C26 How frequent are pauses in amyotrophic lateral sclerosis progression? Results from a population-based cohort

27-11-2019

MNDA SYMPOSIUM PERTH DEC19 PRESENTATIONS

Session 3 Demographics and clinical features

Italy

Background: Amyotrophic Lateral Sclerosis (ALS) diagnostic criteria state that for diagnosis to be confirmed a ‘progressive spread of symptoms or signs within a region or to other regions’ should be present. However, it is scarcely documented whether ALS progression could show any pause.

Objectives: To assess the frequency of pauses (ie plateaus) in ALS progression.

Methods: Data from the Piedmont and Aosta Valley ALS register were used. All patients incidents in the 2007– 2014 period and who underwent multiple visits were enrolled. Subjects were followed-up until 31st December 2018. A plateau was defined as a stable ALSFRS-R total score lasting 6 months or more.

Results: A total of 1044 ALS patients were included in the study. Of these, 833 (79.9%) died during the follow-up period. A total of 227 (21.7%) patients experienced at least one plateau lasting 6 months or more. Of these, 157 (69.2%) experienced only one plateau, 48 (21.1%) two plateaus while in 21 (9.3%) and 8 (3.5%) patients progression pauses three and four times respectively. Median duration of plateau was 321 days (IQR 229.5–478.0). Plateaus were more common at very high and very low ALSFRS-R scores, with 44 (18 plateaus) and 1 (15 plateaus) being the two most common scores. Of the 227 patients who had at lest one plateau, 110 (10.5%) died during the study period and experienced a total of 142 plateaus, Of these, 44 (31%) occurred in the first quarter, 39 (27.5%) in the second quarter, 44 (31%) in the third quarter and only 15 (10.6%) in the last quarter of the overall clinical course. Finally, a younger age at diagnosis resulted the only factor significantly correlated to the risk of developing a plateau (OR 0.96, 95% CI 0.94–0.97, p-value <0.001).

Discussion and conclusions: Using a population-based cohort, we have found that plateaus are common in ALS progression and should not question the diagnosis. Our finding is in agreement with a previous study using data from the Pooled Resource Open Access Clinical Trials database.

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