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

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（CPEO）6例。曾在外院诊断重症肌无力，给予溴吡斯的明和（或）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（P＜0．05），肌酸激酶在眼咽型远端型肌病患者中较高，但差异无统计学意义（P＞0．05）。结论对于眼外肌麻痹貌似重症肌无力的患者，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。

Clinical and pathological analysis of 11 cases of extraocular muscle paralysis was misdiagnosed as muscular disease myasthenia gravis

Objective To analyse the clinical and pathological characteristics of patients with myopathies that were misdiag-nosed 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 between October 2010 and May 2014 were retrospec-tively reviewed and analyzed .Results Among the 11 patients ,6 male and 5 female ,aged 16-66 years old .All had paralysis of ex-traocular muscle manifestations ,including oculopharyngeal muscular dystrophy(OPMD) in 3 cases ,oculopharyngeal distal myopa-thy in 2 cases ,chronic progressive external ophthalmoplegia(CPEO) in 6 cases .Muscle biopsy showed the characteristic pathologi-cal changes .Statistical analysis showed that the age of onset in OPMD patients was older(P<0 .05) ,creatine kinase level in oculo-pharyngeal distal myopathy patients was higher ,but the difference was not significant (P>0 .05) .Conclusion Certain rare myopa-thy should be considered for patients with external ophthalmoplegia seemingly myasthenia gravis .Muscle biopsy can provide clue for differential diagnosis .