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Chronic progressive multiple sclerosis

MedGen UID:
140733
•Concept ID:
C0393665
•
Disease or Syndrome
Synonyms: Chronic Progressive Multiple Sclerosis; Multiple Sclerosis, Chronic Progressive
SNOMED CT: Progressive multiple sclerosis (816984002); Multiple sclerosis (MS) progressive (816984002); Chronic progressive multiple sclerosis (816984002)
 
Monarch Initiative: MONDO:0005284

Definition

A form of multiple sclerosis characterized by a progressive deterioration in neurologic function which is in contrast to the more typical relapsing remitting form. If the clinical course is free of distinct remissions, it is referred to as primary progressive multiple sclerosis. When the progressive decline is punctuated by acute exacerbations, it is referred to as progressive relapsing multiple sclerosis. The term secondary progressive multiple sclerosis is used when relapsing remitting multiple sclerosis evolves into the chronic progressive form. (From Ann Neurol 1994;36 Suppl:S73-S79; Adams et al., Principles of Neurology, 6th ed, pp903-914) [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic progressive multiple sclerosis

Professional guidelines

PubMed

Inclusion criteria used in trials of people with progressive multiple sclerosis.
Dubuisson N, Marta M, Gnanapavan S, Turner B, Baker D, Thomson A, Schmierer K, Giovannoni G
Mult Scler 2020 Mar;26(3):279-283. Epub 2018 Oct 15 doi: 10.1177/1352458518803769. PMID: 30318994
Evaluation of Study and Patient Characteristics of Clinical Studies in Primary Progressive Multiple Sclerosis: A Systematic Review.
Ziemssen T, Rauer S, Stadelmann C, Henze T, Koehler J, Penner IK, Lang M, Poehlau D, Baier-Ebert M, Schieb H, Meuth S
PLoS One 2015;10(9):e0138243. Epub 2015 Sep 22 doi: 10.1371/journal.pone.0138243. PMID: 26393519Free PMC Article
The treatment of multiple sclerosis: current and future.
Polman CH, Hartung HP
Curr Opin Neurol 1995 Jun;8(3):200-9. doi: 10.1097/00019052-199506000-00008. PMID: 7551119

Curated

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

Recent clinical studies

Etiology

The effect of ibudilast on thalamic volume in progressive multiple sclerosis.
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
The window of opportunity for treatment of progressive multiple sclerosis.
Sorensen PS, Fox RJ, Comi G
Curr Opin Neurol 2020 Jun;33(3):262-270. doi: 10.1097/WCO.0000000000000811. PMID: 32251026
Pharmacological Approaches to the Management of Secondary Progressive Multiple Sclerosis.
Nandoskar A, Raffel J, Scalfari AS, Friede T, Nicholas RS
Drugs 2017 May;77(8):885-910. doi: 10.1007/s40265-017-0726-0. PMID: 28429241
Challenge of progressive multiple sclerosis therapy.
Thompson AJ
Curr Opin Neurol 2017 Jun;30(3):237-240. doi: 10.1097/WCO.0000000000000453. PMID: 28338498
The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review.
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923

Diagnosis

Progressive Multiple Sclerosis.
Amezcua L
Continuum (Minneap Minn) 2022 Aug 1;28(4):1083-1103. doi: 10.1212/CON.0000000000001157. PMID: 35938658
Secondary Progressive Multiple Sclerosis: New Insights.
Cree BAC, Arnold DL, Chataway J, Chitnis T, Fox RJ, Pozo Ramajo A, Murphy N, Lassmann H
Neurology 2021 Aug 24;97(8):378-388. Epub 2021 Jun 4 doi: 10.1212/WNL.0000000000012323. PMID: 34088878Free PMC Article
Progressive Multiple Sclerosis.
Ontaneda D
Continuum (Minneap Minn) 2019 Jun;25(3):736-752. doi: 10.1212/CON.0000000000000727. PMID: 31162314
The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review.
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923
Primary-progressive multiple sclerosis.
Miller DH, Leary SM
Lancet Neurol 2007 Oct;6(10):903-12. doi: 10.1016/S1474-4422(07)70243-0. PMID: 17884680

Therapy

Toward Precision Phenotyping of Multiple Sclerosis.
Pitt D, Lo CH, Gauthier SA, Hickman RA, Longbrake E, Airas LM, Mao-Draayer Y, Riley C, De Jager PL, Wesley S, Boster A, Topalli I, Bagnato F, Mansoor M, Stuve O, Kister I, Pelletier D, Stathopoulos P, Dutta R, Lincoln MR
Neurol Neuroimmunol Neuroinflamm 2022 Nov;9(6) Epub 2022 Aug 30 doi: 10.1212/NXI.0000000000200025. PMID: 36041861Free PMC Article
Safety of Ocrelizumab in Patients With Relapsing and Primary Progressive Multiple Sclerosis.
Hauser SL, Kappos L, Montalban X, Craveiro L, Chognot C, Hughes R, Koendgen H, Pasquarelli N, Pradhan A, Prajapati K, Wolinsky JS
Neurology 2021 Oct 19;97(16):e1546-e1559. Epub 2021 Sep 2 doi: 10.1212/WNL.0000000000012700. PMID: 34475123Free PMC Article
Beneficial effects of autologous mesenchymal stem cell transplantation in active progressive multiple sclerosis.
Petrou P, Kassis I, Levin N, Paul F, Backner Y, Benoliel T, Oertel FC, Scheel M, Hallimi M, Yaghmour N, Hur TB, Ginzberg A, Levy Y, Abramsky O, Karussis D
Brain 2020 Dec 1;143(12):3574-3588. doi: 10.1093/brain/awaa333. PMID: 33253391
Ocrelizumab versus Placebo in Primary Progressive Multiple Sclerosis.
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
Role of the innate and adaptive immune responses in the course of multiple sclerosis.
Hemmer B, Kerschensteiner M, Korn T
Lancet Neurol 2015 Apr;14(4):406-19. doi: 10.1016/S1474-4422(14)70305-9. PMID: 25792099

Prognosis

Secondary Progressive Multiple Sclerosis: New Insights.
Cree BAC, Arnold DL, Chataway J, Chitnis T, Fox RJ, Pozo Ramajo A, Murphy N, Lassmann H
Neurology 2021 Aug 24;97(8):378-388. Epub 2021 Jun 4 doi: 10.1212/WNL.0000000000012323. PMID: 34088878Free PMC Article
Beneficial effects of autologous mesenchymal stem cell transplantation in active progressive multiple sclerosis.
Petrou P, Kassis I, Levin N, Paul F, Backner Y, Benoliel T, Oertel FC, Scheel M, Hallimi M, Yaghmour N, Hur TB, Ginzberg A, Levy Y, Abramsky O, Karussis D
Brain 2020 Dec 1;143(12):3574-3588. doi: 10.1093/brain/awaa333. PMID: 33253391
Role of the innate and adaptive immune responses in the course of multiple sclerosis.
Hemmer B, Kerschensteiner M, Korn T
Lancet Neurol 2015 Apr;14(4):406-19. doi: 10.1016/S1474-4422(14)70305-9. PMID: 25792099
The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review.
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923
Progressive multiple sclerosis.
Bradl M, Lassmann H
Semin Immunopathol 2009 Nov;31(4):455-65. doi: 10.1007/s00281-009-0182-3. PMID: 19730864

Clinical prediction guides

Serum glial fibrillary acidic protein and disability progression in progressive multiple sclerosis.
Abdelhak A, Antweiler K, Kowarik MC, Senel M, Havla J, Zettl UK, Kleiter I, Skripuletz T, Haarmann A, Stahmann A, Huss A, Gingele S, Krumbholz M, Benkert P, Kuhle J, Friede T, Ludolph AC, Ziemann U, Kümpfel T, Tumani H
Ann Clin Transl Neurol 2024 Feb;11(2):477-485. Epub 2023 Dec 19 doi: 10.1002/acn3.51969. PMID: 38111972Free PMC Article
Cognitive function in primary and secondary progressive multiple sclerosis: A multiparametric magnetic resonance imaging study.
Mistri D, Cacciaguerra L, Valsasina P, Pagani E, Filippi M, Rocca MA
Eur J Neurol 2023 Sep;30(9):2801-2810. Epub 2023 Jun 8 doi: 10.1111/ene.15900. PMID: 37246467
Predicting disability worsening in relapsing and progressive multiple sclerosis.
Simmons SB, Schippling S, Giovannoni G, Ontaneda D
Curr Opin Neurol 2021 Jun 1;34(3):312-321. doi: 10.1097/WCO.0000000000000928. PMID: 33709974
The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review.
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923
Primary progressive multiple sclerosis: progress and challenges.
Rice CM, Cottrell D, Wilkins A, Scolding NJ
J Neurol Neurosurg Psychiatry 2013 Oct;84(10):1100-6. Epub 2013 Feb 16 doi: 10.1136/jnnp-2012-304140. PMID: 23418213

Recent systematic reviews

Optical coherence tomography (OCT) measurements and disability in multiple sclerosis (MS): A systematic review and meta-analysis.
Mirmosayyeb O, Yazdan Panah M, Mokary Y, Ghaffary EM, Ghoshouni H, Zivadinov R, Weinstock-Guttman B, Jakimovski D
J Neurol Sci 2023 Nov 15;454:120847. Epub 2023 Oct 30 doi: 10.1016/j.jns.2023.120847. PMID: 37924591
Real-world evaluation of ocrelizumab in multiple sclerosis: A systematic review.
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
Early diagnosis of secondary progressive multiple sclerosis: focus on fluid and neurophysiological biomarkers.
Ferrazzano G, Crisafulli SG, Baione V, Tartaglia M, Cortese A, Frontoni M, Altieri M, Pauri F, Millefiorini E, Conte A
J Neurol 2021 Oct;268(10):3626-3645. Epub 2020 Jun 5 doi: 10.1007/s00415-020-09964-4. PMID: 32504180
Neurofilaments in progressive multiple sclerosis: a systematic review.
Williams T, Zetterberg H, Chataway J
J Neurol 2021 Sep;268(9):3212-3222. Epub 2020 May 23 doi: 10.1007/s00415-020-09917-x. PMID: 32447549Free PMC Article
Physiotherapy Rehabilitation for People With Progressive Multiple Sclerosis: A Systematic Review.
Campbell E, Coulter EH, Mattison PG, Miller L, McFadyen A, Paul L
Arch Phys Med Rehabil 2016 Jan;97(1):141-51.e3. Epub 2015 Aug 14 doi: 10.1016/j.apmr.2015.07.022. PMID: 26281954

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Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
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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

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • MeSH
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