CAJ | 학술논문

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,l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;l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.
목적 탐토이급성진행성안기마비위돌출표현적환자림상진단、감별진단급기예후.방법 대선취자남경의과대학제일부속의원신경내과2014년1지3월수치적이급성진행성안기마비위돌출림상증상적4례환자적림상표현、보조검사결과、진치경과여예후작회고성분석,병복습상관문헌.결과 4례환자이전안기마비위핵심표현,합병면신경마비、척신경마비등.기중2례위뇌막암병(MC),뇌막자격정양성,뇌척액세포학검사가견이형세포,균방기치료,l례흔쾌사망,1례진행성악화;1례위필식뇌간뇌염(BBE),합병의식장애급연수、부신경마비,경쌍중혈장치환급격소치료후명현개선,목전증상기본소실;l례위Miller-Fisher종합정합병격림파리종합정,공제실조명현차합병쌍측면탄,정맥병구단백충격치료5d,3주후증상기본소실,목전증상급체정완전소실.결론 MC、BBE、Miller Fisher종합정(MFS)、격림파리종합정(GBS)균가이급성안기마비위돌출증상,가합병기타로신경、척신경마비증상.뇌척액검사재명학진단중발휘착중요작용.MC예후겁차,기여2충질병경혈장치환、정맥병구단백충격등치료후예후량호.
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 cerebrospinal fluid with positive meningeal irritation sign,therefore,they were diagnosed as having meningeal carcinomatosis (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 ofMC,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.