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From Neurology (Drs. Tremlett, Paty, and Devonshire) and Health Care and Epidemiology (Dr. Tremlett), Faculty of Medicine, University of British Columbia, Vancouver, Canada.
Address correspondence and reprint requests to Dr. Helen Tremlett, Faculty of Medicine (Neurology), Rm S159, 2211 Wesbrook Mall, University of British Columbia, Vancouver, BC V6T 2B5, Canada; e-mail: tremlett{at}interchange.ubc.ca
Background: Primary progressive multiple sclerosis (PPMS) has a distinct clinical phenotype and has historically been understudied with few longitudinal natural history studies spanning a reasonable time period. The authors examined patient characteristics, disease progression, and associated risk factors in the PP population of British Columbia, Canada.
Method: The authors report on the PP population from an upcoming publication of the natural history study of definite MS in British Columbia, Canada. The main outcome was sustained progression to Expanded Disability Status Scale (EDSS) 6, secondary outcome time to EDSS 8. Risk factors for progression included sex, onset age, onset symptoms, and for time to EDSS 8, time to EDSS 6.
Results: Of the 2,837 patients with definite MS in the original study, 352 (12.4%) had PPMS. Mean disease duration was 17.2 years (0.3 to 49.6 years), mean onset age was 40.1 years (SD 11.5), and 53% (187) were female. One-quarter of the population had reached EDSS 6 after 7.3 years from onset, yet another 25% still did not require a cane after 25 years. Sex, onset age, and onset symptoms did not predict progression (p > 0.05). A shorter time to EDSS 6 predicted a shorter time to EDSS 8 (p < 0.0005).
Discussion: Progression of disability was slower than found in previous primary progressive multiple sclerosis natural history studies. However, considerable variation existed, with few predictors, other than "sooner to cane, sooner to wheelchair."
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the December 27 issue to find the title link for this article.
Deceased.
H.T. is funded by a "Don Paty Career Development Award" from the MS Society of Canada and the Christopher Foundation. The BC-wide MS database was funded by an unrestricted grant from D.P. and the MS/MRI research group. This analysis was funded by a grant from the MS Society of Canada.
Disclosure: The authors report no conflicts of interest.
Received July 14, 2005. Accepted in final form September 16, 2005.
This article has been cited by other articles:
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