Progression and effect of cognitive-behavioral changes in patients with amyotrophic lateral sclerosis

Bock, Meredith; Duong, Y-Nhy; Kim, Anthony; Allen, Isabel Elaine; Murphy, Jennifer; Lomen‐Hoerth, Catherine

doi:10.1212/cpj.0000000000000397

Cited by 28 publications

(26 citation statements)

References 40 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%

“…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: 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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“…Cognitive impairment in ALS is widely regarded as a negative prognostic indicator and linked to reduced survival ( 17 , 18 , 213 ). Neuropsychological deficits in ALS are thought to be associated with increased caregiver burden ( 214 , 215 ) and reduced quality of life ( 216 ). The recognition of the far-reaching effects of neuropsychological deficits on nearly all aspects of ALS care, caregiver support, resource allocation, and prognosis, led to the inclusion of specialist neuropsychologists as core members of ALS multidisciplinary teams worldwide ( 217 , 218 ).…”

Section: Relevance To Clinical Carementioning

confidence: 99%

Clinical and Radiological Markers of Extra-Motor Deficits in Amyotrophic Lateral Sclerosis

et al. 2018

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Amyotrophic lateral sclerosis (ALS) is now universally recognized as a complex multisystem disorder with considerable extra-motor involvement. The neuropsychological manifestations of frontotemporal, parietal, and basal ganglia involvement in ALS have important implications for compliance with assistive devices, survival, participation in clinical trials, caregiver burden, and the management of individual care needs. Recent advances in neuroimaging have been instrumental in characterizing the biological substrate of heterogeneous cognitive and behavioral deficits in ALS. In this review we discuss the clinical and radiological aspects of cognitive and behavioral impairment in ALS focusing on the recognition, assessment, and monitoring of these symptoms.

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“…To date there is no cure and treatment mainly aims to improve or sustain quality of life (QoL) for the patients. Various factors influence QoL and health-related QoL in ALS, including disease progression, depression, anxiety, pain and dysphagia [[3], [4], [5], [6]]. There is a link between mental health and physical health, and many studies showed that psychological factors are related to the course and severity of several physical diseases [7].…”

Section: Introductionmentioning

confidence: 99%

Poor emotional well-being is associated with rapid progression in amyotrophic lateral sclerosis

et al. 2019

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The study aimed to determine the impact of emotional well-being on disease aggressiveness and survival in amyotrophic lateral sclerosis (ALS). In 224 patients with ALS (without significant cognitive deficits) the revised ALS Functional Rating Scale (physical function), the ALS Assessment Questionnaire (ALSAQ-40) for health-related Quality of Life and survival data were collected. Data analysis comprised logistic regression, Kaplan-Meier curves analyses and Cox regression model. Most patients reported to be worried about how the disease will affect them in the future and 67% reported to feel depressed. Patients with good emotional well-being were characterized by better physical function (higher ALSFRS-R) and lower disease aggressiveness. The association between high emotional well-being and lower disease aggressiveness was confirmed in the univariate analysis and also after adjustment for known predictors of disease progression. In the Kaplan-Meier survival curve analysis the overall 1-year, 2-year and 3-year mortality did not significantly differ between patients with poor and good emotional well-being. Our study demonstrates an association between emotional well-being and disease progression. Knowing that subjective well-being is neither a necessary nor a sufficient cause of health, longitudinal studies are necessary to explore when well-being does and does not influence disease progress and survival in ALS.

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Progression and effect of cognitive-behavioral changes in patients with amyotrophic lateral sclerosis

Cited by 28 publications

References 40 publications

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

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

Clinical and Radiological Markers of Extra-Motor Deficits in Amyotrophic Lateral Sclerosis

Poor emotional well-being is associated with rapid progression in amyotrophic lateral sclerosis

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