Natural history of primary progressive multiple sclerosis

Abstract

The relationship of primary progressive multiple sclerosis (PPMS) to relapsing -remitting MS (RRMS) and secondary progressive MS (SPMS) remains unclear. Natural history data from a population-based cohort of patients with PPMS followed for approximately 25 years demonstrate remarkable similarities in the progressive phases of PPMS and SPMS. Immunogenetic and magnetic resonance imaging studies in large numbers of patients also fail to differentiate between the two MS categories. PPMS thus resembles SPMS without the relapses, although the two forms do differ with respect to sex ratio. A n unfavourable outcome in PPMS is predicted by rapid early progression of disability and involvement of three or more systems. Natural history studies provide information on likely long-term outcomes and can be used in the design and interpretation of clinical trials in PPMS. The evidence that PPMS is distinct remains weak.

Keywords

natural history primary progressive multiple sclerosis

Get full access to this article

View all access options for this article.

References

Rice GPA

Kremenchutzky M

Cottrell DA

Baskerville J

Ebers GC.

Observations from the natural history cohort of London, Ontario. In

Filippi M

Comi G

eds. Topics in neuroscience: primary progressive multiple sclerosis. Milano: Springer Verlag Italia, 2002.

Richards RG

Sampson FC

Beard SM

Tappenden P.

A review of the natural history and epidemiology of multiple sclerosis: implications for resource allocation and health economic models. Health Technol Assess2002; 6: 1-73.

Weinshenker BG

Bass B

Rice GPA

Noseworthy J

Carriere W

Baskerville J

et al.

The natural history of multiple sclerosis: a geographically based study. 1. Clinical course and disability. Brain1989; 112: 133-146.

Thompson AJ

Polman CH

Miller DH

McDonald WI

Brochet B

Filippi M

et al.

Primary progressive multiple sclerosis (Review). Brain1997; 120: 1085-1096.

Andersson PB

Waubant E

Gee L

Goodkin DE.

Multiple sclerosis that is progressive from the time of onset. Clinical characteristics and progression of disability. Arch Neurol1999; 56: 1138-1142.

Ebers GC.

Natural history of multiple sclerosis. J Neurol Neurosurg Psychiatry2001; 71: 16-19.

Cottrell DA

Kremenchutzky M

Rice GPA

Hader W

Baskerville J

Ebers GC.

The natural history of multiple sclerosis: a geographically based study. 5. The clinical features and natural history of primary progressive multiple sclerosis. Brain1999; 122: 625-639.

Cottrell DA

Kremenchutzky M

Rice GPA

Hader W

Baskerville J

Ebers GC.

The natural history of multiple sclerosis: a geographically based study. 6. Applications to planning and interpretation of clinical therapeutic trials in primary progressive multiple sclerosis. Brain1999; 122: 641-647.

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.

10.

Noseworthy JH

Paty DW

Wonacott T

Feasby T

Ebers GC.

Multiple sclerosis after age 50. Neurology1983; 33: 1537-1544.

11.

Losseff NA

Kingsley DP

McDonald WI

Miller DH

Thompson AJ.

Clinical and magnetic resonance imaging predictors of disability in primary and secondary progressive multiple sclerosis. Mult Scler1996; 1: 218-222.

12.

Kremenchutzky M

Lee D

Rice GPA

Ebers GC.

Diagnostic brain MRI findings in primary progressive multiple sclerosis. Mult Scler2000; 6: 81-85.

13.

Ebers GC

Koopman WJ

Hader W

Sadnovick AD

Kremenchutzky M

Mandalfino P

et al.

The natural history of multiple sclerosis: a geographically based study. 8. Familial multiple sclerosis. Brain2000; 123: 641-649.

14.

Weinshenker BG

Santrach P

Bissonet AS

et al.

Major histocompatibility complex class II alleles and the course and outcome of MS: a population-based study. Neurology1998; 51: 742-747.

15.

Masterman T

Ligers A

Olsson T

Andersson M

Olerup O

Hillert J.

HLA-DR15 is associated with lower age at onset in multiple sclerosis. Ann Neurol2000; 48: 211-219.

16.

Masterman T

Hillert J.

HLA-DR15 and age of onset in multiple sclerosis. Eur J Neurol2002; 9: 177-185.

17.

Weinshenker BG

Rice GPA

Noseworthy JH

Carrier W

Baskerville J

Ebers GC.

The natural history of multiple sclerosis: a geographically based study. 4. Applications of planning and interpretation of clinical therapeutic trials. Brain1991; 114: 1057-1067.

18.

Ebers GC.

The natural history of multiple sclerosis. Neurol Sci2000; 21: S815-S817.