UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate®

多系统萎缩:预后与治疗

Authors
Stewart A Factor, DO
Christine Doss Esper, MD
Section Editor
Howard I Hurtig, MD
Deputy Editor
John F Dashe, MD, PhD
Translators
张玉生, 主任医师,副教授

引言

多系统萎缩(multiple system atrophy, MSA)是一组神经变性疾病的统称术语,这些综合征包括:橄榄体脑桥小脑萎缩(olivopontocerebellar atrophy, OPCA)、纹状体黑质变性和Shy-Drager综合征,其特征为不同程度的自主神经功能障碍、小脑异常、帕金森综合征和皮质脊髓变性。

本文将总结MSA的预后和治疗。MSA的其他临床方面见其他专题。 (参见“多系统萎缩:临床特征与诊断”)

预后

MSA通常在1-18年期间进展,常常比特发性帕金森病的进展快[1]。

在主要为回顾性的研究中,MSA从发病到残疾里程碑的中位时间如下:

发生自主神经功能障碍,2.5年[2]

           

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: 2017-07 . | This topic last updated: 2017-05-09.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
References
Top
  1. Wenning GK, Ben Shlomo Y, Magalhães M, et al. Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. Brain 1994; 117 ( Pt 4):835.
  2. Tada M, Onodera O, Tada M, et al. Early development of autonomic dysfunction may predict poor prognosis in patients with multiple system atrophy. Arch Neurol 2007; 64:256.
  3. Watanabe H, Saito Y, Terao S, et al. Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain 2002; 125:1070.
  4. Schrag A, Wenning GK, Quinn N, Ben-Shlomo Y. Survival in multiple system atrophy. Mov Disord 2008; 23:294.
  5. Ben-Shlomo Y, Wenning GK, Tison F, Quinn NP. Survival of patients with pathologically proven multiple system atrophy: a meta-analysis. Neurology 1997; 48:384.
  6. Bjornsdottir A, Gudmundsson G, Blondal H, Olafsson E. Incidence and prevalence of multiple system atrophy: a nationwide study in Iceland. J Neurol Neurosurg Psychiatry 2013; 84:136.
  7. Wenning GK, Geser F, Krismer F, et al. The natural history of multiple system atrophy: a prospective European cohort study. Lancet Neurol 2013; 12:264.
  8. Low PA, Reich SG, Jankovic J, et al. Natural history of multiple system atrophy in the USA: a prospective cohort study. Lancet Neurol 2015; 14:710.
  9. Wenning GK, Colosimo C, Geser F, Poewe W. Multiple system atrophy. Lancet Neurol 2004; 3:93.
  10. Petrovic IN, Ling H, Asi Y, et al. Multiple system atrophy-parkinsonism with slow progression and prolonged survival: a diagnostic catch. Mov Disord 2012; 27:1186.
  11. O'Sullivan SS, Massey LA, Williams DR, et al. Clinical outcomes of progressive supranuclear palsy and multiple system atrophy. Brain 2008; 131:1362.
  12. Calandra-Buonaura G, Guaraldi P, Sambati L, et al. Multiple system atrophy with prolonged survival: is late onset of dysautonomia the clue? Neurol Sci 2013; 34:1875.
  13. Stefanova N, Bücke P, Duerr S, Wenning GK. Multiple system atrophy: an update. Lancet Neurol 2009; 8:1172.
  14. Munschauer FE, Loh L, Bannister R, Newsom-Davis J. Abnormal respiration and sudden death during sleep in multiple system atrophy with autonomic failure. Neurology 1990; 40:677.
  15. Jin K, Okabe S, Chida K, et al. Tracheostomy can fatally exacerbate sleep-disordered breathing in multiple system atrophy. Neurology 2007; 68:1618.
  16. Colosimo C, Pezzella FR. The symptomatic treatment of multiple system atrophy. Eur J Neurol 2002; 9:195.
  17. Iwasaki Y, Wakata N, Kinoshita M. Parkinsonism induced by pyridostigmine. Acta Neurol Scand 1988; 78:236.
  18. Kao KP, Kwan SY, Lin KP, Chang YC. Coexistence of Parkinson's disease and myasthenia gravis: a case report. Clin Neurol Neurosurg 1993; 95:137.
  19. Schenck CH, Mahowald MW. Rapid eye movement sleep parasomnias. Neurol Clin 2005; 23:1107.
  20. Panossian LA, Avidan AY. Review of sleep disorders. Med Clin North Am 2009; 93:407.
  21. Lee PH, Lee JE, Kim HS, et al. A randomized trial of mesenchymal stem cells in multiple system atrophy. Ann Neurol 2012; 72:32.
  22. Palma JA, Kaufmann H. Novel therapeutic approaches in multiple system atrophy. Clin Auton Res 2015; 25:37.