Abstract
To compare two recently developed staging systems for amyotrophic lateral sclerosis (ALS) [King's College and Milano-Torino staging (MITOS) systems] in an incident, population-based cohort of patients with ALS. Since 2009, a prospective registry has been recording all incident cases of ALS in the Emilia Romagna region in Italy. For each patient, detailed clinical information, including the ALS functional rating scale score, is collected at each follow-up. Our study on 545 incident cases confirmed that King's College stages occurred at predictable times and were quite evenly spaced out throughout the disease course (occurring at approximately 40%, 60% and 80% of the disease course), whereas MITOS stages were mostly skewed towards later phases of the disease. In the King's College system there was a decrease in survival and an increase in deaths with escalating stages, whereas in the MITOS system survival curves pertaining to intermediate stages overlapped and the number of deaths was fairly homogenous throughout most stages. The King's College staging system had a higher homogeneity (i.e. smaller differences in survival among patients in the same stage) and a higher discriminatory ability (i.e. greater differences in survival among patients in different stages), being more suitable for individualized prognosis and for measuring efficacy of therapeutic interventions.
Highlights
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive disability [1] and great inter-individual variability, making prognosis still a challenge
The present study was aimed at assessing advantages and drawbacks of two recently proposed staging systems for ALS patients when applied in an incident, population-based, cohort
Stages 0-4 of the MITOS staging system are reached after a standardized median times (SMT) of 35, 67, 79, 100 and 104% of the disease course in our cohort
Summary
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive disability [1] and great inter-individual variability, making prognosis still a challenge. The most widely used functional scale in ALS [the ALS Functional Rating Scale- Revised (ALSFRS-R)], has relevant intrinsic limitations: it is multidimensional, representing the sum of mean scores of three different domains, not satisfying rigorous measurement standards [3, 4] To address this urgent unmet need, recently, two staging systems have been developed [5, 6], based on simple clinical milestones marking the course of the disease. The second staging system (namely MITOS system)[6] is based on the loss of independent functions in four key domains of the ALSFRS-R: the sum of the lost functions determines the stage Both systems have only been tested separately in clinical trials/referral-centre populations. The stages were well distributed during the disease course, without reversion to earlier stages , with good correlation with
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