Abstract
Approximately 50% of amyotrophic lateral sclerosis (ALS) patients have cognitive and behavioural dysfunction in varying degrees and forms. Previous studies have shown that cognitive and behavioural changes may indicate a poor prognosis, and cognitive function gradually deteriorates over the course of disease, but the results of different studies have been inconsistent. In addition, there are relatively limited long-term follow-up studies tracking death as an endpoint. The purpose of this study was to investigate the clinical prognostic characteristics of ALS patients with cognitive behavioural changes through long-term follow-up in a cohort. A total of 87 ALS patients from 2014 to 2015 in the Third Hospital of Peking University were selected and divided into a pure ALS group, an ALS with behavioural variant of frontotemporal dementia (ALS-bvFTD) group, and an ALS with cognitive and behaviour changes group. All patients were followed up for 60 months. The main end point was death and tracheotomy. There was no significant difference in survival curve between pure ALS and ALS with cognitive and behavioural change group, but the survival time of ALS-bvFTD group was significantly lower than the other two groups (P < 0.001). For those who was followed up to the endpoint, the survival time of the ALS-bvFTD group was significantly shorter than that of the pure ALS group (t = 5.33, p < 0.001) or the ALS with cognitive and behaviour changes group (t = 4.25, p < 0.001). The progression rate of ALS Functional Rating Scale-Revised (FRS-R) scores from recruitment to endpoint was significantly faster in the ALS-bvFTD group than in the pure ALS group (z = 2.68, p = 0.01) or the ALS with cognitive and behavioural changes group (z = 2.75, p = 0.01). There was no significant difference in survival time (t = 0.52, P = 0.60) or FRS-R score progression rate (z = 0.31, p = 0.76) between the pure ALS group and the ALS with cognitive and behavioural changes group. The total Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS) score was positively correlated with survival time (r = 0.38, p = 0.01). ALS-bvFTD patients have shorter survival time. The total ECAS score may be correlated with survival time.
Highlights
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with high disability and mortality rates
There was no significant difference in survival curve between pure ALS and ALS with cognitive and behavioural change group, but the survival time of ALS-behavioural variant of frontotemporal dementia (bvFTD) group was significantly lower than the other two groups (P < 0.001)
There was no significant difference in survival time (t = 0.52, P = 0.60) or Functional Rating Scale–Revised (FRS-R) score progression rate (z = 0.31, p = 0.76) between the pure ALS group and the ALS with cognitive and behavioural changes group
Summary
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with high disability and mortality rates. The clinical heterogeneity of this disease is very strong, and patients with different phenotypes have very different prognosis. In recent years, increasing attention has been paid to cognitive and behavioural changes in ALS. Up to 50% of ALS patients may have different degrees and forms of cognitive and behavioural dysfunction. In 2017, Strong et al proposed the concept of amyotrophic lateral sclerosis frontotemporal spectrum disease (ALS-FTSD) [2]. Previous studies have shown that patients with cognitive and behavioural changes may have a poor prognosis [6,7,8]. The purpose of this study was to investigate the clinical prognostic characteristics of ALS patients with cognitive and behavioural changes through 60-month follow-up in a cohort
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