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Updated: Jun 17, 2026
Comprehensive Autopsy Program for Individuals with Multiple Sclerosis
Published on: July 19, 2019
The natural history of primary progressive multiple sclerosis
Marcus Koch1, Elaine Kingwell, Peter Rieckmann
1Faculty of Medicine, Division of Neurology, University of British Columbia, Vancouver, Canada. m.w.koch@neuro.umcg.nl
Background:
Primary progressive multiple sclerosis (PPMS) carries the worst prognosis of the multiple sclerosis (MS) subtypes and is currently untreatable. A previous analysis of the British Columbia MS database challenged the view that disability progression is rapid in PPMS, but identified few predictors of disease progression. Here, we extend previous analyses in an updated PPMS retrospective cohort study of prevalent cases.
Methods:
We used Kaplan-Meier survival analyses and Cox regression models to investigate the influence of gender, age at onset, and onset symptoms on time to and age at Expanded Disability Status Scale (EDSS) 6.0 in patients with PPMS.
Results:
Of 5,779 patients with definite MS, 552 (10%) had PPMS. Median time to EDSS 6.0 was 14.0 years (95% confidence interval [CI] 11.3-16.7), reached at a median age of 58.6 years (95% CI 56.8-60.3). Sensory onset symptoms were associated with a longer time to and an older age at EDSS 6.0 (multivariable hazard ratios 0.55 [95% CI 0.35-0.87] and 0.54 [0.35-0.85]). Younger age at disease onset was associated with a longer time to but a younger age at EDSS 6.0. Gender and other onset symptoms were not associated with these outcomes. Fifty patients with PPMS (9%) fulfilled criteria for benign MS (EDSS < or =3.0 after 10 years' disease duration).
Conclusions:
We identified 2 predictors of a slower disease progression in primary progressive multiple sclerosis. Sensory onset symptoms were associated with both a longer time to and a higher age at Expanded Disability Status Scale (EDSS) 6.0. A younger age at disease onset was associated with a longer time to EDSS 6.0, but patients with an early disease onset reached EDSS 6.0 at a younger age.
Insights
Predictors of slower disease progression in primary progressive multiple sclerosis (PPMS) were identified. Sensory onset symptoms and younger age at onset were linked to longer time to Expanded Disability Status Scale (EDSS) 6.0.
Area of Science:
- Neurology
- Clinical Research
- Epidemiology
Background:
- Primary progressive multiple sclerosis (PPMS) is an aggressive subtype of multiple sclerosis (MS) with no current treatments.
- Previous studies suggested rapid disability progression in PPMS, but predictors were not well-defined.
- This study updates analyses on a PPMS cohort to identify progression predictors.
Purpose of the Study:
- To investigate predictors of disease progression in primary progressive multiple sclerosis.
- To determine the influence of gender, age at onset, and onset symptoms on time to and age at Expanded Disability Status Scale (EDSS) 6.0.
Main Methods:
- Retrospective cohort study of prevalent primary progressive multiple sclerosis cases.
- Kaplan-Meier survival analyses and Cox regression models were employed.
- Analysis focused on time to and age at Expanded Disability Status Scale (EDSS) 6.0.
Main Results:
- Out of 5,779 MS patients, 552 (10%) had PPMS.
- Median time to EDSS 6.0 was 14.0 years, reached at a median age of 58.6 years.
- Sensory onset symptoms were associated with longer time to and older age at EDSS 6.0. Younger age at onset correlated with longer time to EDSS 6.0 but reaching it at a younger age.
Conclusions:
- Sensory onset symptoms predict slower progression in PPMS, indicating longer time and older age to reach EDSS 6.0.
- Younger age at disease onset also predicts longer time to EDSS 6.0, though the age at reaching this milestone is younger.
- These findings identify key predictors for disease progression in PPMS, aiding in prognosis and management strategies.
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