Cognitive-behavioral longitudinal assessment in ALS: the Italian Edinburgh Cognitive and Behavioral ALS screen (ECAS)

Poletti, Barbara; Solca, Federica; Carelli, Laura; Faini, Andrea; Madotto, Fabiana; Lafronza, Annalisa; Monti, Alessia; Zago, Stefano; Ciammola, Andrea; Ticozzi, Nicola; Abrahams, Sharon; Silani, Vincenzo

doi:10.1080/21678421.2018.1473443

Cited by 41 publications

(26 citation statements)

References 59 publications

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“…Indeed, nearly two-thirds of cases had some evidence of cognitive impairment in more advanced stages. In contrast to previous publications, [2][3][4][5][6][7] our data point to a correlation between the severity of motor impairment and the severity of cognitive deficits, and suggest that cognitive function may not remain stable in patients with ALS. Importantly, our data indicate that the presence of bulbar signs is strongly associated with more severe cognitive impairment at all stages of disease.…”

Section: Discussioncontrasting

confidence: 99%

“…Amyotrophic lateral sclerosis (ALS) can no longer be considered a disease limited to the motor system but rather a multisystem disorder that involves cognitive domains in at least half of the cases. 1 Longitudinal studies point to a relative stability of cognitive function in patients who are not impaired at first examination, and a progression of the impairment in patients already compromised, [2][3][4][5][6][7] although such studies are complicated by the progressive loss of speech and motor function in the hands that hinders the accuracy of neuropsychological testing and causes a high patient attrition rate, further complicating our ability to evaluate the pattern of cognitive impairment in patients with ALS over time. 2,3 A recent study performed on a cross-sectional clinical-based cohort of patients evaluated using the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has shown that cognitive and behavioural deficits are more frequent and severe with advanced disease.…”

Section: Resultsmentioning

confidence: 99%

“…1 Longitudinal studies point to a relative stability of cognitive function in patients who are not impaired at first examination, and a progression of the impairment in patients already compromised, [2][3][4][5][6][7] although such studies are complicated by the progressive loss of speech and motor function in the hands that hinders the accuracy of neuropsychological testing and causes a high patient attrition rate, further complicating our ability to evaluate the pattern of cognitive impairment in patients with ALS over time. 2,3 A recent study performed on a cross-sectional clinical-based cohort of patients evaluated using the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has shown that cognitive and behavioural deficits are more frequent and severe with advanced disease. 8 The aim of our study was to assess the relationship between patients' cognitive impairment, classified according to the recently published revised ALS-FTD Consensus Criteria, 9 and patients' motor impairment, classified according to the King ALS staging system and Milano Torino Staging system (MiToS).…”

Section: Resultsmentioning

confidence: 99%

“…8 Previously published longitudinal studies on ALS cognition purported that patients with normal cognition at first examination remained stable over time. [2][3][4][5][6][7] However, these studies suffered severe attrition rates over the course of their followup (only 50% of patients were retested at 6 months and fewer than 25% at 1 year). Cognitive impairment is known to be associated with a more rapid decline of motor function in ALS.…”

Section: Discussionmentioning

confidence: 99%

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Cognitive impairment across ALS clinical stages in a population-based cohort

et al. 2019

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ObjectiveTo assess the association of the degree of severity of motor impairment to that of cognitive impairment in a large cohort of patients with amyotrophic lateral sclerosis (ALS).MethodsThis is a population-based cross-sectional study on patients with ALS incident in Piemonte, Italy, between 2007 and 2015. Cognitive status was classified according to the revised ALS–FTD Consensus Criteria. The King system and the Milano Torino Staging system (MiToS) were used for defining the severity of motor impairment.ResultsOf the 797 patients included in the study, 163 (20.5%) had ALS–frontotemporal dementia (FTD), 38 (4.8%) cognitive and behavioral impairment (ALScbi), 132 (16.6%) cognitive impairment (ALSci), 63 (7.9%) behavioral impairment (ALSbi), 16 (2.0%) nonexecutive impairment, and 385 (48.2%) were cognitively normal. According to King staging, the frequency of cases with ALS-FTD progressively increased from 16.5% in stage 1–44.4% in stage 4; conversely, the frequency of ALSci, ALSbi, and ALScbi increased from King stage 1 to King stage 3 and decreased thereafter. A similar pattern was observed with the MiToS staging. ALS-FTD was more frequent in patients with bulbar involvement at time of cognitive testing. Patients with C9ORF72 expansion (n = 61) showed more severe cognitive impairment with increasing King and MiToS stages.ConclusionOur findings suggest that ALS motor and cognitive components may worsen in parallel, and that cognitive impairment becomes more pronounced when bulbar function is involved. Our data support the hypothesis that ALS pathology disseminates in a regional ordered sequence, through a cortico-efferent spreading model.

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Section: Discussioncontrasting

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Cognitive impairment across ALS clinical stages in a population-based cohort

et al. 2019

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“…High scores indicate a more severe involvement 37 . The ECAS feasibility in ALS has been confirmed administering longitudinally this test in comparison with standard neuropsychological tests 38 .…”

Section: Ecasmentioning

confidence: 87%

The 6-min walk test as a new outcome measure in Amyotrophic lateral sclerosis

Russo

Lunetta²,

Zuccarino³

et al. 2020

Sci Rep

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One of the issues highlighted in amyotrophic lateral sclerosis (ALS) clinical trials is the lack of appropriate outcome measures. The aim of this multicentric study was to evaluate the 6-min walk test (6MWT) as tool to monitor the natural history of a cohort of ALS patients followed up over a 6-month interval. Forty-four ambulant patients were assessed at baseline and after 1, 3 and 6 months. Eight out of forty-four lost the ability to walk before the end of the study. The 6MWT and the objective measures linked to motor function, such as 10 m walking test (10MWT) and Time-up and go (TUG), the ALSFRS-R and the ALSFRS-R items 7–9 showed a good responsiveness to change over the 6-month interval. There was a strong correlation between 6 and 10MWT, TUG, ALSFRS-R, ALSFRS-R items 7–9 and FVC% at baseline. There was no correlation with Edinburgh Cognitive and Behavioural ALS Screen (ECAS) and Modified Borg Scale (MBS). The Δ of 6MWT from T0 to T6 significantly correlated with the Δs of 10MWT and TUG. There was no correlation with the Δs of ALSFRS-R, ALSFRS-R items 7 9, ECAS, MBS and FVC%. The discordance between changes of the 6MWT and ALSFRS-R at 6 month highlights the different content validity among these instruments. The concordance among 6MWT, 10MWT and TUG indicates that the 6MWT is an objective, sensitive and robust tool to measure motor performances in a longitudinal setting. The main limitations of our study were the small sample size and the high percentage of patients (18%) lost at follow-up. Therefore, further studies on larger cohorts, and exploring the relation between 6MWT and need of ventilator support or survival could strengthen our results.

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Loneliness as neurobehavioral issue in amyotrophic lateral sclerosis

Consonni,

Faltracco,

Dalla Bella

et al. 2024

Ann Clin Transl Neurol

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ObjectiveIn elderly people loneliness represents a risk factor for dementia and may negatively impact on mental and physical health. The specific contribute of loneliness to cognitive and behavioral functioning have not yet been determined in amyotrophic lateral sclerosis (ALS). Our hypothesis was that loneliness may be related to motor dysfunction with a negative impact on cognitive and behavioral decline, possibly related to specific cortical involvement.MethodsIn 200 ALS patients (ALSpts) and 50 healthy controls (HCs) we measured loneliness, mood, and quality of life (QoL). ALSpts underwent comprehensive clinical, genetic, and neuropsychological assessment to define phenotypes. Seventy‐seven ALSpts performed 3T MRI scans to measure cortical thickness. Between‐group, partial correlation and regression analyses were used to examined clinical, neuropsychological, and cortical signatures of loneliness.ResultsFeelings of loneliness were documented in 38% of ALSpts (ALS/L+pts) and in 47% of HCs. In both groups loneliness was associated with anxiety (P < 0.001), depression (P ≤ 0.005), and poor QoL (P < 0.001). ALS/L+pts had similar motor dysfunctions and cognitive abilities than non‐lonely ALSpts, but distinct behavioral profiles (P ≤ 0.005) and frontoparietal involvement (P < 0.05). Loneliness in ALS is related to behavioral changes, apathy, and emotional dysregulation (P < 0.001).InterpretationOur cross‐sectional study indicates that, in ALS, the satisfaction of social environment is associated with a sense of life well‐being that is not limited to the motor status, proving instead that loneliness can impact on disease‐related neurobehavioral changes with a possible flashback on brain architecture. This suggests that sociality could promote personal resilience against behavioral and affective decline in ALS.

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Cognitive-behavioral longitudinal assessment in ALS: the Italian Edinburgh Cognitive and Behavioral ALS screen (ECAS)

Cited by 41 publications

References 59 publications

Cognitive impairment across ALS clinical stages in a population-based cohort

Cognitive impairment across ALS clinical stages in a population-based cohort

The 6-min walk test as a new outcome measure in Amyotrophic lateral sclerosis

Loneliness as neurobehavioral issue in amyotrophic lateral sclerosis

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