【作者】 梁文昭；

【导师】 江新梅；

【作者基本信息】 吉林大学 ， 临床医学， 2009， 硕士

【摘要】 回顾分析61例我院诊断为运动神经元病患者病例资料,跟踪随访,深入了解其就诊及治疗情况,寻找提高其生存质量及延长生存时间的方法,进一步加深临床医生对运动神经元病患者的认识及关注。对61例就诊于我院诊断为运动神经元病患者的病例资料进行回顾性分析,分别统计其发病年龄,起病形式,确诊时间,影像学资料、治疗方法及其随访资料进行分析。61诊断为运动神经元病的患者,男:女为34:27,平均发病年龄47岁。临床表现多以肢体无力起病,部分患者首发症状为球麻痹。多数患者行肌肉活检及肌电图检查提示神经源性肌肉萎缩。随访患者19例,随访率30.6%。随访19例患者中5例死亡;4例已经卧床,生活不能自理;平均出现时间为在起病后28.3±6.3月;10例表示未见到明显进展。MND起病隐匿,首发症状多样,现阶段的诊断主要依靠临床表现并结合神经电生理及肌肉病理学依据,对该病的治疗尚没有特异性手段,患者治疗并不积极。病程情况因人而异,出现球麻痹后进展较快,生存质量严重受损,生存时间明显下降。临床医生应提高认识,更多关注。更多还原

【Abstract】 Objective: Retrospective analysis of 61 cases diagnosed in the first hospital of Jilin university in patients as motor neuron disease and follow-up to find out the situation of current diagnosis and treatment of MNDs, and look for the ways improved the quality of life and prolong the living time. To deepen the Clinician awareness of patients and concerns.Materials and Methods: The data of 61 cases from January 2000 to March 2009 in the First Hospital of Jilin University which all diagnosis as MNDs . Statistics of its prevalence were age, onset forms, diagnosis time, imaging, treatment and follow-up analysis of descriptive information.Results: In the 61 cases, 34 cases are male,27 cases are female.The average age of onset is 47.8±17.5 years old. The first symptom to diagnosis interval on an average of 27.5±35.5 months.Most patients began with the symptom of limbs weakness,and some of them began with the bulbar paralysis.35 cases of them already have bulbar paralysis.30 cases do the musle biopsy,all of them show the performance of neurogenic muscle atrophy that consistent with motor neuron disease of muscle pathology.53 patients do the EMG, and 48(91 %)cases show the positive neurogenic injury, 4(8%)have a uncertainty result, and one patient’s EMG shows diffent results in different hospitals. 19 cases of patients have imaging reports, 7(37%) cases shows cerebral ischemia, 11(58%)have bulging disc, 1(5%) is normal. Follow-up 19 (31.1%) patients after they leaving the hospital. 5(23.8%)cases took the treatment of Riluzole and 2 of them hold on the treatment untilnow. But the other three dropped in after a short time. 4(19.1%)cases took the intravenous injection of Edaravone, which the specific cause is unkown. 8 (38.1%) were insisting in take the medicine treatment of vitamin E. 3 (14.3%) cases have received the treatment of stem cell transplantation. 14(66.7%) cases are under the Chinese medicine treatment. 14 (66.7%) patients have the Difficulty of swallowing,only one of them take the gastrostomy tube feeding. Breathing difficulties are known to occur in 7 (33.3%) cases. Only 1 (14.3%) cases of them take the TIPPV, maintaining 3 months and died because of pulmonary infection and electrolyte disturbance. In the other 6 cases ,3 of them are already died of respiratory failure, the average survival time was 12.3±7.9 months. 2 (9.5%) patients do the training under the professional guidance of doctors. They claimed that musles are more powerful,and the quality of life improved.5 cases died in the 19 patients follow-up of which 4 are died of the respiratory failure. 4 of the 19 cases follow-up are already in bed with out the ability of self-care for an average time of 28.3±6.3 after the onset appears. 10 cases did not see the significant progress that need the long term follow-up.Conclusion: Motor neron disease is difficulty in diagnosis because of its occult onset. And the diagnosis of it often based on the symptomatic features of the patients and need the supports of the EMG and musle biopsy reports. There is no specific treatment method,the ways to treat always be the symptomatic and supportive treatment. The progression of MND depends on the individual. It goes fast when the bulbar paralysis happened. The survival time and the quality of life declined rapidly. We should pay more attention to the patients with MNDs and do better follow-up and management.更多还原