The Cerebellum

, Volume 11, Issue 1, pp 223–226 | Cite as

Orthostatic Hypotension Is Differentially Associated with the Cerebellar Versus the Parkinsonian Variant of Multiple System Atrophy: a Comparative Study

  • Gregor Karl Wenning
  • Roberta Granata
  • Florian Krismer
  • Susanne Dürr
  • Klaus Seppi
  • Werner Poewe
  • Katharine Bleasdale-Barr
  • Christopher J. Mathias
Original Paper


Orthostatic hypotension (OH) is a cardinal feature of autonomic failure in multiple system atrophy (MSA); however, there are few comparative data on OH in the motor subtypes of MSA. In the present retrospective study, postural blood pressure drop after 3 min of standing was determined in 16 patients with the cerebellar variant of MSA (MSA-C) and in 17 patients with the Parkinson variant (MSA-P). Twenty idiopathic Parkinson’s disease (IPD) patients matched for age, sex, disease duration and dopaminergic therapy served as control group. OH frequency and severity were more pronounced in MSA-C followed by MSA-P and IPD. Differences in brainstem pathology are likely to account for the tight association of MSA-C and OH. A simple standing test should be obligatory in the work-up of patients with sporadic late-onset ataxias.


Orthostatic hypotension Multiple system atrophy Parkinson’s disease Postural blood pressure test Autonomic failure 



We thank the referring consultant neurologists at the National Hospital for Neurology and Neurosurgery, Queen Square, London.

Conflicts of Interest

Financial disclosure related to this manuscript: All authors have nothing to disclose.

Financial disclosure: Gregor K. Wenning has received research grants from the Austrian science fund (FWF), Innsbruck Medical University (MUI) and the Österreichische Nationalbank (OeNB). He also received honoraria for consultancies from AstraZeneca and TEVA. Klaus Seppi has received honoraria for speaking and consulting from Novartis, Boehringer Ingelheim, Lundbeck, Schwarz Pharma, UCB Pharma and GlaxoSmithKline. Florian Krismer was supported by a research grant from österreichische Nationalbank (OeNB). Werner Poewe has received consultancy and lecture fees from AstraZeneca, Teva, Novartis, GSK, Boehringer Ingelheim, UCB, Orion Pharma and Merck Serono in relation to clinical drug development programmes for PD. Roberta Granata, Susanne Dürr, Katharine Bleasdale-Barr and Christopher J. Mathias have nothing to disclose.


  1. 1.
    Wenning GK, Ben Shlomo Y, Magalhaes M, Daniel SE, Quinn NP. Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. Brain. 1994;117(Pt 4):835–45.PubMedCrossRefGoogle Scholar
  2. 2.
    Wenning GK, Scherfler C, Granata R, Bosch S, Verny M, Chaudhuri KR, et al. Time course of symptomatic orthostatic hypotension and urinary incontinence in patients with postmortem confirmed parkinsonian syndromes: a clinicopathological study. J neurol neurosurg psychiatry. 1999;67(5):620–3.PubMedCrossRefGoogle Scholar
  3. 3.
    Wenning GK, Tison F, Ben Shlomo Y, Daniel SE, Quinn NP. Multiple system atrophy: a review of 203 pathologically proven cases. Mov Disord. 1997;12(2):133–47.PubMedCrossRefGoogle Scholar
  4. 4.
    Kirchhof K, Apostolidis AN, Mathias CJ, Fowler CJ. Erectile and urinary dysfunction may be the presenting features in patients with multiple system atrophy: a retrospective study. Int J Impot Res. 2003;15(4):293–8.PubMedCrossRefGoogle Scholar
  5. 5.
    Oertel WH, Wachter T, Quinn NP, Ulm G, Brandstadter D. Reduced genital sensitivity in female patients with multiple system atrophy of parkinsonian type. Mov Disord. 2003;18(4):430–2.PubMedCrossRefGoogle Scholar
  6. 6.
    Daniel S. The neuropathology and neurochemistry of multiple system atrophy. In: Mathias CJ, Bannister R, editors. Autonomic failure: a textbook of clinical disorders of the autonomic nervous system. Oxford: Oxford University Press; 1999. p. 321–8.Google Scholar
  7. 7.
    Sung JH, Mastri AR, Segal E. Pathology of Shy-Drager syndrome. J Neuropathol Exp Neurol. 1979;38(4):353–68.PubMedCrossRefGoogle Scholar
  8. 8.
    Shy GM, Drager GA. A neurological syndrome associated with orthostatic hypotension: a clinical–pathologic study. Arch Neurol. 1960;2:511–27.PubMedCrossRefGoogle Scholar
  9. 9.
    Benarroch EE, Schmeichel AM, Sandroni P, Low PA, Parisi JE. Differential involvement of hypothalamic vasopressin neurons in multiple system atrophy. Brain. 2006;129(Pt 10):2688–96.PubMedCrossRefGoogle Scholar
  10. 10.
    Papp MI, Lantos PL. The distribution of oligodendroglial inclusions in multiple system atrophy and its relevance to clinical symptomatology. Brain. 1994;117(Pt 2):235–43.PubMedCrossRefGoogle Scholar
  11. 11.
    Benarroch EE, Schmeichel AM, Parisi JE. Involvement of the ventrolateral medulla in parkinsonism with autonomic failure. Neurology. 2000;54(4):963–8.PubMedGoogle Scholar
  12. 12.
    Goldstein DS. Dysautonomia in Parkinson’s disease: neurocardiological abnormalities. Lancet neurol. 2003;2(11):669–76.PubMedCrossRefGoogle Scholar
  13. 13.
    Braune S. The role of cardiac metaiodobenzylguanidine uptake in the differential diagnosis of parkinsonian syndromes. Clin Auton Res. 2001;11(6):351–5.PubMedCrossRefGoogle Scholar
  14. 14.
    Courbon F, Brefel-Courbon C, Thalamas C, Alibelli MJ, Berry I, Montastruc JL, et al. Cardiac MIBG scintigraphy is a sensitive tool for detecting cardiac sympathetic denervation in Parkinson’s disease. Mov Disord. 2003;18(8):890–7.PubMedCrossRefGoogle Scholar
  15. 15.
    Kollensperger M, Seppi K, Liener C, Boesch S, Heute D, Mair KJ, et al. Diffusion weighted imaging best discriminates PD from MSA-P: a comparison with tilt table testing and heart MIBG scintigraphy. Mov Disord. 2007;22:1771–6.PubMedCrossRefGoogle Scholar
  16. 16.
    Raffel DM, Koeppe RA, Little R, Wang CN, Liu S, Junck L, et al. PET measurement of cardiac and nigrostriatal denervation in Parkinsonian syndromes. J Nucl Med. 2006;47(11):1769–77.PubMedGoogle Scholar
  17. 17.
    Gilman S, Low P, Quinn N, Albanese A, Ben-Shlomo Y, Fowler C, et al. Consensus statement on the diagnosis of multiple system atrophy. American Autonomic Society and American Academy of Neurology. Clin Auton Res. 1998;8(6):359–62.PubMedCrossRefGoogle Scholar
  18. 18.
    Hughes AJ, Daniel SE, Kilford L, Lees AJ. Accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinico-pathological study of 100 cases. J neurol neurosurg psychiatry. 1992;55(3):181–4.PubMedCrossRefGoogle Scholar
  19. 19.
    The Consensus Committee of the American Autonomic Society and the American Academy of Neurology. Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. Neurology. 1996;46(5):1470.Google Scholar
  20. 20.
    Smith GD, Mathias CJ. Differences in cardiovascular responses to supine exercise and to standing after exercise in two clinical subgroups of Shy-Drager syndrome (multiple system atrophy). J neurol neurosurg psychiatry. 1996;61(3):297–303.PubMedCrossRefGoogle Scholar
  21. 21.
    Senard JM, Rai S, Lapeyre-Mestre M, Brefel C, Rascol O, Rascol A, et al. Prevalence of orthostatic hypotension in Parkinson’s disease. J neurol neurosurg psychiatry. 1997;63(5):584–9.PubMedCrossRefGoogle Scholar
  22. 22.
    Thaisetthawatkul P, Boeve BF, Benarroch EE, Sandroni P, Ferman TJ, Petersen R, et al. Autonomic dysfunction in dementia with Lewy bodies. Neurology. 2004;62(10):1804–9.PubMedGoogle Scholar
  23. 23.
    Krygowska-Wajs A, Furgala A, Laskiewicz J, Szczudlik A, Thor PJ. Early diagnosis of orthostatic hypotension in idiopathic Parkinson’s disease. Folia medica Cracoviensia. 2002;43(1–2):59–67.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Gregor Karl Wenning
    • 1
  • Roberta Granata
    • 1
  • Florian Krismer
    • 1
  • Susanne Dürr
    • 1
  • Klaus Seppi
    • 4
  • Werner Poewe
    • 4
  • Katharine Bleasdale-Barr
    • 2
  • Christopher J. Mathias
    • 2
    • 3
  1. 1.Autonomic Unit, Division of Clinical Neurobiology, Department of NeurologyMedical University InnsbruckInnsbruckAustria
  2. 2.Autonomic UnitThe National Hospital for Neurology and Neurosurgery and Institute of NeurologyLondonUK
  3. 3.Neurovascular Medicine Unit, Faculty of MedicineImperial College London at St. Mary’s HospitalLondonUK
  4. 4.Department of NeurologyMedical University InnsbruckInnsbruckAustria

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