Characterising aggressive multiple sclerosis

Abstract

Objective To explore the occurrence and characteristics of aggressive multiple sclerosis (AMS) in adult-onset multiple sclerosis (MS) patients. Methods Prospectively collected data from British Columbia, Canada, were retrospectively analysed. AMS was defined in three different ways (AMS1, 2 and 3): 'AMS1'-confirmed Expanded Disability Status Scale (EDSS) ≥6 within 5 years of MS onset; 'AMS2'-confirmed EDSS ≥6 by age 40; and 'AMS3'-secondary progressive MS within 3 years of a relapsing-onset course. Three respective 'non-aggressive' MS comparison cohorts were selected. Patients' characteristics were compared between aggressive and non-aggressive cohorts using multivariable logistic regression, with findings expressed as adjusted OR (AOR) and 95% CI. Results Application of the three definitions to the source population of 5891 patients resulted in 235/4285 (5.5%) patients fulfilling criteria for AMS1 (59.6% were female; 74.5% had relapsing-onset MS), 388/2762 (14.0%) for AMS2 (65.2% were female; 92.8% had relapsing-onset MS) and 195/4918 (4.0%) patients for AMS3 (61.0% were female). Compared to the respective control cohorts, those with AMS were more likely to be male (AOR=1.5, 95% CI 1.1 to 2.0 (AMS1); 1.6, 95% CI 1.3 to 2.1 (AMS2); 1.8, 95% CI 1.3 to 2.4 (AMS3)), older at MS symptom onset (AOR=1.1; 95% CI 1.1 to 1.1 (AMS1 and AMS3)) and have primary progressive MS (AOR=2.3, 95% CI 1.6 to 3.3 (AMS1); 2.7, 95% CI 1.7 to 4.4 (AMS2)). Conclusions AMS was identified in 4-14% of patients, depending on the definition used. Although there was a relative preponderance of men and primary progressive MS presenting with AMS, the majority of patients were still women and those with relapsing-onset MS.

Malignant multiple sclerosis: clinical and demographic prognostic factors

“…The term fulminant MS, although adopted previously, is currently associated with Marburg' s variant in which progression is monophasic with inexorable deterioration and rapid death 7 . Malignant/aggressive forms occur in 4-14% of cases 3,4 ; and this Brazilian cohort, in which 8.5% of patients developed severe disability within five years, confirmed these data. This percentage includes both RRMS and PPMS; however, if only the former were considered, this rate would fall to 5.8%.…”

“…Certain patients reach greater degrees of disability within a short period of time. This severe progressive form is referred to as malignant multiple sclerosis (MMS) when an Expanded Disability Status Scale (EDSS) score of 6 is reached within five years of disease onset 3,4 . Early recognition of potentially severe cases based on prognostic factors is crucial when making therapeutic decisions to reduce the risk of disability and impaired quality of life.…”

Intensive care unit admission in multiple sclerosis

“…We found that the risk of death was increased in the year after ICU admission. The particularly high mortality rate among young persons raised the question of whether this was attributable to aggressive MS, 29 infection, or other causes. Although limited by small numbers, our findings did not suggest that aggressive MS had a differentially greater role at young ages.…”

Severity Grading in Multiple Sclerosis

“…11,21 Many terms have been proposed for this phenomenon-malignant MS, aggressive MS, catastrophic MS, fulminant MS-with a seemingly equal number of definitions, both qualitative and quantitative. [22][23][24] The lack of consensus likely stems from the difficulty of applying a uniform definition of severe MS to patients in different stages of the disease, as can be illustrated with the following examples.…”