165例运动神经元病临床分析

发布时间：2018-06-19 12:56

本文选题：运动神经元病 + 临床特征　；参考：《山西医科大学》2014年硕士论文

【摘要】：目的：通过对运动神经元病（motor neuron disease, MND）患者的一般资料、临床特征、辅助检查、病情演变等资料进行分析，了解MND的发病特点，并对误诊原因及相关因素进行探讨，为该病早期诊治及预防提供临床依据。方法：回顾性分析2006年1月至2013年6月就诊于山西医科大学第一医院、山西医科大学第二医院、山西省人民医院MND患者的住院病例资料，包括：性别、年龄、居住地、既往史、误诊情况、伴随疾病、个人史、家族史、首发症状、发病年龄、病程、发病至确诊时间、神经系统查体、相关辅助检查、化验结果、职业等；进一步随访确定临床分型；整理资料，统计分析。结果：入组患者165例，，患者发病年龄为20-80岁，平均发病年龄为52.2±10.4岁，男女发病高峰均为50-59岁，男女患者患病比例为1.84:1。肌萎缩侧索硬化122例(73.9%)，其中确诊患者有101例，临床或电生理拟诊患者21例；进行性脊肌萎缩26例(15.8%)；进行性延髓麻痹15例(9.1%)；原发性侧索硬化2例(1.2%)。122例肌萎缩侧索硬化患者中，以上肢肌无力或肌萎缩起病者有55例（45.1%），其中以远端起病者有37例，以下肢肌无力或肌萎缩起病者有34例（27.9%），其中以远端起病者有21例；初步推算本地区MND患者被误诊构成比约为42.4%，被误诊为脊椎病变的病例占48.6%，行手术治疗患者14例；被误诊为脑血管病的病例占35.7%，均伴有明显的脑血管病危险因素；77.1%的误诊事件发生在基层医院；165例MND患者105例行CSF检测，其中32例患者CSF蛋白轻度升高，1例CSF寡克隆带检测阳性；发病的患者中，重体力工作者76例（46.1%），长期接触化学毒物、重金属者48例（29.1%），吸烟者51例（39.1%）。结论：本研究中男女患者患病比例为1.84:1；发病年龄集中在50-59岁；肌萎缩侧索硬化是MND最常见的类型，主要以单侧上肢远端肌无力或肌萎缩起病；MND误诊构成比较高，被误诊为脊椎病变患者居首位，部分患者因误诊行手术治疗，术后疗效不佳，给患者及其家属带来了巨大的经济及心理负担；被误诊为脑血管病患者，均伴随有明确的脑血管病危险因素；误诊事件多发生在基层医院，提示基层医院MND误诊率较高，因此，应加强基层医师关于MND知识的宣教；本课题提示职业暴露、手术外伤、毒物药物接触史、吸烟等在MND发病中所占比例较高，提示MND发病可能与这些因素相关；影像学及电生理检查对MND的诊断及鉴别诊断有重大意义，临床上应积极完善相关辅助检查。
[Abstract]:Objective: to analyze the general data, clinical features, auxiliary examination and disease evolution of motor neuron disease (MND), and to explore the causes of misdiagnosis and related factors. To provide clinical basis for early diagnosis, treatment and prevention of the disease. Methods: the data of MND patients in the first Hospital of Shanxi Medical University, the second Hospital of Shanxi Medical University and the people's Hospital of Shanxi Province from January 2006 to June 2013 were analyzed retrospectively, including gender, age, residence, past history. Misdiagnosis, associated disease, personal history, family history, initial symptoms, age of onset, course of disease, time from onset to diagnosis, neurological examination, related auxiliary examination, laboratory results, occupation, etc. Collate data, statistical analysis. Results: 165 patients were enrolled, the age of onset was 20-80 years old, the average age of onset was 52.2 卤10.4 years old, the peak of male and female patients was 50-59 years old, the ratio of male and female patients was 1.84: 1. Of the 122 cases of amyotrophic lateral sclerosis, 101 were diagnosed, 21 were clinically or electrophysiologically diagnosed; 26 were progressive spinal atrophy; 15 were progressive medullary palsy; 15 were progressive medullary palsy; and 2 were primary lateral sclerosis. 122 were amyotrophic lateral sclerosis. There were 55 cases with upper limb myasthenia or muscle atrophy, including 37 cases with distal onset, 34 cases with lower extremity myasthenia or muscle atrophy, and 21 cases with distal onset. The misdiagnosis ratio of MND patients in this area was estimated to be about 42.4%, the proportion of patients misdiagnosed as spinal diseases was 48.6 cases, 14 cases were treated by operation, and 35.7cases were misdiagnosed as cerebrovascular diseases, all of them were accompanied by obvious risk factors of cerebrovascular diseases. 77.1% of misdiagnosis occurred in 165 MND patients in primary hospital, 105 cases were detected by CSF, among them 32 cases were slightly elevated in CSF protein and 1 case was positive for CSF oligoclonal band detection, 76 cases of heavy physical workers were exposed to chemical poison for a long time. The heavy metals were found in 48 cases and the smokers in 51 cases. Conclusion: in this study, the prevalence ratio of male and female patients was 1.84: 1, the onset age was 50-59 years old, amyotrophic lateral sclerosis was the most common type of MND, and the misdiagnosis of MND was mainly caused by unilateral upper limb myasthenia or muscular atrophy. The patients who were misdiagnosed as spinal diseases were the first, some of whom were misdiagnosed by surgical treatment, but the effect was not good, which brought great economic and psychological burden to the patients and their families, and was misdiagnosed as cerebrovascular disease patients, and some of the patients were misdiagnosed as cerebrovascular disease patients. Misdiagnosis events occurred in primary hospitals, suggesting that the misdiagnosis rate of MND in primary hospitals was high, so we should strengthen the propaganda and education of primary physicians on MND knowledge. The history of exposure to toxic drugs and smoking accounted for a high proportion of MND, suggesting that MND may be related to these factors, imaging and electrophysiological examination are of great significance in the diagnosis and differential diagnosis of MND. The related auxiliary examination should be improved in clinic.
【学位授予单位】：山西医科大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R744.8

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