本文關鍵詞：視神經脊髓炎譜系疾病臨床特點和早期誤診原因分析　出處：《廣西醫(yī)科大學》2016年碩士論文　論文類型：學位論文

  更多相關文章： 視神經脊髓炎譜系疾病 多發(fā)性硬化 磁共振成像 診斷標準 誤診

【摘要】：目的探討我院視神經脊髓炎譜系疾病(neuromyelitis optica spectrum disorder, NMOSD)患者的臨床特點及早期誤診原因,提高對該疾病的認識。方法應用2015年NMOSD診斷標準對廣西醫(yī)科大學第一附屬醫(yī)院2012年7月至2015年12月收治的視神經脊髓炎(neuromyelitis optica, NMO)、多發(fā)性硬化(multiple sclerosis,MS)患者進行重新診斷,最終100例患者臨床診斷為NMOSD,其中原診NMO 72例,原診MS 28例。對其臨床及影像學資料進行回顧性分析,并分析原診NMO組首次誤診和原診MS更新為NMOSD的原因。隨訪比較更新診斷為NMOSD組和原診NMO組首次發(fā)病1年內的平均復發(fā)次數有無差異。結果1.本研究中,女性89例,男性11例,女：男約為8.09：1；首次發(fā)病年齡在12-76歲之間,平均發(fā)病年齡(38.23±13.83)歲,發(fā)病高峰為20-49歲。2.33%患者發(fā)病前有明確的誘因,其中以上呼吸道感染最常見,約占84.9%。13%患者合并自身免疫性疾病(以干燥綜合癥多見)。31%患者自身抗體陽性,其中ANA陽性多見,占19%。3.首發(fā)癥狀：視力下降最多見,占65%,脊髓癥狀占46%,腦部癥狀占24%。4.復發(fā)癥狀：100例患者中,脊髓癥狀最常見,占64.5%。其次為視神經癥狀(占30.6%)及腦部癥狀(占9.8%)。5.頭顱MRI異常率達73%。其中,皮質下/深部白質區(qū)占53.8%,延髓占30.8%,橋腦占16.9%,側腦室旁占15.4%,中腦占13.8%,第三腦室旁占7.7%,小腦占6.2%,大腦半球占4.6%。脊髓MRI病灶部位以頸、胸髓多見,累及3個或3個以上椎體節(jié)段,其中累及5個以上椎體的患者占73.7%。矢狀位上,病灶呈彌漫性損害者49例(51.5%),彌漫性和線樣損害者22例(23.2%),線樣損害者24例(25.3%)。軸位上病灶累及脊髓灰質為主,也可同時累及白質,形態(tài)多樣,呈圓點、蛇眼樣、蝴蝶癥(H型),也可有偏心樣損害。6.NMO早期癥狀不典型,易出現誤診。本研究中4例患者出現誤診情況。其中2例誤診為急性胃腸炎,1例誤診為體位性低血壓,1例誤診為膽汁反流癥。7.更新診斷為NMOSD組和原診NMO組比較,首次發(fā)病1年內的平均復發(fā)次數存在差異,有統(tǒng)計學意義(P0.05)。結論1. NMOSD好發(fā)于中青年女性,臨床癥狀呈多樣性。2.NMOSD可出現腦部病灶,以皮質下/深部白質區(qū)最多見,易累及第三腦室、中腦導水管、第四腦室的室管膜周部位。脊髓長節(jié)段病灶、線樣征對NMOSD有一定診斷價值。3.NMOSD早期誤診為MS的主要原因：對NMOSD腦部病灶特點認識不夠充分。
[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 鹵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.
【學位授予單位】：廣西醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2016
【分類號】：R744.52

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