• 四川大學(xué)華西醫(yī)院神經(jīng)內(nèi)科（成都，610041）;

【摘要】　目的　 研究不同亞型多系統(tǒng)萎縮（multiple system atrophy，MSA）患者的臨床特點(diǎn)。　方法　 回顧分析2009年1月—2011年1月收治的105例“很可能的”MSA患者的臨床資料，包括發(fā)病年齡、首發(fā)癥狀、臨床表現(xiàn)、治療反應(yīng)性等。　結(jié)果　 105例MSA患者中，男57例，女48例，發(fā)病年齡58歲。以小腦性共濟(jì)失調(diào)為主要特點(diǎn)的MSA（MSA with predominant cerebellar ataxia，MSA-C）患者76例，以帕金森綜合征為主要特點(diǎn)的多系統(tǒng)萎縮（MSA with predominant parkinsonism，MSA-P）患者29例。39例患者僅以小腦功能障礙為首發(fā)癥狀；29例患者僅以帕金森綜合征為首發(fā)癥狀，23例患者僅以自主神經(jīng)功能障礙為首發(fā)癥狀，其余14例患者的首發(fā)表現(xiàn)至少包括2種癥狀組合。至最后一次隨訪時(shí)，54例患者同時(shí)存在小腦功能障礙、帕金森綜合征、自主神經(jīng)功能障礙和錐體束征，51例患者表現(xiàn)為自主神經(jīng)功能障礙與小腦功能障礙和（或）帕金森綜合征的不同形式的組合。　結(jié)論　 MSA患者以MSA-C為主。由于在病程早期，MSA與其他帕金森綜合征或小腦性共濟(jì)失調(diào)疾病的鑒別較為困難，因此，仔細(xì)動(dòng)態(tài)觀察患者臨床特點(diǎn)的演變情況，對(duì)MSA的診斷至關(guān)重要。
【Abstract】　Objective　 To investigate subtypes and clinical features of multiple system atrophy (MSA). 　Methods　 The clinical data of 105 probable MSA patients treated in our hospital from January 2009 to January 2011 were analyzed, including the age at onset, initial symptoms, clinical manifestations and responsivity to levodopa. 　Results　 The 105 probable MSA patients consisted of 57 males and 48 females, including 76 patients (72.4%) of MSA with predominant cerebellar ataxia (MSA-C) and 29 patients (27.6%) of MSA with predominant parkinsonism (MSA-P). The mean age at onset was 58 years. The initial symptom of 39 patients was pure cerebellar dysfunction. Twenty-nine patients presented pure parkinsonism as the initial symptom. The initial symptom of 23 patients was pure dysautonomia. By the last clinical visit, 54 patients had cerebellar dysfunction, parkinsonism, autonomic failure and pyramidal signs. 　Conclusion　 The study revealed a predominance of MSA-C patients. The differentiation of MSA and other forms of parkinsonism or cerebellar ataxia may be difficult at the early stage. For more accurate diagnosis, it is important to carefully observe the clinical progression.

引用本文： 趙璧,黃睿,宋偉,商慧芳. 多系統(tǒng)萎縮患者的臨床特點(diǎn)分析. 華西醫(yī)學(xué), 2011, 26(5): 663-666. doi: 復(fù)制

1. 　Gilman S, Wenning GK, Low PA, et al. Second consensus statement on the diagnosis of multiple system atrophy[J]. Neurology, 2008, 71(9): 670-676.
2. 　Santens P, Crevits L, Van der Linden C. Raynaud’s phenomenon in a case of multiple system atrophy[J]. Mov Disord, 1996, 11(5): 586-588.
3. 　Wenning GK, Tison F, Ben-Shlomo Y, et al. Multiple system atrophy: a review of 203 pathologically proven cases[J]. Mov Disord, 1997, 12(2): 133-147.
4. 　Watanabe H, Saito Y, Terao S, et al. Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients[J]. Brain, 2002, 125(Pt 5): 1070-1083.
5. 　Yabe I, Soma H, Takei A, et al. MSA-C is the predominant clinical phenotype of MSA in Japan: analysis of 142 patients with probable MSA[J]. J Neurol Sci, 2006, 249(2): 115-121.
6. 　Jamora RD, Gupta A, Tan AK, et al. Clinical characteristics of patients with multiple system atrophy in Singapore[J]. Ann Acad Med Singapore, 2005, 34(9): 553-557.
7. 　Vanacore N, Bonifati V, Fabbrini G, et al. Epidemiology of multiple system atrophy. ESGAP Consortium. European Study Group on Atypical Parkinsonisms[J]. Neurol Sci, 2001, 22(1): 97-99.
8. 　李鑫, 石正洪, 李生泮, 等. 不同類(lèi)型多系統(tǒng)萎縮臨床表現(xiàn)與磁共振成像特征分析[J]. 中國(guó)全科醫(yī)學(xué), 2010, 13(14): 1548-1551.
9. 　何振巍, 張朝東. 多系統(tǒng)萎縮的臨床診斷研究進(jìn)展[J]. 中風(fēng)與神經(jīng)疾病雜志, 2010, 27(1): 83-85.
10. 　Geser F, Wenning GK, Seppi K, et al. Progression of multiple system atrophy (MSA): a prospective natural history study by the European MSA Study Group (EMSA SG)[J]. Mov Disord, 2006, 21(2): 179-186.
11. 　Litvan I, Goetz CG, Jankovic J, et al. What is the accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathologic study[J]. Arch Neurol, 1997, 54(8): 937-944.
12. 　Papapetropoulos S, Tuchman A, Laufer D, et al. Causes of death in multiple system atrophy[J]. J Neurol Neurosurg Psychiatry, 2007, 78(3): 327-329.
13. 　Sakakibara R, Hattori T, Uchiyama T, et al. Urinary dysfunction and orthostatic hypotension in multiple system atrophy: which is the more common and earlier manifestation?[J]. J Neurol Neurosurg Psychiatry, 2000, 68(1): 65-69.
14. 　Boesch SM, Wenning GK, Ransmayr G, et al. Dystonia in multiple system atrophy[J]. J Neurol Neurosurg Psychiatry, 2002, 72(3): 300-303.
15. 　Slawek J, Derejko M, Lass P, et al. Camptocormia or Pisa syndrome in multiple system atrophy[J]. Clin Neurol Neurosurg, 2006, 108(7): 699-704.
16. 　Geser F, Wenning GK. Disproportionate antecollis: a warning sign for multiple system atrophy[J]. Mov Disord, 2007, 22(13): 1986.
17. 　Hughes RG, Gibbin KP, Lowe J. Vocal fold abductor paralysis as a solitary and fatal manifestation of multiple system atrophy[J]. J Laryngol Otol, 1998, 112(2): 177-178.
18. 　Silber MH, Levine S. Stridor and death in multiple system atrophy[J]. Mov Disord, 2000, 15(4): 699-704.
19. 　Reich SG. The cold hands sign in MSA. Multiple system atrophy[J]. Neurology,2003, 60(4): 719.
20. 　Parvizi J, Joseph J, Press DZ, et al. Pathological laughter and crying in patients with multiple system atrophy-cerebellar type[J]. Mov Disord, 2007, 22(6): 798-803.
21. 　Shimohata T, Shinoda H, Nakayama H, et al. Daytime hypoxemia, sleep-disordered breathing, and laryngopharyngeal findings in multiple system atrophy[J]. Arch Neurol, 2007, 64(6): 856-861.
  1. 1. 　Gilman S, Wenning GK, Low PA, et al. Second consensus statement on the diagnosis of multiple system atrophy[J]. Neurology, 2008, 71(9): 670-676.
  2. 2. 　Santens P, Crevits L, Van der Linden C. Raynaud’s phenomenon in a case of multiple system atrophy[J]. Mov Disord, 1996, 11(5): 586-588.
  3. 3. 　Wenning GK, Tison F, Ben-Shlomo Y, et al. Multiple system atrophy: a review of 203 pathologically proven cases[J]. Mov Disord, 1997, 12(2): 133-147.
  4. 4. 　Watanabe H, Saito Y, Terao S, et al. Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients[J]. Brain, 2002, 125(Pt 5): 1070-1083.
  5. 5. 　Yabe I, Soma H, Takei A, et al. MSA-C is the predominant clinical phenotype of MSA in Japan: analysis of 142 patients with probable MSA[J]. J Neurol Sci, 2006, 249(2): 115-121.
  6. 6. 　Jamora RD, Gupta A, Tan AK, et al. Clinical characteristics of patients with multiple system atrophy in Singapore[J]. Ann Acad Med Singapore, 2005, 34(9): 553-557.
  7. 7. 　Vanacore N, Bonifati V, Fabbrini G, et al. Epidemiology of multiple system atrophy. ESGAP Consortium. European Study Group on Atypical Parkinsonisms[J]. Neurol Sci, 2001, 22(1): 97-99.
  8. 8. 　李鑫, 石正洪, 李生泮, 等. 不同類(lèi)型多系統(tǒng)萎縮臨床表現(xiàn)與磁共振成像特征分析[J]. 中國(guó)全科醫(yī)學(xué), 2010, 13(14): 1548-1551.
  9. 9. 　何振巍, 張朝東. 多系統(tǒng)萎縮的臨床診斷研究進(jìn)展[J]. 中風(fēng)與神經(jīng)疾病雜志, 2010, 27(1): 83-85.
  10. 10. 　Geser F, Wenning GK, Seppi K, et al. Progression of multiple system atrophy (MSA): a prospective natural history study by the European MSA Study Group (EMSA SG)[J]. Mov Disord, 2006, 21(2): 179-186.
  11. 11. 　Litvan I, Goetz CG, Jankovic J, et al. What is the accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathologic study[J]. Arch Neurol, 1997, 54(8): 937-944.
  12. 12. 　Papapetropoulos S, Tuchman A, Laufer D, et al. Causes of death in multiple system atrophy[J]. J Neurol Neurosurg Psychiatry, 2007, 78(3): 327-329.
  13. 13. 　Sakakibara R, Hattori T, Uchiyama T, et al. Urinary dysfunction and orthostatic hypotension in multiple system atrophy: which is the more common and earlier manifestation?[J]. J Neurol Neurosurg Psychiatry, 2000, 68(1): 65-69.
  14. 14. 　Boesch SM, Wenning GK, Ransmayr G, et al. Dystonia in multiple system atrophy[J]. J Neurol Neurosurg Psychiatry, 2002, 72(3): 300-303.
  15. 15. 　Slawek J, Derejko M, Lass P, et al. Camptocormia or Pisa syndrome in multiple system atrophy[J]. Clin Neurol Neurosurg, 2006, 108(7): 699-704.
  16. 16. 　Geser F, Wenning GK. Disproportionate antecollis: a warning sign for multiple system atrophy[J]. Mov Disord, 2007, 22(13): 1986.
  17. 17. 　Hughes RG, Gibbin KP, Lowe J. Vocal fold abductor paralysis as a solitary and fatal manifestation of multiple system atrophy[J]. J Laryngol Otol, 1998, 112(2): 177-178.
  18. 18. 　Silber MH, Levine S. Stridor and death in multiple system atrophy[J]. Mov Disord, 2000, 15(4): 699-704.
  19. 19. 　Reich SG. The cold hands sign in MSA. Multiple system atrophy[J]. Neurology,2003, 60(4): 719.
  20. 20. 　Parvizi J, Joseph J, Press DZ, et al. Pathological laughter and crying in patients with multiple system atrophy-cerebellar type[J]. Mov Disord, 2007, 22(6): 798-803.
  21. 21. 　Shimohata T, Shinoda H, Nakayama H, et al. Daytime hypoxemia, sleep-disordered breathing, and laryngopharyngeal findings in multiple system atrophy[J]. Arch Neurol, 2007, 64(6): 856-861.