The hidden scale: the true contribution of primary progressive multiple sclerosis to overall disability statistics
https://doi.org/10.14412/2074-2711-2026-2-79-84
Abstract
Primary progressive multiple sclerosis (PPMS) is the most severe phenotype of the disease, characterised by a steady progression of neurological deficit from the time of onset. This review presents a comprehensive analysis of the epidemiological, clinical, neuroimaging and socio-economic aspects of PPMS. It is shown that PPMS is associated with accelerated progression to disability thresholds (≥ 6.0 points on the Expanded Disability Status Scale – EDSS, on average within 5–9 years), early loss of working capacity and the creation of a significant burden for patients and their families, which is exacerbated by the need for long-term care. Key pathogenetic differences between PPMS and relapsing-remitting MS (RRMS), as well as current therapeutic strategies, are discussed. The need for early diagnosis, timely initiation of pathogenetic therapy and the development of a multidisciplinary care system to improve patient prognosis and quality of life is emphasised.
Keywords
multiple sclerosis,
primary progressive multiple sclerosis,
anti-B-cell therapy,
ocrelizumab,
disability
Supporting agencies: The investigation has not been sponsored
About the Authors
A. N. Boyko
N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia; Federal Center of Brain and Neurotechnologies, FMBA of Russia
Russian Federation
Competing Interests:
Russian Federation
Alexey Nikolaevich Boyko
Institute of Clinical Neurology; Department of Neuroimmunology
117997; 1, Ostrovityanovа St.; 1, Ostrovityanovа St., Build. 10; Moscow
Competing Interests:
There are no conflicts of interest. The authors are solely responsible for submitting the final version of the manuscript for publication. All the authors have participated in developing the concept of the article and in writing the manuscript. The final version of the manuscript has been approved by all the authors
A. D. Kukushkina
Federal Center of Brain and Neurotechnologies, FMBA of Russia; Zhadkevich Moscow City Clinical Hospital
Russian Federation
Competing Interests:
Russian Federation
Institute of Clinical Neurology; Department of Neuroimmunology
117997; 1, Ostrovityanovа St., Build. 10;; ; 121374; 14, Mozhaiskoe Shosse; Moscow
Competing Interests:
There are no conflicts of interest. The authors are solely responsible for submitting the final version of the manuscript for publication. All the authors have participated in developing the concept of the article and in writing the manuscript. The final version of the manuscript has been approved by all the authors
References
1. Montalban X, Hauser SL, Kappos L, et al. ORATORIO Clinical Investigators. Ocrelizumab versus Placebo in Primary Progressive Multiple Sclerosis. N Engl J Med. 2017 Jan 19;376(3):209-20. doi: 10.1056/NEJMoa1606468
2. Mahad DH, Trapp BD, Lassmann H. Pathological mechanisms in progressive multiple sclerosis. Lancet Neurol. 2015 Feb;14(2):183-93. doi: 10.1016/S1474-4422(14)70256-X
3. Salter A, Thomas NP, Tyry T, et al. A contemporary profile of primary progressive multiple sclerosis participants from the NARCOMS Registry. Mult Scler. 2018 Jun;24(7):951-62. doi: 10.1177/1352458517711274
4. Brola W, Sobolewski P, Zak M, et al. Profile of Polish patients with primary progressive multiple sclerosis. Mult Scler Relat Disord. 2019 Aug;33:33-8. doi: 10.1016/j.msard.2019.05.009
5. Engelhard J, Oleske DM, Schmitting S, et al. Multiple sclerosis by phenotype in Germany. Mult Scler Relat Disord. 2022 Jan;57:103326. doi: 10.1016/j.msard.2021.103326
6. Miller DH, Leary SM. Primary-progressive multiple sclerosis. Lancet Neurol. 2007 Oct;6(10):903-12. doi: 10.1016/S1474-4422(07)70243-0
7. Clinical management of multiple sclerosis around the world Key findings about the diagnosis and clinical management of MS Atlas of MS 3rd edition. 2021.
8. Boyko AN, Melnikov MV. Prevalence and Incidence of Multiple Sclerosis in Russian Federation: 30 Years of Studies. Brain Sci. 2020 May 18;10(5):305. doi: 10.3390/brainsci10050305
9. Boyko AN, Kukel TM, Lysenko MA, et al. Clinical epidemiology of multiple sclerosis in Moscow. Сlinical demographic characteristics in population of one region of Moscow. S.S. Korsakov Journal of Neurology and Psychiatry. 2014;114(2-2):10-5 (In Russ.).
10. Kotov SV, Iakushina TI, Lizhdvoi VYu. Clinical and epidemiological aspects of multiple sclerosis in the Moscow region. S.S. Korsakov Journal of Neurology and Psychiatry. 2012;112(3):60-2 (In Russ.).
11. Spirin NN, Kasatkin DS, Stepanov IO, et al. Registry-based comparison of multiple sclerosis epidemiology trend data in 1999 and 2019: the case of Yaroslavl. S.S. Korsakov Journal of Neurology and Psychiatry. 2020;120(7-2):48-53 (In Russ.). doi: 10.17116/jnevro202012007248
12. Maslova NN, Andreeva EA, Belyy YuA, et al. Clinical, epidemiological and neuro-ophthalmological features of patients with multiple sclerosis: study of Kaluga region. Annals of Clinical and Experimental Neurology. 2014;8(3):30-7 (In Russ.).
13. Maslova NN, Pysina AM. Epidemiology of disseminated sclerosis in Smolensk Region. Bulletin of Siberian Medicine. 2008;7(5-1):236-41 (In Russ.).
14. Smagina IV, Elchaninova EYu, Elchaninova SA. Multiple sclerosis in the Altai region of Russia: a prospective epidemiological study. S.S. Korsakov Journal of Neurology and Psychiatry. 2019;119(2-2):7-11 (In Russ.). doi: 10.17116/jnevro2019119227
15. Ivanova EV, Bakhtiyarova KZ, Zaplakhova OV, et al. Clinical and epidemiological study of multiple sclerosis in the city of Ufa. Practical Medicine. 2017;1(1-102):88-91 (In Russ.).
16. Zikhova AR, Berezgova LM, Tlapshokova LB, et al. Epidemiological characteristics of multiple sclerosis in the Kabardino-Balkaria Republic. S.S. Korsakov Journal of Neurology and Psychiatry. 2013;113(10-2):5-7 (In Russ.).
17. Zhelnin AV, Trushnikova TN. Comparative characteristics and analysis of the prevalence of multiple sclerosis in Perm Krai, Sverdlovsk, Tyumen, Nizhny Novgorod regions, the Chuvash Republic and the Republic of Bashkortostan. Perm Medical Journal. 2008;15(5):103-7 (In Russ.).
18. Yurchenko AN, Yurchenko YuN, Smagina IV. Epidemiology of multiple sclerosis in the Bryansk region. S.S. Korsakov Journal of Neurology and Psychiatry. 2015;115(8-2):91-2 (In Russ.).
19. Khabirov FA, Babicheva NN, Esin RG, et al. Clinical and sociodemographic characteristics of multiple sclerosis in the republic of Tatarstan. Practical Medicine. 2013;1(68):15-8 (In Russ.).
20. Smagina IV, Ignatova YuN, Yelchaninova SA, et al. Multiple sclerosis prevalence and risk factors among the Altai region population. Bulletin of Siberian Medicine. 2011;10(2):39-43 (In Russ.).
21. Kashlaev EYu, Zaitseva OI, Kosova EV, et al. Clinical and epidemiological characteristics of patients with multiple sclerosis in the Republic of Khakassia. Bulletin of the Khakass State University named after N.F. Katanov. 2014;(8):47-8 (In Russ.).
22. Boynova IV, Samarina DV, Katorova AV, et al. Clinical and epidemiological features of multiple sclerosis in the Russian Federation. Modern Problems of Science and Education. 2022;(5):139 (In Russ.).
23. Goncharova ZA. Clinical and epidemiological peculiarities of multiple sclerosis in Rostov region. Medical Herald of the South of Russia. 2010;(1):62-6 (In Russ.).
24. Gavrilenko AA, Evdokimova ZS, Vasikovskaia GA, et al. Epidemiology of multiple sclerosis in the Primosky Krai and Far East regions. S.S. Korsakov Journal of Neurology and Psychiatry. 2012;112(2-2):5-8 (In Russ.).
25. Khabirov FA, Khaybullin TI, Granatov EV, et al. Multiple sclerosis in the Republic of Tatarstan: epidemiological and clinical characteristics. Practical Medicine. 2020;18(5):50-7 (In Russ.). doi: 10.32000/2072-1757-2020-5-50-57
26. Rajabi M, Shafaeibajestan S, Asadpour S, et al. Primary Progressive Multiple Sclerosis: New Therapeutic Approaches. Neuropsychopharmacol Rep. 2025 Sep;45(3):e70039. doi: 10.1002/npr2.70039
27. Zhelnin AV. Epidemiological and clinical features of multiple sclerosis in the Perm region. Saratov Scientific Medical Journal. 2013;9(1):69-71 (In Russ.).
28. Taranu D, Balz LT, Holbrook J, et al. Cognitive impairment, mood, and fatigue in various multiple sclerosis subtypes: a one-year follow-up study. J Neurol. 2025 May 14;272(6):398. doi: 10.1007/s00415-025-13115-y
29. Brochet B, Ruet A. Cognitive Impairment in Multiple Sclerosis With Regards to Disease Duration and Clinical Phenotypes. Front Neurol. 2019 Mar 20;10:261. doi: 10.3389/fneur.2019.00261
30. Rice CM, Cottrell D, Wilkins A, et al. Primary progressive multiple sclerosis: progress and challenges. J Neurol Neurosurg Psychiatry. 2013 Oct;84(10):1100-6. doi: 10.1136/jnnp-2012-304140
31. World Health Organization. Global status report on neurology. World Health Organization; 2025. P. 1-285.
32. Kavaliunas A, Danylaite Karrenbauer V, Gyllensten H, et al. Cognitive function is a major determinant of income among multiple sclerosis patients in Sweden acting independently from physical disability. Mult Scler. 2019 Jan;25(1):104-12. doi: 10.1177/1352458517740212
33. Boiko A, Vorobeychik G, Paty D, et al. Early onset multiple sclerosis: a longitudinal study. Neurology. 2002 Oct 8;59(7):1006-10. doi: 10.1212/wnl.59.7.1006
34. Signori A, Gallo F, Bovis F, et al. Long-term impact of Interferon or Glatiramer acetate in multiple sclerosis : A systematic review and meta-analysis. Mult Scler Relat Disord. 2016;6:57-63.
35. Coret F, Perez-Miralles FC, Gascon F, et al. Onset of secondary progressive multiple sclerosis is not influenced by current relapsing multiple sclerosis therapies. Mult Scler J Exp Transl Clin. 2018 Jun 26;4(2):2055217318783347. doi: 10.1177/2055217318783347
36. Popova EV, Boyko AN, Barabanova MA, et al. Primary progressive multiple sclerosis: current issues of timely diagnosis. S.S. Korsakov Journal of Neurology and Psychiatry. 2017;117(10-2):35-40 (In Russ.). doi: 10.17116/jnevro201711710235-40
37. Tremlett H, Zhao Y, Devonshire V. Natural history comparisons of primary and secondary progressive multiple sclerosis reveals differences and similarities. J Neurol. 2009 Mar;256(3):374-81. doi: 10.1007/s00415-009-0039-7
38. Kobelt G, Thompson A, Berg J, et al. New insights into the burden and costs of multiple sclerosis in Europe. Mult Scler. 2017 Jul;23(8):1123-36. doi: 10.1177/1352458517694432
39. Qian Z, Li Y, Guan Z, et al. Global, regional, and national burden of multiple sclerosis from 1990 to 2019: Findings of global burden of disease study 2019. Front Public Health. 2023 Feb 17;11:1073278. doi: 10.3389/fpubh.2023.1073278
40. Kavaliunas A, Danylaite Karrenbauer V, Binzer S, et al. Systematic Review of the Socioeconomic Consequences in Patients With Multiple Sclerosis With Different Levels of Disability and Cognitive Function. Front Neurol. 2022 Jan 6;12:737211. doi: 10.3389/fneur.2021.737211
41. Kobelt G, Berg J, Lindgren P, et al. Costs and quality of life of patients with multiple sclerosis in Europe. J Neurol Neurosurg Psychiatry. 2006 Aug;77(8):918-26. doi: 10.1136/jnnp.2006.090365
42. Dubois B, Kobelt G, Berg J, et al. New insights into the burden and costs of multiple sclerosis in Europe: Results for Belgium. Mult Scler. 2017 Aug;23(2 Suppl):29-40. doi: 10.1177/1352458517708100
43. Berger T, Kobelt G, Berg J, et al. New insights into the burden and costs of multiple sclerosis in Europe: Results for Austria. Mult Scler. 2017 Aug;23(2 Suppl):17-28. doi: 10.1177/1352458517708099
44. Vlasov YaV, Churakov MV, Kurapov MA, et al. Primary-progressive multiple sclerosis in Russia: a medical-sociological study involving patients and neurologists. S.S. Korsakov Journal of Neurology and Psychiatry. 2018;118(8-2):40-6 (In Russ.). doi: 10.17116/jnevro201811808240
45. Karampampa K, Gustavsson A, Miltenburger C, et al. Treatment experience, burden and unmet needs (TRIBUNE) in MS study: results from Spain. Mult Scler. 2012 Jun;18(2 Suppl):35-9. doi: 10.1177/1352458512441566d
46. Smyrke N, Dunn N, Murley C, et al. Standardized mortality ratios in multiple sclerosis : Systematic review with meta-analysis. Acta Neurol Scand. 2022 Mar;145(3):360-70. doi: 10.1111/ane.13559
47. Lunde HMB, Assmus J, Myhr KM, et al. Survival and cause of death in multiple sclerosis: a 60-year longitudinal population study. J Neurol Neurosurg Psychiatry. 2017 Aug;88(8):621-5. doi: 10.1136/jnnp-2016-315238
48. Campbell CW, Jones EJ, Merrills J. Palliative and end-of-life care in advanced Parkinson's disease and multiple sclerosis. Clin Med (Lond). 2010 Jun;10(3):290-2. doi: 10.7861/clinmedicine.10-3-290
49. Wolinsky JS, Arnold DL, Brochet B, et al. Long-term follow-up from the ORATORIO trial of ocrelizumab for primary progressive multiple sclerosis: a post-hoc analysis from the ongoing open-label extension of the randomised, placebo-controlled, phase 3 trial. Lancet Neurol. 2020 Dec;19(12):998-1009. Erratum in: Lancet Neurol. 2021 Jan;20(1):e1. doi: 10.1016/S1474-4422(20)30437-3
50. Watson C, Thirumalai D, Barlev A, et al. Treatment Patterns and Unmet Need for Patients with Progressive Multiple Sclerosis in the United States: Survey Results from 2016 to 2021. Neurol Ther. 2023 Dec;12(6):1961-79. doi: 10.1007/s40120-023-00532-2
Review
For citations:
Boyko AN, Kukushkina AD. The hidden scale: the true contribution of primary progressive multiple sclerosis to overall disability statistics. Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2026;18(2):79-84. (In Russ.) https://doi.org/10.14412/2074-2711-2026-2-79-84
Views:
661
JATS XML
Email this article 