多系统萎缩C型12例临床分析
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摘要
目的分析以小脑症状为主的多系统萎缩C型(MSA-C)患者的临床诊治特点。方法选取2010年1月—2018年1月首都医科大学石景山教学医院/北京市石景山医院神经内科诊治的MSA-C患者12例作为研究对象,回顾分析MSA-C型患者的临床特点、辅助检查结果及诊治经过。结果 12例患者中,男9例,女3例,发病年龄52～68(56. 7±2. 1)岁,均为隐匿起病,明确诊断时病程1. 5～7(3. 9±1. 2)年;12例患者均以小脑症状为突出表现,小脑性共济失调12例,小脑性言语改变8例,眼震4例;12例患者均伴有不同程度的自主神经功能障碍,排尿障碍9例(男性8例),性功能障碍8例,体位性低血压9例,多汗3例;4例患者伴有肌张力增高等锥体外系症状;以共济失调为首发症状5例,以自主神经功能障碍为首发症状7例;磁共振检查显示典型脑桥"十字征"6例,小脑萎缩8例。病程早期误诊为脑血管病7例。予对症处理后,预后不佳,死亡3例,余9例症状加重。结论病程初期,MSA-C常因临床少见、早期症状不典型、表现多样、未详细追溯病史及查体而被误诊,注意隐匿起病、慢性进展、以小脑症状为主的多系统受累及特征性影像学表现是临床早期准确诊断的关键。
Objective To analyze the clinical diagnosis and treatment characteristics of multiple system atrophy type C(MSA-C) patients with cerebellar symptoms. Methods From January 2010 to January 2018,12 patients with MSA-C diagnosed and treated in the Department of Neurology,Shijingshan Teaching Hospital of Capital Medical University and Beijing Shijingshan Hospital were selected as the subjects. The clinical characteristics,auxiliary examination results and diagnosis and treatment of MSA-C were retrospectively analyzed. Results Among the 12 patients,9 were males and 3 were females,the onset age was 52-68(56. 7 ± 2. 1) years old,all patients were concealed onset,the course of diagnosis was 1. 5 ～ 7(3. 9 ±1. 2) years; 12 patients were characterized by cerebellar symptoms,12 cases of cerebellar ataxia,8 cases of cerebellar speech changes,4 cases of nystagmus; 12 patients were accompanied by varying degrees of autonomic nervous dysfunction,9 cases of dysuria(8 males). There were 8 cases of sexual dysfunction,9 cases of orthostatic hypotension and 3 cases of hyperhidrosis,4 cases with extrapyramidal symptoms of hypertonia,5 cases with ataxia and 7 cases with autonomic nervous dysfunction,6 cases with typical pontine " cross sign" and 8 cases with cerebellar atrophy. 7 cases were misdiagnosed as cerebrovascular disease.After symptomatic treatment,the prognosis was poor,3 cases died,and the remaining 9 cases were aggravated. Conclusion In the early stage of the disease,MSA-C is often misdiagnosed because of its rare clinical manifestations,atypical early symptoms,various manifestations,lack of detailed retrospective history and physical examination.
Objective To analyze the clinical diagnosis and treatment characteristics of multiple system atrophy type C(MSA-C) patients with cerebellar symptoms. Methods From January 2010 to January 2018,12 patients with MSA-C diagnosed and treated in the Department of Neurology,Shijingshan Teaching Hospital of Capital Medical University and Beijing Shijingshan Hospital were selected as the subjects. The clinical characteristics,auxiliary examination results and diagnosis and treatment of MSA-C were retrospectively analyzed. Results Among the 12 patients,9 were males and 3 were females,the onset age was 52-68(56. 7 ± 2. 1) years old,all patients were concealed onset,the course of diagnosis was 1. 5 ～ 7(3. 9 ±1. 2) years; 12 patients were characterized by cerebellar symptoms,12 cases of cerebellar ataxia,8 cases of cerebellar speech changes,4 cases of nystagmus; 12 patients were accompanied by varying degrees of autonomic nervous dysfunction,9 cases of dysuria(8 males). There were 8 cases of sexual dysfunction,9 cases of orthostatic hypotension and 3 cases of hyperhidrosis,4 cases with extrapyramidal symptoms of hypertonia,5 cases with ataxia and 7 cases with autonomic nervous dysfunction,6 cases with typical pontine " cross sign" and 8 cases with cerebellar atrophy. 7 cases were misdiagnosed as cerebrovascular disease.After symptomatic treatment,the prognosis was poor,3 cases died,and the remaining 9 cases were aggravated. Conclusion In the early stage of the disease,MSA-C is often misdiagnosed because of its rare clinical manifestations,atypical early symptoms,various manifestations,lack of detailed retrospective history and physical examination.
引文
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[9]张和振,王贺波.多系统萎缩临床诊断及磁共振特征研究进展[J].河北医药,2013,32(20):3155-3157. DOI:10. 3969/j. issn.1002-7386. 2013. 20. 065.
[10]李金遥,吴慧杰,王灿,等.多系统萎缩与脊髓小脑共济失调的鉴别诊断[J].中风与神经疾病杂志,2017,34(5):472-473.
[11] Kuzdas-Wood D,Stefanova N,Jellinger KA,et al. Towards translational therapies for multiple system atrophy[J]. Prog Neurobiol,2014,118(100):19-35. DOI:10. 1016/j. pneurobio. 2014. 02. 007.
[12] Fanciulli A,Wenning GK. Multiple-system atrophy[J]. N Engl J Med,2015,372(3):249-263. DOI:10. 1056/NEJMral311488.
[13]楚勤英,傅小玲,迟铖,等.丁苯酞对多系统萎缩患者治疗效果分析[J].中华老年多器官疾病杂志,2016,15(12):907-911. DOI:10. 11915/j. issn. 1671-5403. 2016. 12. 217.
[14]邱峰,刘建国,张海玲,等.改良“鸡尾酒”疗法对多系统萎缩的疗效观察[J].中华老年心脑血管病杂志,2014,16(9):958-960.DOI:10. 3969/j. issn. 1009-0126. 2014. 09. 018.
[15]王万宏,李琳琳,毕鸿雁,等.多系统萎缩中医康复治疗的研究进展[J].医学综述,2018,24(11):2236-2240. DOI:10. 3969/j.issn. 1006-2084. 2018. 11. 031.
[2]王会喜,许予明,高敬平.多系统萎缩35例临床表现与误诊分析[J].疑难病杂志,2011,10(11):862-863. DOI:10. 3969/j. issn.1671-6450. 2011. 11. 027.
[3]孙占芳,江泓,唐北沙.多系统萎缩研究新进展[J].中国神经精神疾病杂志,2010,36(10):635-637. DOI:10. 3969/j. issn. 1002-0152. 2010. 10. 018.
[4]赵振华,陈枝挺,刘昌云.老年起病的多系统萎缩-C型患者的临床特征及其影像学特点[J].中国老年学杂志,2012,32(17):3663-3665. DOI:10. 3969/j. issn. 1005-9202. 2012. 17. 020.
[5]刘姜冰,刘一辉,张新江.多系统萎缩28例临床特征分析[J].疑难病杂志,2013,12(12):953-954. DOI:10. 3969/j. issn. 1671-6450. 2013. 12. 018.
[6]顾卫红.多系统萎缩的诊断与治疗[J].中国现代神经疾病杂志,2012,12(3):257-260. DOI:10. 3969/j. issn. 1672-6731. 2012. 03. 006.
[7]杨虹,曾昆,魏桂荣,等.自主神经功能障碍型多系统萎缩临床诊断及治疗[J].中国循环杂志,2011,26(5):367-369. DOI:10.3969/j. issn. 1000-3614. 2011. 05. 014.
[8]林志坚,陈旭辉,车秀娟,等.磁共振成像在多系统萎缩诊断与鉴别诊断中的应用价值[J].神经损伤与功能重建,2014,9(1):40-43. DOI:10. 3870/sjsscj. 2014. 01. 010.
[9]张和振,王贺波.多系统萎缩临床诊断及磁共振特征研究进展[J].河北医药,2013,32(20):3155-3157. DOI:10. 3969/j. issn.1002-7386. 2013. 20. 065.
[10]李金遥,吴慧杰,王灿,等.多系统萎缩与脊髓小脑共济失调的鉴别诊断[J].中风与神经疾病杂志,2017,34(5):472-473.
[11] Kuzdas-Wood D,Stefanova N,Jellinger KA,et al. Towards translational therapies for multiple system atrophy[J]. Prog Neurobiol,2014,118(100):19-35. DOI:10. 1016/j. pneurobio. 2014. 02. 007.
[12] Fanciulli A,Wenning GK. Multiple-system atrophy[J]. N Engl J Med,2015,372(3):249-263. DOI:10. 1056/NEJMral311488.
[13]楚勤英,傅小玲,迟铖,等.丁苯酞对多系统萎缩患者治疗效果分析[J].中华老年多器官疾病杂志,2016,15(12):907-911. DOI:10. 11915/j. issn. 1671-5403. 2016. 12. 217.
[14]邱峰,刘建国,张海玲,等.改良“鸡尾酒”疗法对多系统萎缩的疗效观察[J].中华老年心脑血管病杂志,2014,16(9):958-960.DOI:10. 3969/j. issn. 1009-0126. 2014. 09. 018.
[15]王万宏,李琳琳,毕鸿雁,等.多系统萎缩中医康复治疗的研究进展[J].医学综述,2018,24(11):2236-2240. DOI:10. 3969/j.issn. 1006-2084. 2018. 11. 031.