视神经脊髓炎谱系疾病临床特点和早期误诊原因分析

发布时间：2018-01-02 04:21

本文关键词：视神经脊髓炎谱系疾病临床特点和早期误诊原因分析　出处：《广西医科大学》2016年硕士论文　论文类型：学位论文

【摘要】：目的探讨我院视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder, NMOSD)患者的临床特点及早期误诊原因,提高对该疾病的认识。方法应用2015年NMOSD诊断标准对广西医科大学第一附属医院2012年7月至2015年12月收治的视神经脊髓炎(neuromyelitis optica, NMO)、多发性硬化(multiple sclerosis,MS)患者进行重新诊断,最终100例患者临床诊断为NMOSD,其中原诊NMO 72例,原诊MS 28例。对其临床及影像学资料进行回顾性分析,并分析原诊NMO组首次误诊和原诊MS更新为NMOSD的原因。随访比较更新诊断为NMOSD组和原诊NMO组首次发病1年内的平均复发次数有无差异。结果1.本研究中,女性89例,男性11例,女：男约为8.09：1；首次发病年龄在12-76岁之间,平均发病年龄(38.23±13.83)岁,发病高峰为20-49岁。2.33%患者发病前有明确的诱因,其中以上呼吸道感染最常见,约占84.9%。13%患者合并自身免疫性疾病(以干燥综合症多见)。31%患者自身抗体阳性,其中ANA阳性多见,占19%。3.首发症状：视力下降最多见,占65%,脊髓症状占46%,脑部症状占24%。4.复发症状：100例患者中,脊髓症状最常见,占64.5%。其次为视神经症状(占30.6%)及脑部症状(占9.8%)。5.头颅MRI异常率达73%。其中,皮质下/深部白质区占53.8%,延髓占30.8%,桥脑占16.9%,侧脑室旁占15.4%,中脑占13.8%,第三脑室旁占7.7%,小脑占6.2%,大脑半球占4.6%。脊髓MRI病灶部位以颈、胸髓多见,累及3个或3个以上椎体节段,其中累及5个以上椎体的患者占73.7%。矢状位上,病灶呈弥漫性损害者49例(51.5%),弥漫性和线样损害者22例(23.2%),线样损害者24例(25.3%)。轴位上病灶累及脊髓灰质为主,也可同时累及白质,形态多样,呈圆点、蛇眼样、蝴蝶症(H型),也可有偏心样损害。6.NMO早期症状不典型,易出现误诊。本研究中4例患者出现误诊情况。其中2例误诊为急性胃肠炎,1例误诊为体位性低血压,1例误诊为胆汁反流症。7.更新诊断为NMOSD组和原诊NMO组比较,首次发病1年内的平均复发次数存在差异,有统计学意义(P0.05)。结论1. NMOSD好发于中青年女性,临床症状呈多样性。2.NMOSD可出现脑部病灶,以皮质下/深部白质区最多见,易累及第三脑室、中脑导水管、第四脑室的室管膜周部位。脊髓长节段病灶、线样征对NMOSD有一定诊断价值。3.NMOSD早期误诊为MS的主要原因：对NMOSD脑部病灶特点认识不够充分。
[Abstract]:Objective to investigate the optica spectrum disorder of neuromyelitis in our hospital. The clinical features and causes of early misdiagnosis in patients with NMOSD. Methods the diagnosis criteria of 2015 NMOSD were used to treat optic neuromyelitis in the first affiliated Hospital of Guangxi Medical University from July 2012 to December 2015. Neuromyelitis optica. NMOSD was rediagnosed in 100 patients with multiple sclerosis. Among them 72 cases were diagnosed as NMO and 28 cases as MS. The clinical and imaging data were analyzed retrospectively. The causes of the first misdiagnosis and the renewal of MS to NMOSD in the primary NMO group were analyzed. The average recurrence times in the NMOSD group and the original NMO group within one year after the first onset were compared with those in the original NMO group. Results 1. In this study. 89 female, 11 male, female: about 8.09: 1; The mean age of onset was 38.23 卤13.83 years old. The peak of onset was 20-49 years old. 2.33% patients had definite inducement before onset. Among them, upper respiratory tract infection was the most common, accounting for 84.9. 13% of the patients with autoimmune diseases (Sjogren's syndrome is more common in patients with autoantibody positive. 31% of the patients with positive autoantibodies, in which ANA is more positive. The first symptom was 19.3.The first symptom: vision decreased most, 65%, spinal cord accounted for 46%, brain symptom accounted for 24.4.Of 100 patients with recurrent symptoms, spinal cord symptom was the most common. The second was optic nerve symptom (30.6%) and brain symptom (9.8%). The abnormal rate of cranial MRI was 73.The subcortical / deep white matter area accounted for 53.8%. The medulla oblongata accounted for 30.8%, pontine for 16.9g, paraventricular for 15.4, midbrain for 13.8am, paraventricular for 7.7and cerebellum 6.2%. The focal site of spinal cord MRI was cervical and thoracic spinal cord, involving 3 or more vertebrae segments, of which more than 5 vertebrae were involved in 73.7. sagittal position. Diffuse lesions were found in 49 cases (51.5%), diffuse and linear lesions in 22 cases (23. 2%) and linear lesions in 24 cases (25. 3%). White matter may also be involved in the same time, with a variety of morphology, a round point, snake eye, butterfly syndrome H type, or eccentricity damage. 6. NMO early symptoms are not typical. In this study, 4 cases were misdiagnosed, 2 cases were misdiagnosed as acute gastroenteritis, 1 case was misdiagnosed as postural hypotension. One case was misdiagnosed as bile regurgitation. There was a difference in the average number of recurrence within one year between the newly diagnosed NMOSD group and the original NMO group. Conclusion 1. NMOSD is more common in young and middle-aged women, and its clinical symptoms are diverse. 2. NMOSD may present brain lesions. 2. The subcortical / deep white matter area was the most common, involving the third ventricle, aqueduct aqueduct, periependymal part of 4th ventricle and long segment of spinal cord. Linear signs have certain diagnostic value for NMOSD. 3. The main causes of early misdiagnosis of NMOSD as MS: insufficient understanding of the characteristics of brain lesions in NMOSD.
【学位授予单位】：广西医科大学
【学位级别】：硕士
【学位授予年份】：2016
【分类号】：R744.52

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