,分布于下丘脑、脑桥、中脑、第四脑室周围。结论实验室检查有助于视神经脊髓炎的诊断与鉴别诊断,第三、第四脑室周围和脑桥等病灶分布具有特征性,与脑内水通道蛋白4高表达区域相一致。

Objective To explore clinical features, brain magnetic resonance imaging （MRI） findings in neuromyelitis optica （NMO）. Methods Twenty patients with NMO proved by clinical history or laboratory examinations were reviewed. Results Thirteen cases initially presented with acute myelitis, 7 cases with optic neuritis. Among all patients, 18 cases had a relapsing form of the disease. The main clinical manifestations included acute vision loss, concentric narrowing visual field or double vision （7 cases）, muscle weakness, reduced sensation below the level of lesions, or autonomic dysfunction （13 cases）. Ophthalmoscopy showed acute optic neuritis, papilledema （3 cases）, retrobulbar optic neuritis （5 cases）, or optic atrophy （4 cases）. Immunoglobulin IgG-24 synthetic rate rose in 8 patients （8/18）, serum NMO-IgG was positive in 6 cases （6/12）, and oligoclonal bands （OCB） presented positive in 5 patients （5/18）. Seven cases with optic neuritis showed extended P100 latency and （or） poor waveforms differentiation. Brain MRI was positive in 8 patients, and lesions typically localized in hypothalamus, pons, midbrain or periventricle areas. Conclusion , Laboratory tests may contribute to the diagnosis and differential diagnosis of NMO. Hypothalamus, brainstem, pons or periventricle lesions, corresponded to high sites of aquaporin 4 （AQP4） in the brain, are typical features of NMO.