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Primary progressive multiple sclerosis

MedGen UID:
155968
Concept ID:
C0751964
Disease or Syndrome
Synonyms: Multiple Sclerosis, Primary Progressive; Primary Progressive Multiple Sclerosis
SNOMED CT: Primary progressive multiple sclerosis (428700003); Multiple sclerosis (MS) primary progressive (428700003)
 
Monarch Initiative: MONDO:0000451

Definition

A multiple sclerosis that is characterized by steady worsening of neurologic functioning, without any distinct relapses or periods of remission. The rate of progression may vary over time, with occasional plateaus or temporary improvements, but the progression is continuous. [from MONDO]

Professional guidelines

PubMed

Carlson AK, Fox RJ
Neurol Clin 2024 Feb;42(1):39-54. Epub 2023 Aug 21 doi: 10.1016/j.ncl.2023.07.002. PMID: 37980122
Correale J, Gaitán MI, Ysrraelit MC, Fiol MP
Brain 2017 Mar 1;140(3):527-546. doi: 10.1093/brain/aww258. PMID: 27794524
Katz Sand I
Curr Opin Neurol 2015 Jun;28(3):193-205. doi: 10.1097/WCO.0000000000000206. PMID: 25887774

Curated

UK NICE Guideline NG220, Multiple sclerosis in adults: management, 2022

Recent clinical studies

Etiology

Montalvo M, Flanagan EP
Handb Clin Neurol 2024;200:193-201. doi: 10.1016/B978-0-12-823912-4.00017-7. PMID: 38494277
Reich DS, Arnold DL, Vermersch P, Bar-Or A, Fox RJ, Matta A, Turner T, Wallström E, Zhang X, Mareš M, Khabirov FA, Traboulsee A; Tolebrutinib Phase 2b Study Group
Lancet Neurol 2021 Sep;20(9):729-738. doi: 10.1016/S1474-4422(21)00237-4. PMID: 34418400Free PMC Article
Montalban X, Hauser SL, Kappos L, Arnold DL, Bar-Or A, Comi G, de Seze J, Giovannoni G, Hartung HP, Hemmer B, Lublin F, Rammohan KW, Selmaj K, Traboulsee A, Sauter A, Masterman D, Fontoura P, Belachew S, Garren H, Mairon N, Chin P, Wolinsky JS; ORATORIO Clinical Investigators
N Engl J Med 2017 Jan 19;376(3):209-220. Epub 2016 Dec 21 doi: 10.1056/NEJMoa1606468. PMID: 28002688
Lublin F, Miller DH, Freedman MS, Cree BAC, Wolinsky JS, Weiner H, Lubetzki C, Hartung HP, Montalban X, Uitdehaag BMJ, Merschhemke M, Li B, Putzki N, Liu FC, Häring DA, Kappos L; INFORMS study investigators
Lancet 2016 Mar 12;387(10023):1075-1084. Epub 2016 Jan 28 doi: 10.1016/S0140-6736(15)01314-8. PMID: 26827074
Katz Sand I
Curr Opin Neurol 2015 Jun;28(3):193-205. doi: 10.1097/WCO.0000000000000206. PMID: 25887774

Diagnosis

Carlson AK, Fox RJ
Neurol Clin 2024 Feb;42(1):39-54. Epub 2023 Aug 21 doi: 10.1016/j.ncl.2023.07.002. PMID: 37980122
Olek MJ
Ann Intern Med 2021 Jun;174(6):ITC81-ITC96. Epub 2021 Jun 8 doi: 10.7326/AITC202106150. PMID: 34097429
Kamińska J, Koper OM, Piechal K, Kemona H
Postepy Hig Med Dosw (Online) 2017 Jun 30;71(0):551-563. doi: 10.5604/01.3001.0010.3836. PMID: 28665284
Correale J, Gaitán MI, Ysrraelit MC, Fiol MP
Brain 2017 Mar 1;140(3):527-546. doi: 10.1093/brain/aww258. PMID: 27794524
Katz Sand I
Curr Opin Neurol 2015 Jun;28(3):193-205. doi: 10.1097/WCO.0000000000000206. PMID: 25887774

Therapy

Montalban X, Matthews PM, Simpson A, Petrie JL, Sammon C, Ramagopalan S, Disanto G, Kuhle J
Ann Clin Transl Neurol 2023 Mar;10(3):302-311. Epub 2023 Feb 2 doi: 10.1002/acn3.51732. PMID: 36728340Free PMC Article
Lamb YN
Drugs 2022 Feb;82(3):323-334. Epub 2022 Feb 22 doi: 10.1007/s40265-022-01672-9. PMID: 35192158Free PMC Article
Olek MJ
Ann Intern Med 2021 Jun;174(6):ITC81-ITC96. Epub 2021 Jun 8 doi: 10.7326/AITC202106150. PMID: 34097429
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article
Montalban X, Hauser SL, Kappos L, Arnold DL, Bar-Or A, Comi G, de Seze J, Giovannoni G, Hartung HP, Hemmer B, Lublin F, Rammohan KW, Selmaj K, Traboulsee A, Sauter A, Masterman D, Fontoura P, Belachew S, Garren H, Mairon N, Chin P, Wolinsky JS; ORATORIO Clinical Investigators
N Engl J Med 2017 Jan 19;376(3):209-220. Epub 2016 Dec 21 doi: 10.1056/NEJMoa1606468. PMID: 28002688

Prognosis

Montalvo M, Flanagan EP
Handb Clin Neurol 2024;200:193-201. doi: 10.1016/B978-0-12-823912-4.00017-7. PMID: 38494277
Bar-Or A, Thanei GA, Harp C, Bernasconi C, Bonati U, Cross AH, Fischer S, Gaetano L, Hauser SL, Hendricks R, Kappos L, Kuhle J, Leppert D, Model F, Sauter A, Koendgen H, Jia X, Herman AE
EBioMedicine 2023 Jul;93:104662. Epub 2023 Jun 22 doi: 10.1016/j.ebiom.2023.104662. PMID: 37354600Free PMC Article
Wolinsky JS
Mult Scler 2017 Oct;23(12):1636-1641. doi: 10.1177/1352458517729458. PMID: 29041867
Kamińska J, Koper OM, Piechal K, Kemona H
Postepy Hig Med Dosw (Online) 2017 Jun 30;71(0):551-563. doi: 10.5604/01.3001.0010.3836. PMID: 28665284
Hawker K, O'Connor P, Freedman MS, Calabresi PA, Antel J, Simon J, Hauser S, Waubant E, Vollmer T, Panitch H, Zhang J, Chin P, Smith CH; OLYMPUS trial group
Ann Neurol 2009 Oct;66(4):460-71. doi: 10.1002/ana.21867. PMID: 19847908

Clinical prediction guides

Nicholson S, Russo AW, Brewer K, Bien H, Tobyne SM, Eloyan A, Klawiter EC
Mult Scler 2023 Dec;29(14):1819-1830. Epub 2023 Nov 10 doi: 10.1177/13524585231204710. PMID: 37947294Free PMC Article
Chisari CG, Bianco A, Brescia Morra V, Calabrese M, Capone F, Cavalla P, Chiavazza C, Comi C, Danni M, Filippi M, Iaffaldano P, Lanzillo R, Lo Fermo S, Lucisano A, Lugaresi A, Lus G, Marfia GA, Marinelli F, Mirabella M, Moiola L, Perin C, Realmuto S, Toscano S, Trojano M, Vecchio D, Patti F; Italian MS Registry
Neurotherapeutics 2023 Oct;20(6):1696-1706. Epub 2023 Aug 23 doi: 10.1007/s13311-023-01415-y. PMID: 37610702Free PMC Article
Bar-Or A, Thanei GA, Harp C, Bernasconi C, Bonati U, Cross AH, Fischer S, Gaetano L, Hauser SL, Hendricks R, Kappos L, Kuhle J, Leppert D, Model F, Sauter A, Koendgen H, Jia X, Herman AE
EBioMedicine 2023 Jul;93:104662. Epub 2023 Jun 22 doi: 10.1016/j.ebiom.2023.104662. PMID: 37354600Free PMC Article
Montalban X, Hauser SL, Kappos L, Arnold DL, Bar-Or A, Comi G, de Seze J, Giovannoni G, Hartung HP, Hemmer B, Lublin F, Rammohan KW, Selmaj K, Traboulsee A, Sauter A, Masterman D, Fontoura P, Belachew S, Garren H, Mairon N, Chin P, Wolinsky JS; ORATORIO Clinical Investigators
N Engl J Med 2017 Jan 19;376(3):209-220. Epub 2016 Dec 21 doi: 10.1056/NEJMoa1606468. PMID: 28002688
Montalban X
Curr Opin Neurol 2005 Jun;18(3):261-6. doi: 10.1097/01.wco.0000169742.37159.e9. PMID: 15891409

Recent systematic reviews

Montalban X, Matthews PM, Simpson A, Petrie JL, Sammon C, Ramagopalan S, Disanto G, Kuhle J
Ann Clin Transl Neurol 2023 Mar;10(3):302-311. Epub 2023 Feb 2 doi: 10.1002/acn3.51732. PMID: 36728340Free PMC Article
Lin M, Zhang J, Zhang Y, Luo J, Shi S
Cochrane Database Syst Rev 2022 May 18;5(5):CD013247. doi: 10.1002/14651858.CD013247.pub2. PMID: 35583174Free PMC Article
Kaegi C, Wuest B, Crowley C, Boyman O
Front Immunol 2021;12:788830. Epub 2022 Feb 2 doi: 10.3389/fimmu.2021.788830. PMID: 35185862Free PMC Article
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article
Cristiano E, Rojas JI, Abad P, Adoni T, Barahona J, Becker J, Carrá A, Flores J, Fruns M, Fernández Liguori N, Garcea O, García Bónito J, Giunta D, Gracia F, Hamuy F, Macias Islas MA, Navas C, Boschetti LO, Patrucco L, Sato DK, Correale J
J Neurol Sci 2018 Oct 15;393:4-13. Epub 2018 Jul 31 doi: 10.1016/j.jns.2018.07.024. PMID: 30096568

Supplemental Content

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    Clinical resources

    Practice guidelines

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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NICE, 2022
      UK NICE Guideline NG220, Multiple sclerosis in adults: management, 2022

    Consumer resources

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