Davide Maimone (Italy)
Ospedale Garibaldi Centro Department of NeurologyAuthor Of 2 Presentations
LONG-TERM DISABILITY PROGRESSION IN CHILDHOOD AND ADOLESCENT ONSET MULTIPLE SCLEROSIS PATIENTS
- Ermelinda De Meo (Italy)
Abstract
Background and Aims:
Early onset pediatric multiple sclerosis (MS) is extremely rare, occurring in 0.2-0.6% of all MS cases. The aim of this study is to describe and compare disease course and prognosis of early and late (ie, disease onset before and after age 11 years) onset pediatric MS.
Methods:
Prospectively collected clinical information from Italian MS Register from 1993 pediatric MS patients, of whom 172 with early onset, was analyzed. Cox proportional hazards regression models adjusted for sex and disease-modifying treatments (DMT) exposure were used to assess the risk of reaching confirmed Expanded Disability Status Scale (EDSS) scores of 3, 4, and 6 in early vs late onset pediatric MS, along with prognostic factors.
Results:
A greater proportion of males, isolated brainstem involvement, and longer time to first relapse was observed in early vs late onset pediatric MS patients. Compared to late onset, early onset pediatric MS patients took longer time from disease onset to convert to a secondary progressive phenotype and to reach all 3 disability milestones, thus reaching them at the same age. Recovery from first demyelinating event, time to first relapse, annualized relapse rate during the first 3 years of disease and DMT exposure were independent predictors for long-term disability in early onset pediatric MS patients.
Conclusions:
The different natural history of early vs late onset pediatric MS underscores the existence of specific pathophysiological mechanisms as well as a greater capability to counteract damage in early onset pediatric MS patients.
PROGRESSION INDEPENDENT OF RELAPSE ACTIVITY IN EARLY MULTIPLE SCLEROSIS PATIENTS
- Emilio Portaccio (Italy)
Abstract
Background and Aims:
Disability accrual in multiple sclerosis (MS) may occur as relapse-associated worsening (RAW) or progression independent of relapse activity (PIRA). We investigated the contribution of RAW and PIRA to confirmed disability accumulation (CDA) in patients with clinically isolated syndrome (CIS) and early relapsing-remitting (RR) MS.
Methods:
Relapsing-onset MS patients assessed within one year from onset and with follow-up >/= 5 years (n=5,340) were extracted from the Italian MS Registry. CDA was defined by an increase in Expanded Disability Status Scale (EDSS) score confirmed at 6 months, and classified per temporal association with relapses. Predictors of PIRA and RAW were assessed using multivariable Cox regression models.
Results:
PIRA occurred in 1472 (27.6%) and RAW in 240 (17.6%) patients. Predictors of PIRA were older age (HR=1.02;95%CI 1.02-1.03,p<0.001), RR course (HR=1.46;95%CI 1.30-1.64,p<0.001), longer disease duration (HR=1.49;95%CI 1.22-1.82,p<0.001), lower EDSS (HR=0.89;95%CI 0.85-0.93,p<0.001), lower number of relapses before the event (HR=0.93;95%CI 0.91-0.95,p<0.001). RAW was associated with younger age (HR=0.99;95%CI 0.98-0.99,p<0.001), RR course (HR=1.56; 95%CI 1.35-1.80,p<0.001), lower EDSS (HR=0.92;95%CI 0.87-0.97,p=0.002), higher number of relapses before the event (HR=1.07;95%CI 1.05-1.09,p<0.001). Longer exposure to disease modifying drugs (DMD) reduced the risk of both PIRA and RAW (p<0.001).
Conclusions:
in this early relapsing-onset MS cohort, PIRA was an important contributor to CDA. Our findings indicate that insidious progression appears even in the earliest phases of the disease, suggesting that inflammation and neurodegeneration can represent a single disease continuum. The analysis on progression independent of relapse and radiological activity is ongoing.