Abstract
Epidemiology seeks to prevent disease by identifying risk factors, be they genetic and/or environmental, that are of etiological relevance through the use of descriptive studies, natural experiments, and very occasionally randomized trials. In addition, clinical epidemiology can examine the utility of diagnostic tests, determine predictors of disease prognosis, and test whether therapies can modify disease progression. This chapter will focus on descriptive studies that have either measured disease frequency (prevalence or incidence) or estimated the risk associated with environmental exposures. We have chosen to focus solely on progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) as there is hardly any data for other atypical parkinsonian disorders such as corticobasal degeneration or dementia with Lewy bodies. For any uncommon disease, undertaking epidemiological studies presents several major challenges: case identification, representativeness of cases, and obtaining adequate sample sizes. For both PSP and MSA, there are additional problems as both these conditions can be difficult to diagnose. As there are no adequate tests that are both highly sensitive and specific, the gold standard remains the diagnostic expertise of a movement disorders specialist (1).
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References
Hughes AJ, Daniel SE, Ben-Shlomo Y, Lees AJ. The accuracy of diagnosis of parkinsonian syndromes in a specialist movement disorder service. Brain 2002;125:861–870
Gilman S, Low P, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci 1999;163(1):94–98.
Litvan I, Agid Y, Calne D, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 1996;47(1):1–9.
Litvan I, Bhatia KV, Burn DJ, et al. Movement Disorders Society Scientific Issues Committee report: SIC Task Force appraisal of clinical diagnostic criteria for Parkinsonian disorders. Mov Disord 2003;18(5): 467–486.
Rajput AH, Offord KP, Beard M, Kurland LT. Epidemiology of parkinsonism: incidence, classification and mortality. Ann Neurol 1984;16:278–282.
Golbe LI, Davis PH, Schoenberg, et al. Prevalence and Natural History of Progressive Supranuclear Palsy. Neurology 1988;38:1031–1034.
Radhakrishnan K, Thacker AK, Maloo JC, et al Descriptive epidemiology of some rare neurological diseases in Benghazi, Libya. Neuroepidemiology 1988;7(3):159–164.
Morgante L, Rocca WA, Di Rosa AE, et al. Prevalence of Parkinson’s disease and other types of Parkinsonism: a door-to-door survey in three Sicillian municipalities. Neurology 1992;42:1901–1907.
de Rijk MC, Breteler MMB, Graveland GA, et al. Prevalence of Parkinson’s disease in the elderly: the Rotterdam Study. Neurology, 1995;45(12):2143–2146.
Trenkwalder C, Schwarz J, Gebhard J, et al. Starnberg trial on epidemiology of Parkinsonism and hypertension in the elderly. Prevalence of Parkinson’s disease and related disorders assessed by a door-to-door survey of inhabitants older than 65 years. Arch Neurol 1995;52(10):1017–1022.
Bower JH, Maranganore DM, McDonnell SK, Rocca W. Incidence of progressive supranuclear palsy and multiple system atrophy in Olmsted County, Minnesota, 1976 to 1990. Neurology 1997;49(5):1284–1288.
Wermuth, L, Joensen P, Bunger N, Jeune B. High prevalence of Parkinson’s disease in the Faroe Islands. Neurology 1997;49(2):426–432.
Chio A, Magnani C, Schiffer D. Prevalence of Parkinson’s disease in Northwestern Italy: comparison of tracer methodology and clinical ascertainment of cases. Mov Disord 1998;13(3):400–405.
Schrag A., Ben-Shlomo Y, Quinn NP. Prevalence of progressive supranuclear palsy and multiple system atrophy: a cross-sectional study. Lancet 1999;354:1771–1775.
Nath U, Ben-Shlomo Y, Thomson RG, et al. The Prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK. Brain 2001;124:1438–1449.
Matthews WB, Compston A, Allen IV, Martyn CN. McAlpine’s Mmultiple sclerosis, 2 ed. Edinburgh: Churchill Livingstone, 1990.
de Rijk MC, Tzourio C, Breteler MM, et al. Prevalence of parkinsonism and Parkinson’s disease in Europe: the EUROPARKINSON Collaborative Study. European Community Concerted Action on the Epidemiology of Parkinson’s disease. J Neurol Neurosurg Psychiatry 1997;62:10–15.
Steele JC, Caparros-Lefebvre D, Lees AJ, Sacks OW. Progressive supranuclear palsy and its relation to pacific foci of the parkinsonism-dementia complex and Guadeloupean parkinsonism. Parkinsonism Relat Disord 2003;9: 39–54.
Chaudhuri KR, Hu MT, Brooks DJ. Atypical parkinsonism in Afro-Caribbean and Indian origin immigrants to the UK. Mov Disord 2000;15(1):18–23.
Duvoisin RC, Yahr MD, Schweitzer MD, Merritt HH. Parkinsonism before and since the epidemic of Encephalitis Lethargic. Arch Neurol 1963;9:232–236.
Langston JW, Ballard P, Tetrud JW, Irwin I. Chronic parkinsonism in humans due to a product of meperidine-analog synthesis. Science 1983;219:970–980.
Sanchez-Ramos JR, Hefti F, Weiner WJ. Paraquat and Parkinson’s disease. Neurology 1987;37: 728.
Caparros-Lefebvre D, Elbaz A, and the Caribbean Parkinsonism Study Group. Possible relation of atypical parkinsonism in the French West Indies with consumption of tropical plants: a case-control study. Lancet 1999; 354:281–286.
Davis PH, Golbe LI, Duvoisin RC, Schoenberg BS. Risk factors for progressive supranuclear palsy. Neurology 1988;38(10):1546–1552.
Golbe LI, Rubin RS, Cody RP et al. Follow-up study of risk factors in progressive supranuclear palsy. Neurology 1996;47(1):148–54.
Nee LE, Gomez MR, Dambrosia J, Bale S, Eldridge R, Polinsky RJ. Environmental-occupational risk factors and familial associations in multiple system atrophy: a preliminary investigation. Clin Auton Res 1991;1(1): 9–13.
Vanacore, N., et al., Smoking habits in multiple system atrophy and progressive supranuclear palsy. European Study Group on Atypical Parkinsonisms. Neurology 2000;54(1):114–119.
Hanna PA, Jankovic J, Kirkpatrick JB. Multiple system atrophy: the putative causative role of environmental toxins. Arch Neurol 1999;56(1):90–94.
McCrank E. PSP risk factors. Neurology 1990; 40:1637.
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© 2005 Humana Press Inc., Totowa, NJ
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Zermansky, A., Ben-Shlomo, Y. (2005). Epidemiology of Progessive Supranuclear Palsy and Multiple System Atrophy. In: Litvan, I. (eds) Atypical Parkinsonian Disorders. Current Clinical Neurology. Humana Press. https://doi.org/10.1385/1-59259-834-X:023
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DOI: https://doi.org/10.1385/1-59259-834-X:023
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