急性进行性眼肌麻痹四例临床分析并文献复习  

Clinical analysis of 4 patients with acute progressive ophthalmoplegia and literature review

作　　者：沈飞飞[1] 张克忠[1] 张利[1] 丛晓银[1] 陈洁[1] 郑锡凤[1] 许安顺 苏兵[1] 

机构地区：[1]南京医科大学第一附属医院神经内科,南京210029

出　　处：《中华神经医学杂志》2015年第4期519-522,共4页Chinese Journal of Neuromedicine

摘　　要：目的探讨以急性进行性眼肌麻痹为突出表现的患者临床诊断、鉴别诊断及其预后。方法对选取自南京医科大学第一附属医院神经内科2014年1至3月收治的以急性进行性眼肌麻痹为突出临床症状的4例患者的临床表现、辅助检查结果、诊治经过与预后作回顾性分析，并复习相关文献。结果4例患者以全眼肌麻痹为核心表现，合并面神经麻痹、脊神经麻痹等。其中2例为脑膜癌病（MC），脑膜刺激征阳性，脑脊液细胞学检查可见异形细胞，均放弃治疗，1例很快死亡，1例进行性恶化；1例为毕式脑干脑炎（BBE），合并意识障碍及延髓、副神经麻痹，经双重血浆置换及激素治疗后明显改善，目前症状基本消失；1例为Miller-Fisher综合征合并格林巴利综合征，共济失调明显且合并双侧面瘫，静脉丙球蛋白冲击治疗5d，3周后症状基本消失，目前症状及体征完全消失。结论MC、BBE、Miller Fisher综合征（MFS）、格林巴利综合征（GBS）均可以急性眼肌麻痹为突出症状，可合并其他颅神经、脊神经麻痹症状。脑脊液检查在明确诊断中发挥着重要作用。MC预后极差。其余2种疾病经血浆置换、静脉丙球蛋白冲击等治疗后预后良好。Objective To investigate the clinical diagnosis, differentiate diagnosis and prognosis of patients with acute progressive ophthalmoplegia. Methods A retrospective analysis on the clinical manifestation, laboratory test, diagnosis, treatment and prognosis of 4 patients with acute progressive ophthalmoplegia, admitted to our hospital from January 2014 to March 2014, was performed and the related literatures were reviewed. Results The 4 patients were characterized by complete ophthalmoplegia, facial palsy and spinal nerve palsy. Two had heterocyst in cytological checkup of eerebrospinal fluid with positive meningeal irritation sign, therefore, they were diagnosed as having meningeal earcinomatosis （MC）; they refused treatment, one died quickly and the other deteriorated gradually. The third patient was diagnosed as having Bickerstaff brainstem encephalitis （BBE） with conscious disturbance, bulboparalysis and accessory nerve palsy who was treated with double plasmapheresis and glucocorticoid therapy and recovered well. The fourth one was diagnosed as Miller-Fisher syndrome （MFS） and combined with Guillain-Barre syndrome （GBS）, treated with human immunoglobulin at dosage of 0.4 g/（kg.d） and recovered after three weeks. Conclusions The clinical manifestations of MC, BBE, MFS and GBS may show acute progressive ophthalmoplegia which may be confused and difficult to diagnose. Examination of cerebrospinal fluid is still a very useful method to help the diagnosis. Prognosis of MC is bad and the others are good. Plasmapheresis and human immunoglobulin are useful to BBE, MFS and GBS.

关 键 词：眼肌麻痹 诊断 鉴别诊断 预后 

分 类 号：R777.45[医药卫生—眼科]