老年发病的运动神经元病临床特点及诊治:15例病例系列分析  

Clinical characteristics,diagnosis and treatment of motor neuron disease in the elderly:A case series of 15 patients

作　　者：刘艳[1] 张法军 刘丹[2] 柏秀娟[1] 平荣宇 张善春[1] 邢建丽[3] 蔡晓平[2] 吴卫平[1] LIU Yan;ZHANG Fajun;LIU Dan;BAI Xiujuan;PING Rongyu;ZHANG Shanchun;XING Jianli;CAI Xiaoping;WU Weiping(National Clinical Research Center for Geriatric Diseases,Department of Neurology,the Second Medical Center,Chinese PLA General Hospital,Beijing 100853,China;The Seventh Department of Health Care,the Second Medical Center,Chinese PLA General Hospital,Beijing 100853,China;Department of Geriatrics,the Seventh Medical Center,Chinese PLA General Hospital,Beijing 100700,China)

机构地区：[1]解放军总医院第二医学中心神经内科,国家老年疾病临床研究中心,北京100853 [2]解放军总医院第二医学中心保健七科,北京100853 [3]解放军总医院第七医学中心老年医学科,北京100700

出　　处：《解放军医学院学报》2024年第7期758-761,共4页Academic Journal of Chinese PLA Medical School

摘　　要：背景运动神经元病(motor neuron disease,MND)是一种神经系统变性疾病,预后不佳,多为中年起病,目前国内缺乏对老年起病的MND的研究。目的探讨60岁以上发病的MND的临床特点及预后。方法收集2008年9—2023年9月在解放军总医院第七医学中心住院的老年起病的MND患者的临床资料,分析其临床特征、诊治及结局。结果共收集15例老年起病的MND患者,男性10例,女5例,平均年龄(66.2±5.71)岁,发病年龄为60~79岁。延髓起病4例,单侧上肢起病6例,单侧下肢起病3例,双下肢起病1例,四肢起病1例。7例患者在起病18个月内出现了延髓症状,呼吸肌受累3例。临床分型方面,肌萎缩侧索硬化9例,进行性脊肌萎缩症4例,进行性延髓麻痹2例。6例在发病初期被误诊,误诊率达40%。5例放弃治疗,其余治疗效果也不佳。结论老年起病的MND男性多于女性,多为单侧起病,延髓起病的比例高,以肢体起病的患者更易、也更早出现延髓症状,呼吸肌受累比例较高。提示老年起病的MND可能生存期更短,共患病多,误诊率高,弃疗率高。Background Motor neuron disease(MND)is a neurodegenerative disease with poor prognosis.It typically occurs in middle-aged individuals,and there is a lack of research on MND in the elderly in China.Objective To explore the clinical characteristics and prognosis of MND in individuals over 60 years old.Methods Clinical data about elderly patients with MND admitted to the Seventh Medical Center of Chinese PLA General Hospital from September 2008 to September 2023 were collected and analyzed for their clinical features,diagnosis,and outcomes.Results A total of 15 elderly patients with MND were included,with 10 males and 5 females.The average age was 66.2±5.71 years,and the onset age ranged from 60 to 79 years.Four cases presented with bulbar onset,6 cases with unilateral upper limb onset,3 cases with unilateral lower limb onset,1 case with bilateral lower limb onset,and 1 case with onset in all four limbs.Seven patients developed bulbar symptoms within 18 months onset,and 3 cases had respiratory muscle involvement.In terms of clinical classification,9 cases had amyotrophic lateral sclerosis,4 cases had progressive spinal muscular atrophy,and 2 cases had progressive bulbar palsy.Six patients were initially misdiagnosed,resulting in a misdiagnosis rate of 40%.Five patients gave up treatment,and the treatment outcomes for the remaining patients were poor.Conclusion MND in older adults is common in males and often presents with unilateral limb onset.Bulbar onset is more frequent,and patients with limb onset are more likely to develop bulbar symptoms earlier.There is a higher proportion of respiratory muscle involvement.The findings suggest that MND with onset in older age may have a shorter survival period,a higher co-morbidity rate,a higher misdiagnosis rate,and a higher treatment abandonment rate.

关 键 词：运动神经元病 老年 临床特征 肌萎缩侧索硬化 进行性延髓麻痹 

分 类 号：R744.8[医药卫生—神经病学与精神病学]