Abstract
MSA is an orphan neurodegenerative disease for which no curative therapy is presently available. Motor as well as non-motor symptoms progress relentlessly, fully revealing the malignant nature of the disorder that has been rightly likened to a beast (Quinn 1989). In the last decades, multiple factors have been identified to play a pivotal prognostic role in the disease. Awareness of these is all the more important for an appropriate management and counselling of MSA patients.
Abstract
MSA is an orphan neurodegenerative disease for which no curative therapy is presently available. Motor as well as non-motor symptoms progress relentlessly, fully revealing the malignant nature of the disorder that has been rightly likened to a beast (Quinn 1989).
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Ben-Shlomo Y, Wenning GK, Tison F, Quinn NP (1997) Survival of patients with pathologically proven multiple system atrophy: a meta-analysis. Neurology 48:384–393
Brown RG, Lacomblez L, Landwehrmeyer BG, Bak T, Uttner I, Dubois B, Agid Y, Ludolph A, Bensimon G, Payan C, Leigh NP (2010) Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain 133:2382–2393
Colosimo C, Morgante L, Antonini A, Barone P, Avarello TP, Bottacchi E, Cannas A, Ceravolo MG, Ceravolo R, Cicarelli G, Gaglio RM, Giglia L, Iemolo F, Manfredi M, Meco G, Nicoletti A, Pederzoli M, Petrone A, Pisani A, Pontieri FE, Quatrale R, Ramat S, Scala R, Volpe G, Zappulla S, Bentivoglio AR, Stocchi F, Trianni G, del Dotto P, Simoni L, Marconi R (2010) Non-motor symptoms in atypical and secondary parkinsonism: the PRIAMO study. J Neurol 257:5–14
Deguchi K, Takeuchi H, Sasaki I, Tsukaguchi M, Touge T, Nishioka M (2001) Impaired novelty P3 potentials in multiple system atrophy – correlation with orthostatic hypotension. J Neurol Sci 190:61–67
Eigenbrodt ML, Rose KM, Couper DJ, Arnett DK, Smith R, Jones D (2000) Orthostatic hypotension as a risk factor for stroke: the atherosclerosis risk in communities (ARIC) study, 1987-1996. Stroke 31:2307–2313
Fanciulli A, Strano S, Colosimo C, Caltagirone C, Spalletta G, Pontieri FE (2013) The potential prognostic role of cardiovascular autonomic failure in alpha-synucleinopathies. Eur J Neurol 20:231–235
Fernagut PO, Vital A, Canron MH, Tison F, Meissner WG (2012) Ambiguous mechanisms of dysphagia in multiple system atrophy. Brain 135:e205; author reply e206
Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ, Wood NW, Colosimo C, Durr A, Fowler CJ, Kaufmann H, Klockgether T, Lees A, Poewe W, Quinn N, Revesz T, Robertson D, Sandroni P, Seppi K, Vidailhet M (2008) Second consensus statement on the diagnosis of multiple system atrophy. Neurology 71:670–676
Hirayama M, Fukatsu H, Watanabe H, Koike Y, Noda A, Ito H, Kobayashi R, Sobue G (2003) Sequential constriction of upper airway and vocal cords in sleep apnoea of multiple system atrophy: low field magnetic resonance fluoroscopic study. J Neurol Neurosurg Psychiatry 74:642–645
Hori N, Takamori M, Hirayama M, Watanabe H, Nakamura T, Yamashita F, Ito H, Mabuchi N, Sobue G (2008) Pupillary supersensitivity and visual disturbance in Parkinson’s disease. Clin Auton Res 18:20–27
Jecmenica-Lukic M, Poewe W, Tolosa E, Wenning GK (2012) Premotor signs and symptoms of multiple system atrophy. Lancet Neurol 11:361–368
Kao AW, Racine CA, Quitania LC, Kramer JH, Christine CW, Miller BL (2009) Cognitive and neuropsychiatric profile of the synucleinopathies: Parkinson disease, dementia with Lewy bodies, and multiple system atrophy. Alzheimer Dis Assoc Disord 23:365–370
Kim HJ, Jeon BS, Lee JY, Yun JY (2011) Survival of Korean patients with multiple system atrophy. Mov Disord 26:909–912
Klockgether T, Ludtke R, Kramer B, Abele M, Burk K, Schols L, Riess O, Laccone F, Boesch S, Lopes-Cendes I, Brice A, Inzelberg R, Zilber N, Dichgans J (1998) The natural history of degenerative ataxia: a retrospective study in 466 patients. Brain 121(Pt 4):589–600
Kollensperger M, Geser F, Seppi K, Stampfer-Kountchev M, Sawires M, Scherfler C, Boesch S, Mueller J, Koukouni V, Quinn N, Pellecchia MT, Barone P, Schimke N, Dodel R, Oertel W, Dupont E, Ostergaard K, Daniels C, Deuschl G, Gurevich T, Giladi N, Coelho M, Sampaio C, Nilsson C, Widner H, Sorbo FD, Albanese A, Cardozo A, Tolosa E, Abele M, Klockgether T, Kamm C, Gasser T, Djaldetti R, Colosimo C, Meco G, Schrag A, Poewe W, Wenning GK (2008) Red flags for multiple system atrophy. Mov Disord 23:1093–1099
Lim TS, Lee PH, Kim HS, Yong SW (2009) White matter hyperintensities in patients with multiple system atrophy. J Neurol 256:1663–1670
Marconi R, Antonini A, Barone P, Colosimo C, Avarello TP, Bottacchi E, Cannas A, Ceravolo MG, Ceravolo R, Cicarelli G, Gaglio RM, Giglia L, Iemolo F, Manfredi M, Meco G, Nicoletti A, Pederzoli M, Petrone A, Pisani A, Pontieri FE, Quatrale R, Ramat S, Scala R, Volpe G, Zappulla S, Bentivoglio AR, Stocchi F, Trianni G, del Dotto P, de Gaspari D, Grasso L, Morgante F, Santangelo G, Fabbrini G, Morgante L (2012) Frontal assessment battery scores and non-motor symptoms in parkinsonian disorders. Neurol Sci 33:585–593
Maule S, Milan A, Grosso T, Veglio F (2006) Left ventricular hypertrophy in patients with autonomic failure. Am J Hypertens 19:1049–1054
Maule S, Milazzo V, Maule MM, di Stefano C, Milan A, Veglio F (2012) Mortality and prognosis in patients with neurogenic orthostatic hypotension. Funct Neurol 27:101–106
May S, Gilman S, Sowell BB, Thomas RG, Stern MB, Colcher A, Tanner CM, Huang N, Novak P, Reich SG, Jankovic J, Ondo WG, Low PA, Sandroni P, Lipp A, Marshall FJ, Wooten F, Shults CW (2007) Potential outcome measures and trial design issues for multiple system atrophy. Mov Disord 22:2371–2377
Mehanna R, Jankovic J (2010) Respiratory problems in neurologic movement disorders. Parkinsonism Relat Disord 16:628–638
Muller J, Wenning GK, Jellinger K, McKee A, Poewe W, Litvan I (2000) Progression of Hoehn and Yahr stages in Parkinsonian disorders: a clinicopathologic study. Neurology 55:888–891
Muller J, Wenning GK, Verny M, McKee A, Chaudhuri KR, Jellinger K, Poewe W, Litvan I (2001) Progression of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders. Arch Neurol 58:259–264
O’Sullivan SS, Massey LA, Williams DR, Silveira-Moriyama L, Kempster PA, Holton JL, Revesz T, Lees AJ (2008) Clinical outcomes of progressive supranuclear palsy and multiple system atrophy. Brain 131:1362–1372
Papapetropoulos S, Tuchman A, Laufer D, Papatsoris AG, Papapetropoulos N, Mash DC (2007) Causes of death in multiple system atrophy. J Neurol Neurosurg Psychiatry 78:327–329
Paterson DS, Trachtenberg FL, Thompson EG, Belliveau RA, Beggs AH, Darnall R, Chadwick AE, Krous HF, Kinney HC (2006) Multiple serotonergic brainstem abnormalities in sudden infant death syndrome. JAMA 296:2124–2132
Petrovic IN, Ling H, Asi Y, Ahmed Z, Kukkle PL, Hazrati LN, Lang AE, Revesz T, Holton JL, Lees AJ (2012) Multiple system atrophy-parkinsonism with slow progression and prolonged survival: a diagnostic catch. Mov Disord 27:1186–1190
Poda R, Guaraldi P, Solieri L, Calandra-Buonaura G, Marano G, Gallassi R, Cortelli P (2012) Standing worsens cognitive functions in patients with neurogenic orthostatic hypotension. Neurol Sci 33:469–473
Quinn N (1989) Multiple system atrophy–the nature of the beast. J Neurol Neurosurg Psychiatry S2(Suppl):78–89
Rose KM, Tyroler HA, Nardo CJ, Arnett DK, Light KC, Rosamond W, Sharrett AR, Szklo M (2000) Orthostatic hypotension and the incidence of coronary heart disease: the Atherosclerosis Risk in Communities study. Am J Hypertens 13:571–578
Rose KM, Eigenbrodt ML, Biga RL, Couper DJ, Light KC, Sharrett AR, Heiss G (2006) Orthostatic hypotension predicts mortality in middle-aged adults: the Atherosclerosis Risk In Communities (ARIC) Study. Circulation 114:630–636
Saito Y, Matsuoka Y, Takahashi A, Ohno Y (1994) Survival of patients with multiple system atrophy. Intern Med 33:321–325
Sakakibara R, Hattori T, Uchiyama T, Kita K, Asahina M, Suzuki A, Yamanishi T (2000) Urinary dysfunction and orthostatic hypotension in multiple system atrophy: which is the more common and earlier manifestation? J Neurol Neurosurg Psychiatry 68:65–69
Schrag A, Geser F, Stampfer-Kountchev M, Seppi K, Sawires M, Kollensperger M, Scherfler C, Quinn N, Pellecchia MT, Barone P, del Sorbo F, Albanese A, Ostergaard K, Dupont E, Cardozo A, Tolosa E, Nilsson CF, Widner H, Lindvall O, Giladi N, Gurevich T, Daniels C, Deuschl G, Coelho M, Sampaio C, Abele M, Klockgether T, Schimke N, Eggert KM, Oertel W, Djaldetti R, Colosimo C, Meco G, Poewe W, Wenning GK (2006) Health-related quality of life in multiple system atrophy. Mov Disord 21:809–815
Schrag A, Wenning GK, Quinn N, Ben-Shlomo Y (2008) Survival in multiple system atrophy. Mov Disord 23:294–296
Schulz JB, Klockgether T, Petersen D, Jauch M, Muller-Schauenburg W, Spieker S, Voigt K, Dichgans J (1994) Multiple system atrophy: natural history, MRI morphology, and dopamine receptor imaging with 123IBZM-SPECT. J Neurol Neurosurg Psychiatry 57:1047–1056
Seppi K, Yekhlef F, Diem A, Luginger Wolf E, Mueller J, Tison F, Quinn NP, Poewe W, Wenning GK (2005) Progression of parkinsonism in multiple system atrophy. J Neurol 252:91–96
Shimohata T, Ozawa T, Nakayama H, Tomita M, Shinoda H, Nishizawa M (2008) Frequency of nocturnal sudden death in patients with multiple system atrophy. J Neurol 255:1483–1485
Siderowf A, Lang AE (2012) Premotor Parkinson’s disease: concepts and definitions. Mov Disord 27:608–616
Tada M, Onodera O, Ozawa T, Piao YS, Kakita A, Takahashi H, Nishizawa M (2007) Early development of autonomic dysfunction may predict poor prognosis in patients with multiple system atrophy. Arch Neurol 64:256–260
Tada M, Kakita A, Toyoshima Y, Onodera O, Ozawa T, Morita T, Nishizawa M, Takahashi H (2009) Depletion of medullary serotonergic neurons in patients with multiple system atrophy who succumbed to sudden death. Brain 132:1810–1819
Testa D, Monza D, Ferrarini M, Soliveri P, Girotti F, Filippini G (2001) Comparison of natural histories of progressive supranuclear palsy and multiple system atrophy. Neurol Sci 22:247–251
Tha KK, Terae S, Yabe I, Miyamoto T, Soma H, Zaitsu Y, Fujima N, Kudo K, Sasaki H, Shirato H (2010) Microstructural white matter abnormalities of multiple system atrophy: in vivo topographic illustration by using diffusion-tensor MR imaging. Radiology 255:563–569
Umoto M, Miwa H, Ando R, Kajimoto Y, Kondo T (2012) White matter hyperintensities in patients with multiple system atrophy. Parkinsonism Relat Disord 18:17–20
Vagaonescu TD, Saadia D, Tuhrim S, Phillips RA, Kaufmann H (2000) Hypertensive cardiovascular damage in patients with primary autonomic failure. Lancet 355:725–726
Watanabe H, Saito Y, Terao S, Ando T, Kachi T, Mukai E, Aiba I, Abe Y, Tamakoshi A, Doyu M, Hirayama M, Sobue G (2002) Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain 125:1070–1083
Wenning GK, Ben Shlomo Y, Magalhaes M, Daniel SE, Quinn NP (1994) Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. Brain 117(Pt 4):835–845
Wenning GK, Tison F, Ben Shlomo Y, Daniel SE, Quinn NP (1997) Multiple system atrophy: a review of 203 pathologically proven cases. Mov Disord 12:133–147
Wenning GK, Geser F, Krismer F, Seppi K, Duerr S, Boesch S, Kollensperger M, Goebel G, Pfeiffer KP, Barone P, Pellecchia MT, Quinn NP, Koukouni V, Fowler CJ, Schrag A, Mathias CJ, Giladi N, Gurevich T, Dupont E, Ostergaard K, Nilsson CF, Widner H, Oertel W, Eggert KM, Albanese A, del Sorbo F, Tolosa E, Cardozo A, Deuschl G, Hellriegel H, Klockgether T, Dodel R, Sampaio C, Coelho M, Djaldetti R, Melamed E, Gasser T, Kamm C, Meco G, Colosimo C, Rascol O, Meissner WG, Tison F, Poewe W (2013) The natural history of multiple system atrophy: a prospective European cohort study. Lancet Neurol 12:264–274
Yamashita F, Hirayama M, Nakamura T, Takamori M, Hori N, Uchida K, Hama T, Sobue G (2010) Pupillary autonomic dysfunction in multiple system atrophy and Parkinson’s disease: an assessment by eye-drop tests. Clin Auton Res 20:191–197
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer-Verlag Wien
About this chapter
Cite this chapter
Fanciulli, A., Wenning, G.K. (2014). Natural History. In: Wenning, G., Fanciulli, A. (eds) Multiple System Atrophy. Springer, Vienna. https://doi.org/10.1007/978-3-7091-0687-7_8
Download citation
DOI: https://doi.org/10.1007/978-3-7091-0687-7_8
Published:
Publisher Name: Springer, Vienna
Print ISBN: 978-3-7091-0686-0
Online ISBN: 978-3-7091-0687-7
eBook Packages: MedicineMedicine (R0)