Abstract
The recognition that the course of MS could show striking variation between patients was made in some of the earliest descriptions of the disease. Whereas the majority of patients follow an initial phase of disease characterized by bouts of relapsing neurological dysfunction, or bout onset MS (BOMS), occurring at unpredictable intervals with variable recovery, a minority of patients exhibit a gradual evolution of symptoms commonly related to spinal cord dysfunction but occasionally cerebellar or cognitive in nature. Despite heated debate and considerable investment in investigative studies, it remains unclear whether patients with primary progressive MS (PPMS) represent a distinct disorder or whether a progressive phase of disease is an inevitable consequence of the evolving pathology of disease, and in particular a reflection of gradual axonal degeneration whose origins lie within a dominant early phase of inflammation and demyelination. The debate is complicated by the fact that, despite recent advances in disease classification, there remains no diagnostic test for MS, let alone its clinical subgroups, and that diagnosis is constructed from probabilistic models of accumulated clinical and paraclinical data. Because of this and recognition of distinct immunological or genetic disorders previously considered to be under the rubric of prior MS disease classifications, it seems likely that additional discrete diseases or disease subgroups will emerge over time, but whether these will include forms of progressive disease remains unclear. In the meantime, the epidemiological data available addressing the progressive phases of disease is wide and varied and able to shed some light on these issues.
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References
Andersson PB, et al. Multiple sclerosis that is progressive from the time of onset: clinical characteristics and progression of disability. Arch Neurol. 1999;56(9):1138–42.
Leray E, et al. Evidence for a two-stage disability progression in multiple sclerosis. Brain. 2010;133(Pt 7):1900–13.
Koch M, De Keyser J, Tremlett H. Timing of birth and disease progression in multiple sclerosis. Mult Scler. 2008;14(6):793–8.
Debouverie M, et al. Multiple sclerosis with a progressive course from onset in Lorraine-Eastern France. J Neurol. 2007;254(10):1370–5.
Cottrell DA. The natural history of multiple sclerosis: a geographically based study 5. The clinical features and natural history of primary progressive multiple sclerosis. Brain. 1999;122(Pt 4):625–39.
Confavreux C, et al. Relapses and progression of disability in multiple sclerosis. N Engl J Med. 2000;343(20):1430–8.
Runmarker B, Andersen O. Prognostic factors in a multiple sclerosis incidence cohort with twenty-five years of follow-up. Brain. 1993;116(Pt 1):117–34.
Tremlett H, Paty D, Devonshire V. Disability progression in multiple sclerosis is slower than previously reported. Neurology. 2006;66(2):172–7.
Confavreux C, Aimard G, Devic M. Course and prognosis of multiple sclerosis assessed by the computerized data processing of 349 patients. Brain. 1980;103(2):281–300.
Lublin FD, Reingold SC. Defining the clinical course of multiple sclerosis: results of an international survey. National Multiple Sclerosis Society (USA) Advisory Committee on Clinical Trials of New Agents in Multiple Sclerosis. Neurology. 1996;46(4):907–11.
Minderhoud JM, van der Hoeven JH, Prange AJ. Course and prognosis of chronic progressive multiple sclerosis. Results of an epidemiological study. Acta Neurol Scand. 1988;78(1):10–5.
Koch M, et al. Progression in multiple sclerosis: further evidence of an age dependent process. J Neurol Sci. 2007;255(1–2):35–41.
Weinshenker BG, et al. The natural history of multiple sclerosis: a geographically based study I. Clinical course and disability. Brain. 1989;112(Pt 1):133–46.
Cossburn M, et al. Age at onset as a determinant of presenting phenotype and initial relapse recovery in multiple sclerosis. Mult Scler. 2012;18(1):45–54.
McDonnell GV, Hawkins SA. Clinical study of primary progressive multiple sclerosis in Northern Ireland, UK. J Neurol Neurosurg Psychiatry. 1998;64(4):451–4.
Simone IL, et al. Course and prognosis in early-onset MS: comparison with adult-onset forms. Neurology. 2002;59(12):1922–8.
Kremenchutzky M, et al. The natural history of multiple sclerosis: a geographically based study 9: observations on the progressive phase of the disease. Brain. 2006;129(Pt 3):584–94.
Tremlett H, Paty D, Devonshire V. The natural history of primary progressive MS in British Columbia, Canada. Neurology. 2005;65(12):1919–23.
Tremlett H, Zhao Y, Devonshire V. Natural history comparisons of primary and secondary progressive multiple sclerosis reveals differences and similarities. J Neurol. 2009;256(3):374–81.
Boiko A, et al. Early onset multiple sclerosis: a longitudinal study. Neurology. 2002;59(7):1006–10.
Ruggieri M, et al. Multiple sclerosis in children under 6 years of age. Neurology. 1999;53(3):478–84.
Riise T, et al. Early prognostic factors for disability in multiple sclerosis, a European multicenter study. Acta Neurol Scand. 1992;85(3):212–8.
Stankoff B, et al. Age at onset determines the occurrence of the progressive phase of multiple sclerosis. Neurology. 2007;68(10):779–81.
Kremenchutzky M. The natural history of multiple sclerosis: a geographically based study 7. Progressive-relapsing and relapsing-progressive multiple sclerosis: a re-evaluation. Brain. 1999;122(Pt 10):1941–50.
Koch M, et al. The natural history of primary progressive multiple sclerosis. Neurology. 2009;73(23):1996–2002.
Koch M, et al. The natural history of secondary progressive multiple sclerosis. J Neurol Neurosurg Psychiatry. 2010;81(9):1039–43.
Debouverie M, et al. Earlier disability of the patients followed in Multiple Sclerosis centers compared to outpatients. Mult Scler. 2009;15(2):251–7.
Grytten Torkildsen N. Survival and cause of death in multiple sclerosis: results from a 50-year follow-up in Western Norway. Mult Scler. 2008;14(9):1191–8.
Pekmezovic T, et al. Survival of multiple sclerosis patients in the Belgrade population. Neuroepidemiology. 2002;21(5):235–40.
Phadke JG, Downie AW. Epidemiology of multiple sclerosis in the north-east (Grampian region) of Scotland – an update. J Epidemiol Community Health. 1987;41(1):5–13.
Levic ZM, et al. Prognostic factors for survival in multiple sclerosis. Mult Scler. 1999;5(3):171–8.
Midgard R, et al. Prognostic factors for survival in multiple sclerosis: a longitudinal, population based study in More and Romsdal, Norway. J Neurol Neurosurg Psychiatry. 1995;58(4):417–21.
Riise T, et al. Prognostic factors for life expectancy in multiple sclerosis analysed by Cox-models. J Clin Epidemiol. 1988;41(10):1031–6.
Poser S, et al. Prognostic indicators in multiple sclerosis. Acta Neurol Scand. 1986;74(5):387–92.
Sumelahti ML, et al. Survival of multiple sclerosis in Finland between 1964 and 1993. Mult Scler. 2002;8(4):350–5.
Miller DH, Hornabrook RW, Purdie G. The natural history of multiple sclerosis: a regional study with some longitudinal data. J Neurol Neurosurg Psychiatry. 1992;55(5):341–6.
Trojano M, et al. Multivariate analysis of predictive factors of multiple sclerosis course with a validated method to assess clinical events. J Neurol Neurosurg Psychiatry. 1995;58(3):300–6.
Kantarci O. Survival and predictors of disability in Turkish MS patients. Turkish Multiple Sclerosis Study Group (TUMSSG). Neurology. 1998;51(3):765–72.
Weinshenker BG. The natural history of multiple sclerosis: a geographically based study 3. Multivariate analysis of predictive factors and models of outcome. Brain. 1991;114(Pt 2):1045–56.
Amato MP, et al. A prospective study on the natural history of multiple sclerosis: clues to the conduct and interpretation of clinical trials. J Neurol Sci. 1999;168(2):96–106.
Confavreux C, Vukusic S, Adeleine P. Early clinical predictors and progression of irreversible disability in multiple sclerosis: an amnesic process. Brain. 2003;126(Pt 4):770–82.
Myhr KM, et al. Disability and prognosis in multiple sclerosis: demographic and clinical variables important for the ability to walk and awarding of disability pension. Mult Scler. 2001;7(1):59–65.
Ebers GC. The natural history of multiple sclerosis. Neurol Sci. 2000;21(4 Suppl 2):S815–7.
Khaleeli Z, et al. Predicting progression in primary progressive multiple sclerosis: a 10-year multicenter study. Ann Neurol. 2008;63(6):790–3.
Ferreira Vasconcelos CC. Differences in the progression of primary progressive multiple sclerosis in Brazilians of African descent versus white Brazilian patients. Mult Scler. 2010;16(5):597–603.
Vukusic S, Confavreux C. Prognostic factors for progression of disability in the secondary progressive phase of multiple sclerosis. J Neurol Sci. 2003;206(2):135–7.
Confavreux C, Vukusic S. Natural history of multiple sclerosis: a unifying concept. Brain. 2006;129(Pt 3):606–16.
Binquet C, et al. The prognostic value of initial relapses on the evolution of disability in patients with relapsing-remitting multiple sclerosis. Neuroepidemiology. 2006;27(1):45–54.
Scalfari A, et al. The natural history of multiple sclerosis: a geographically based study 10: relapses and long-term disability. Brain. 2010;133(Pt 7):1914–29.
Tremlett H, Yinshan Z, Devonshire V. Natural history of secondary-progressive multiple sclerosis. Mult Scler. 2008;14(3):314–24.
Bergamaschi R, et al. Disability and mortality in a cohort of multiple sclerosis patients: a reappraisal. Neuroepidemiology. 2005;25(1):15–8.
Eriksson M, Andersen O, Runmarker B. Long-term follow up of patients with clinically isolated syndromes, relapsing-remitting and secondary progressive multiple sclerosis. Mult Scler. 2003;9(3):260–74.
Thompson AJ, et al. Primary progressive multiple sclerosis. Brain. 1997;120(Pt 6):1085–96.
Ebers GC. Natural history of primary progressive multiple sclerosis. Mult Scler. 2004;10 Suppl 1:S8–13; discussion S13–5.
Trojano M, et al. Age-related disability in multiple sclerosis. Ann Neurol. 2002;51(4):475–80.
Confavreux C, Vukusic S. Age at disability milestones in multiple sclerosis. Brain. 2006;129(Pt 3):595–605.
Koch M, et al. Progression in familial and nonfamilial MS. Mult Scler. 2008;14(3):300–6.
Koch M, et al. Factors associated with the risk of secondary progression in multiple sclerosis. Mult Scler. 2008;14(6):799–803.
Bergamaschi R, et al. Predicting secondary progression in relapsing-remitting multiple sclerosis: a Bayesian analysis. J Neurol Sci. 2001;189(1–2):13–21.
Tremlett H, et al. Impact of multiple sclerosis relapses on progression diminishes with time. Neurology. 2009;73(20):1616–23.
Ebers GC, et al. The natural history of multiple sclerosis: a geographically based study 8: familial multiple sclerosis. Brain. 2000;123(Pt 3):641–9.
Koch M, et al. Disease onset in familial and sporadic primary progressive multiple sclerosis. Mult Scler. 2010;16(6):694–700.
Robertson NP, et al. Clinical concordance in sibling pairs with multiple sclerosis. Neurology. 1996;47(2):347–52.
Hensiek AE, et al. Familial effects on the clinical course of multiple sclerosis. Neurology. 2007;68(5):376–83.
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Robertson, N., Hirst, C. (2013). Epidemiology of Progressive Multiple Sclerosis. In: Wilkins, A. (eds) Progressive Multiple Sclerosis. Springer, London. https://doi.org/10.1007/978-1-4471-2395-8_3
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DOI: https://doi.org/10.1007/978-1-4471-2395-8_3
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