Primary progressive multiple sclerosis – demographic and clinical data from selected voivodeships Review article
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Published:
Jun 30, 2017
Keywords:
multiple sclerosis, clinical course, primary progressive form, epidemiology
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Abstract
Progressive multiple sclerosis, particularly in primary progressive form, is nowadays an object of intensive research and livid discussion. Distinct differences in symptoms, progression, and lesions found in MRI observed in primary progressive form cases lead to speculations that it may in fact be a different demyelinating disease altogether. Primary progressive form is characterized by a gradual increase in spinal cord symptoms with little fluctuation, but without evident relapses. This form of multiple sclerosis is still poorly researched, but it is estimated that primary progressive form cases constitute 10–20% of all multiple sclerosis patients. In the article, based on data from Registry of Multiple Sclerosis Patients, we presented polish observations concerning progression of this form of multiple sclerosis and its differences in comparison to the relapsing- remitting form. We came to the conclusion that the PPMS form is present in around 10% of Polish multiple sclerosis patients, the first symptoms appear around the age of 40 equally often for males and females, and diagnosis of primary progressive form takes more than twice as much time as diagnosis of relapsing-remitting form.
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References
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2. Kingwell E., Marriott J.J., Jetté N. et al.: Incidence and prevalence of multiple sclerosis in Europe: a systematic review. BMC Neurol. 2013; 13: 128.
3. Brola W., Sobolewski P., Flaga S. et al.: Increasing prevalence and incidence of multiple sclerosis in Poland. Neurol. Neurochir. Pol. 2017; 51: 82-85.
4. Potemkowski A.: Stwardnienie rozsiane w świecie i w Polsce – ocena epidemiologiczna. Aktualn. Neurol. 2009; 9: 91-97.
5. Lassmann H.: Brain damage when multiple sclerosis is diagnosed clinically. Lancet 2003; 361: 1317-1318.
6. Lublin F.D., Reingold S.C., Cohen J.A. et al.: Defining the clinical course of multiple sclerosis: the 2013 revisions. Neurology 2014; 83: 278-286.
7. Thompson A.J., Polman C.H., Miller D.H. et al.: Primary progressive multiple sclerosis. Brain 1997; 120: 1085-1096.
8. Miller D.H., Leary S.M.: Primary-progressive multiple sclerosis. Lancet Neurol. 2007; 6: 903-912.
9. Antel J., Antel S., Caramanos Z. et al.: Primary progressive multiple sclerosis: part of the MS disease spectrum or separate disease entity? Acta Neuropathol. 2012; 123: 627-638.
10. Cottrell D.A., Kremenchutzky M., Rice G.P.A. et al.: 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: 625-639.
11. Jacobs L.D., Wende K.E., Brownscheidle C.M. et al.: A profile of multiple sclerosis: the New York State Multiple Sclerosis Consortium. Mult. Scler. 1999; 5: 369-376.
12. Confavreux C., Vukusic S.: Natural history of multiple sclerosis: a unifying concept. Brain 2006; 129: 606-616.
13. Verjans E., Theys P., Delmotte P. et al.: Clinical parameters and intrathecal IgG synthesis as prognostic features in multiple sclerosis. Part I. J. Neurol. 1983; 229: 155-165.
14. Minderhoud J.M., van der Hoeven J.H., Prange A.J.: Course and prognosis of chronic progressive multiple sclerosis. Results of an epidemiological study. Acta Neurol. Scand. 1988; 78: 10-15.
15. Kantarci O.H., Weinshenker B.G.: Natural history of multiple sclerosis. Neurol. Clin. 2005; 23: 17-38.
16. Brola W., Sobolewski P., Flaga S. et al.: Pierwotnie postępująca postać stwardnienia rozsianego w populacji polskich pacjentów. Aktualn. Neurol. 2017; 17(1): 5-14.
17. Shepherd D.I.: Clinical features of multiple sclerosis in north-east Scotland. Acta Neurol. Scand. 1979; 60: 218-230.
18. Confavreux C., Aimard G., Devic M.: Course and prognosis of multiple sclerosis assessed by the computerized data processing of 349 patients. Brain 1980; 103: 281-300.
19. Weinshenker B.G., Bass B., Rice G.P. et al.: The natural history of multiple sclerosis: a geographically based study. I. Clinical course and disability. Brain 1989; 112: 133-146.
20. Andersson P.B., Waubant E., Gee L. et al.: Multiple sclerosis that is progressive from the time of onset: clinical characteristics and progression of disability. Arch Neurol. 1999; 56: 1138-1142.
21. McDonnell G.V., Hawkins S.A.: Clinical study of primary progressive multiple sclerosis in Northern Ireland, UK. J. Neurol. Neurosurg. Psychiatry 1998; 64: 451-454.
22. Tremlett H., Paty D., Devonshire V.: The natural history of primary progressive MS in British Columbia, Canada. Neurology 2005; 65: 1919-1923.
23. Kremenchutzky M., Rice G.P.A., Baskerville J. et al.: The natural history of multiple sclerosis: a geographically based study 9: observations on the progressive phase of the disease. Brain 2006; 129: 584-594.
24. Bashir K., Whitaker J.N.: Clinical and laboratory features of primary progressive and secondary progressive MS. Neurology 1999; 53: 765-771.
25. Ebers G.C.: Disease evolution in multiple sclerosis. J. Neurol. 2006; 253(supl. 6): vi3-vi8.
26. Montalban X.: The importance of long-term data in multiple sclerosis. J. Neurol. 2006; 253(supl. 6): 9-15.
27. Sastre-Garriga J., Ingle G.T., Rovaris M. et al.: Long-term clinical outcome of primary progressive MS: predictive value of clinical and MRI data. Neurology 2005; 65: 633-635.
28. Tremlett H., Paty D., Devonshire V.: Disability progression in multiple sclerosis is slower than previously reported. Neurology 2006; 66: 172-177.
29. Vukusic S., Confavreux C.: Prognostic factors for progression of disability in the secondary progressive phase of multiple sclerosis. J. Neurol. Sci. 2003; 206: 135-137.
30. Wolinsky J.S., Narayana P.A., O’Connor P. et al.; PROMiSe Trial Study Group: Glatiramer acetate in primary progressive multiple sclerosis: results of a multinational, multicenter, double-blind, placebo-controlled trial. Ann. Neurol. 2007; 61: 14-24.
31. Noseworthy J., Paty D., Wonnacott T. et al.: Multiple sclerosis after age 50. Neurology 1983; 33: 1537-1544.
32. Brola W., Sobolewski P., Flaga S. et al.: Prevalence and incidence of multiple sclerosis in central Poland, 2010–2014. BMC Neurol. 2016; 16: 134.