Clinical parameters affecting the course of amyotrophic lateral sclerosis: a longitudinal analysis of the Greek registry for amyotrophic lateral sclerosis (ALS-GR)

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Background Reliable prognostic factors are essential for both clinical and research purposes in amyotrophic lateral sclerosis (ALS). The current study aimed to investigate the prognostic properties of clinical parameters regarding the course of ALS.Methods A mixed retrospective-prospective cohort study was conducted in 11 specialized centers in Greece. Participants with ALS were assessed on the ALS functional rating scale-revised (ALSFRS-R) at baseline and follow-up (on 6-9-12-18-24 months). In case of dropouts, we elucidated whether death had occurred. Adjusted survival analyses were used to explore associations with mortality; adjusted generalized estimating equations were used to examine associations with the rate of functional decline.Results A total of 165 individuals with an average follow-up of 12.6 ± 6.3 months were included in the current longitudinal analysis. Baseline ALSFRS-R was the strongest determinant of survival [Hazard-ratio; HR = 0.949 (0.921–0.977)], followed by age at diagnosis [HR = 1.043 (1.011–1.075)] and restrictive respiratory pattern [HR = 2.321(1.193–4.515)], while cramps [HR = 0.485 (0.249–0.946)] and pain [HR = 2.007 (1.041–3.868)] were the least influential. Regarding functional decline rates, the strongest predictor was dysphagia [β= −0.633 (−0.992, −0.274)], followed by pseudobulbar syndrome [β= −0.460 (−0.832, −0.088)], depression [β= −0.375 (−0.688, −0.062)], pain [β=-0.362 (−0.627, −0.096)]. fatigue [β= −0.338 (−0.644, −0.033)], spasticity [β= −0.362 (−0.644, −0.081)] and the interval between diagnosis and baseline visit to a specialized unit in months [β = 0.031 (0.013, 0.048)].Conclusions Lower baseline ALSFRS-R scores, older age at diagnosis, restrictive respiratory pattern, pain and absence of cramps were linked to shorter survival in ALS. Dysphagia, pseudobulbar syndrome, depression, pain, fatigue, spasticity and shorter intervals between diagnosis and baseline visit to specialized units were related to steeper functional decline.

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