眼外肌瘫痪被误诊为重症肌无力的肌肉疾病xx例临床-第三军医大学学报.docVIP

眼外肌瘫痪被误诊为重症肌无力的肌肉疾病11例临床及病理分析 摘要 目的：分析因眼外肌瘫痪被误诊为重症肌无力的肌病患者临床和肌肉病理特点，为眼外肌麻痹的鉴别诊断开阔思路。 方法：总结2010年10月至2014年5月就诊于重庆医科大学附属第一医院神经内科的11例肌病患者，临床均表现为眼睑下垂及复视，分析其临床、病理特点。 结果：11例患者中男 6例，女5例，年龄16～66 岁，患者均存在眼外肌麻痹表现，其中眼咽型肌营养不良3例，眼咽型远端型肌病2例，慢性进行性眼外肌麻痹（Chronic progressive external ophthalmoplegia，CPEO）6例。曾在外院诊断重症肌无力，给予溴吡斯的明或/和强的松治疗无效。肌肉活检可见特征性病理改变。统计学分析发现眼咽型肌营养不良患者发病年龄较大（p0.05）,肌酸激酶在眼咽型远端型肌病患者中较高，但差异无显著性（p0.05）。 结论：对于眼外肌麻痹貌似重症肌无力的患者，我们需要鉴别某些特殊类型肌病，肌肉活检病理学检查可以提供重要帮助。 关键词：眼外肌瘫痪；慢性进行性眼外肌麻痹；眼咽型远端型肌病；眼咽型肌营养不良 Abstract Objective: To analyse the clinical and pathological characteristics of patients with myopathies that were misdiagnosed as myasthenia gravis because of external ophthalmoplegia, widen the thoughts for differential diagnosis of extraocular muscle paralysis. Methods: The clinical and pathological features of 11 myopathy cases with ptosis and diplopia admitted to the neurology department of the First Affiliated Hospital of Chongqing Medical University berween October 2010 and May 2014 were retrospectively reviewed and analyzed. Results: Among the 11 patients, 6 male and 5 female, aged 16-66 years old. All had paralysis of extraocular muscle manifestations, including oculopharyngeal muscular dystrophy (OPMD) in 3 cases, oculopharyngeal distal myopathy in 2 cases, Chronic progressive external ophthalmoplegia (CPEO) in 6 cases. Muscle biopsy showed the characteristic pathological changes. Statistical analysis showed that the age of onset in OPMD patients is older (p0.05), creatine kinase level in oculopharyngeal distal myopathy patients is higher, but the difference was not significant (p0.05). Conclusion: Certain rare myopathy should be considered for patients with external ophthalmoplegia seemingly myasthenia gravis. Muscle biopsy can provide clue for differential diagnosis. Key words: external ophthalmoplegia; oculopharyngeal muscular dystrophy(OPMD); oculopharyngeal distal myopathy; chronic progressive external ophthalmoplegia (CPEO) 一、前言 眼外肌瘫痪所致的眼睑下垂和复视是重症肌无力的常见症状。重症肌无力患者