Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Aug 11;16(8):e0253279.
doi: 10.1371/journal.pone.0253279. eCollection 2021.

Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up

Shan Ye  1   2 , Pingping Jin  1   2 , Lu Chen  1   2 , Nan Zhang  1   2 , Dongsheng Fan  1   2
Affiliations

Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up

Shan Ye et al. PLoS One. .

Abstract

Objective: 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.

Methods: 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.

Results: 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).

Conclusion: ALS-bvFTD patients have shorter survival time. The total ECAS score may be correlated with survival time.

PubMed Disclaimer

Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Fig 1
Fig 1. Flow of participants through the study.
Fig 2
Fig 2. Survival analysis curve of ALS patients.
Fig 3
Fig 3. Comparison of survival time among subgroups.
Fig 4
Fig 4. Comparison of FRS-R progression rate from enrollment to death among subgroups.
Fig 5
Fig 5. Correlation between total ECAS score and survival time.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Burrell JR, Halliday GM, Kril JJ, et al.. The frontotemporal dementia-motor neuron disease continuum. Lancet. 2016; 388(10047):919–931. doi: 10.1016/S0140-6736(16)00737-6 - DOI - PubMed
    1. Strong MJ, Abrahams S, Goldstein LH, et al.. Amyotrophic lateral sclerosis—frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria. Amyotroph Lateral Scler Frontotemporal Degener. 2017; 18(3–4):153–174. doi: 10.1080/21678421.2016.1267768 - DOI - PMC - PubMed
    1. Iazzolino B, Pain D, Peotta L, et al.. Validation of the revised classification of cognitive and behavioural impairment in ALS. J Neurol Neurosurg Psychiatry. 2019; 90(7):734–739. doi: 10.1136/jnnp-2018-319696 - DOI - PubMed
    1. Goldstein LH, Abrahams S. Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment. Lancet Neurol. 2013;12:368–80 doi: 10.1016/S1474-4422(13)70026-7 - DOI - PubMed
    1. Phukan J, Elamin M, Bede P, Jordan N, Gallagher L, Byrne S, et al.. The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry. 2012;83:102–8. doi: 10.1136/jnnp-2011-300188 - DOI - PubMed

Publication types