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Research article
First published May 2004

Overview of primary progressive multiple sclerosis (PPMS): similarities and differences from other forms of MS, diagnostic criteria, pros and cons of progressive diagnosis

Alan ThompsonView all authors and affiliations
Volume 10, Issue 3_suppl
https://doi.org/10.1191/1352458504ms1024oa

Abstract

Patients with the primary progressive form of multiple sclerosis (PPMS) have a unique clinical course and demonstrate additional demographic and imaging features, which separate them from the relapsing/remitting form of the condition. Whether these features indicate a fundamental difference in the underlying patho genesis of the condition or simply reflect opposite ends of a clinical spectrum is unclear. What is clear, however, is that this form of MS provides a valuable model of progression, which has the potential to explain this most disabling component of the disease process. The lack of the hallmark relapses and remissions in PPMS poses diagnostic difficulties, some of which have been addressed by recently published diagnostic criteria. Following diagnosis, the need for information, specific to this form of MS, must be recognized and addressed.

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References

McDonnell GV, Hawkins SA.Primary progressive multiple sclerosis: increasing clarity but many unanswered questions. J Neurol Sci2002; 199: 1-15.
Google Scholar
Wolinsky JS, the PROMiSe Study Group. The diagnosis of primary progressive multiple sclerosis. J Neurol Sci2003; 206: 145-152.
Google Scholar
McAlpine D.Multiple sclerosis: a reappraisal. In McAlpine D, Lumsden CE, Acheson ED eds. Multiple sclerosis: a reappraisal, second edition. Edinburgh, Scotland: Churchill Livingstone, 1972: 83-301.
Google Scholar
Lublin FD, Reingold SC.Defining the clinical course of multiple sclerosis: results of an international survey. Neurology1996; 46: 907-911.
Google Scholar
Kremenchutzky M, Cottrell D, Rice G, Hader W, Baskerville J, Koopman W, et al.The natural history of multiple sclerosis: a geographically based study. 7. Progressive-relapsing and relapsing -progressive multiple sclerosis: a re-evaluation. Brain1999; 122: 1941-1949.
Google Scholar
Thompson AJ, Polman CH, Miller DH, McDonald WI, Brochet B, Filippi M, et al.Primary progressive multiple sclerosis. Brain1997; 120: 1085-1096.
Google Scholar
McDonnell GV, Hawkins SA.Clinical study of primary progressive multiple sclerosis in Northern Ireland, UK. J Neurol Neurosurg Psychiatry1998; 64: 451-454.
Google Scholar
Stevenson VL, Miller DH, Rovaris M, Barkhof F, Brochet B, Dousset V, et al.Primary and transitional progressive MS: a clinical and MRI cross-sectional study. Neurology1999; 52: 839-845.
Google Scholar
Camp SJ, Stevenson VL, Thompson AJ, Miller DH, Borras C, Auriacombe S, et al.Cognitive function in primary progressive and transitional progressive multiple sclerosis. A controlled study with MRI correlates. Brain1999; 122: 1341-1348.
Google Scholar
Minderhoud JM, van der Hoeven JH, Prange AJ.Course and prognosis of chronic progressive multiple sclerosis. Results of an epidemiological study. Acta Neurol Scand1988; 78: 10-15.
Google Scholar
Confavreux C, Vukusic S, Moreau T, Adeleine P.Relapses and progression of disability in multiple sclerosis. N Engl J Med2000; 343: 1430-1438.
Google Scholar
Cottrell DA, Kremenchutzky M, Rice GPA, Hader W, Koopman WJ, Baskerville J, et al.The natural history of multiple sclerosis: a geographicallybased study. 5: The clinical features and natural history of primary progressive multiple sclerosis. Brain1999; 122: 625-639.
Google Scholar
Thompson AJ, Kermode AG, Wicks D, MacManus DG, Kendall BE, Kingsley DP, et al.Major differences in the dynamics of primary and secondary progressive multiple sclerosis. Ann Neurol1991; 29: 53-62.
Google Scholar
Kidd D, Thorpe JW, Kendall BE, Barker GJ, Miller DH, McDonald WI, et al.MRI dynamics of brain and spinal cord in progressive multiple sclerosis. J Neurol Neurosurg Psychiatry1996; 60: 15-19.
Google Scholar
Stevenson VL, Leary SM, Losseff NA, Parker GJM, Barker GJ, Husmani Y, et al.Spinal cord atrophy and disability in MS. A longitudinal study. Neurology1998; 51: 234-238.
Google Scholar
Lycklama a Nijeholt GJ, Barkhof F, Scheltens P, Castelijns JA, Ader H, van Waesberghe JH, et al.MR of the spinal cord in multiple sclerosis: relation to clinical subtype and disability. Am J Neuroradiol1997; 18: 1041-1048.
Google Scholar
Rovaris M, Bozzali M, Santuccio G, Ghezzi A, Caputo D, Montanari E, et al.In vivo assessment of the brain and cervical cord pathology of patients with primary progressive multiple sclerosis. Brain2001; 124: 2540-2549.
Google Scholar
Stevenson VL, Miller DH, Leary SM, Rovaris M, Barkhof F, Brochet E, et al.One year follow up study of primary and transitional progressive multiple sclerosis. J Neurol Neurosurg Psychiatry2000; 68: 713-718.
Google Scholar
Ingle GT, Stevenson VL, Miller DH, Leary SM, Rovaris M, Barkhof F, et al.Two-year follow-up study of primary and transitional progressive multiple sclerosis. Mult Scler2002; 8: 108-114.
Google Scholar
Ingle GT, Stevenson VL, Miller DH, Thompson AJ.Primary progressive multiple sclerosis: a five year clinical and MR study. Brain2003; 126: 2528-2536.
Google Scholar
Sastre-Garriga J, Ingle GT, Ramio L, Miller DH, Thompson AJ.inflammation in early primary progressive multiple sclerosis. J Neurol Neurosurg Psychiatry2003; 74: 1457-1457.
Google Scholar
Filippi M, Rocca MA, Comi G.The use of quantitative magnetic-resonance-based techniques to monitor the evolution of multiple sclerosis. Lancet Neurol2003; 2: 337-346.
Google Scholar
Revesz T, Kidd D, Thompson AJ, Barnard RO, McDonald WI.A comparison of the pathology of primary and secondary progressive multiple sclerosis. Brain1994; 117: 759-765.
Google Scholar
Brück W, Lucchinetti C, Lassmann H.The pathology of primary progressive multiple sclerosis. Mult Scler2002; 8: 93-97.
Google Scholar
Neuhaus O, Hartung H-P.In search of a disease marker: the cytokine profile of primary progressive multiple sclerosis. Mult Scler2001; 7: 143-144.
Google Scholar
Genain CP, Fuhrmann A, Menge T, von Budingen H-C, Bae S, Swerdlin A, et al.Autoantibody reactivity to myelin/oligodendrocyte glycoprotein (MOG) correlates with progressive forms of multiple sclerosis. American Neurological Association 127th Annual Meeting On-site programme, October 13- 16, 2002. p. 38-38.
Google Scholar
Thompson AJ, Montalban X, Barkhof F, Brochet B, Filippi M, Miller DH, et al.Diagnostic criteria for primary progressive multiple sclerosis: a position paper. Ann Neurol2000; 47: 831-835.
Google Scholar
Kremenchutzky M, Lee D, Rice GPA, Ebers GC.Diagnostic brain MRI findings in primary progressive multiple sclerosis. Mult Scler.2000; 6: 81-85.
Google Scholar
Khan O, Caon C, Ching W, Sonenvirth E, Tselis A, Zvartau-Hind M.Clinical profile and application of diagnostic criteria in primary progressive multiple sclerosis. Mult Scler2002; 8: S33-S33.
Google Scholar
McDonald WI, Compston A, Edan G, Goodkin D, Hartung H-P, Lublin FD, et al.Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the Diagnosis of Multiple Sclerosis (Special Report). Ann Neurol2001; 50: 121-127.
Google Scholar
Leary SM, Miller DH, Stevenson VL, Brex PA, Chard DT, Thompson AJ.Interferon b-1a in primary progressive MS. An exploratory, randomized, controlled trial. Neurology2003; 60: 44-51.
Google Scholar
Montalban X, Brieva L, Tintore M, Borras C, Rio J, Nos C, et al.Single center, DBPC, randomized trial of interferon b-1B in primary progressive and transitional progressive multiple sclerosis: an exploratory phase II study. ACTRIMS/ECTRIMS 2002 Final Programme: 57. Multiple Sclerosis2002; 8: S63-S63.
Google Scholar
Wolinsky JS, Narayana PA, The PROMiSe trial Study Group. Characteristics at entry into the glatiramer acetate study of primary progressive multiple sclerosis: the PROMiSe Trial. J Neurol2001; 248: 134-134. Abstract.
Google Scholar
Kalkers NF, Barkhof F, Bergers E, van Schijndel R, Polman CH.The effect of the neuroprotective agent riluzole on MRI parameters in primary progressive multiple sclerosis: a pilot study. Mult Scler2002; 6: 532-533.
Google Scholar

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Published In

Volume 10, Issue 3_suppl
Pages: S2 - S7
Article first published: May 2004
Issue published: May 2004

Keywords

  1. diagnostic criteria
  2. model of progression
  3. primary progressive MS

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History

Published online: May 1, 2004
Issue published: May 2004
PubMed: 15218803

Authors

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Alan Thompson
Department of Clinical Neurology, Institute of Neurology, Queen Square, London WC1N 3BG, UK, [email protected]
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