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Benign multiple sclerosis: A longitudinal follow-up study of large Western Australian cohort

Pedrini, M.J., Xu, W., Xu, Z., Seewann, A., Burton, J., Carroll, W.M. and Kermode, A.G. (2013) Benign multiple sclerosis: A longitudinal follow-up study of large Western Australian cohort. In: European Committee for Research and Treatment in Multiple Sclerosis (ECTRIMS) 2013, 2 - 5 October 2013, Copenhagen, Denmark.


Background: The majority of multiple sclerosis (MS) cases are characterized by clinical onset with relapses and remissions (RRMS). However, years after the first disease manifestation most patients experience secondary progression (SPMS). The second principal group of MS patients is primary progressive MS (PPMS). In a minority of MS patients, however, disease never leads to substantial disability and shows little or no progression years after the first symptoms, termed benign MS (BMS). The aim of our study was to evaluate disease status after 15, 20, 25, 30 and 35 years in a well characterized Western Australian longitudinal cohort defined as BMS based on Expanded Disability Status Scale (EDSS) <= 3.5 at 10 years from onset. Methods: The cohort comprises a total of 1415 cases, with followup as long as 50 years. Patients with EDSS <= 3.5 at 10 years from disease onset were selected from the Perth Demyelinating Disease Database. EDSS score at the 15, 20, 25, 30 and 35 year epochs was evaluated. Potential modifying factors influencing prognosis including gender, age at onset, first symptoms, initial MRI, CSF, HLA and disease course were also studied. Results: 417 patients had 10 or more years of detailed followup, of whom 210 patients had EDSS of <= 3.5 10 years (50.3%). Female/male ratio was 5.4 : 1. Average onset age in females was 33 years and in males 36 years. RRMS was the dominant disease course at onset (93 %). Fifteen, 20, 25, 30 and 35 year EDSS scores <= 3.5 were obtained for 122, 64, 33, 17 and 8 of 210, which corresponded to 58.1%, 30.5%, 15.7%, 8.1% and 3.8% of benign outcome, respectively. At 10 years from onset 50% of MS patients were found as benign cases. Every 5 years the group of patients with EDSS <= 3.5 (“benign”) was reduced by approximately 50%. Conclusions: In this large well characterized, geographically isolated, predominantly Anglo-Celtic Western Australian cohort clinical progression was similar to other published series from Western countries. Only 15.7% of patients had EDSS <=3.5 at 25 years, and only 3.8% at 35 years.

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