Amyotrophic lateral sclerosis patients show executive impairments on standard neuropsychological measures and an ecologically valid motor-free test of executive functions

Štukovnik, Vita; Zidar, Janez; Podnar, Simon; Repovš, Grega

doi:10.1080/13803391003749236

Cited by 24 publications

(18 citation statements)

References 75 publications

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“…These findings have important clinical implications, with neuropsychological impairment previously associated with reduced survival, 40 , 41 quality of life, 42 , 43 caregiver burden, 44 , 45 and the ability to manage and engage in life-prolonging interventions. 46 , 47 It is therefore possible that quality of life and caregiver burden may also relate to disease stage. Clinically, it may be necessary to consider intervention programs for caregivers to alleviate the impact of neuropsychological impairment, particularly early in the disease course.…”

Section: Discussionmentioning

confidence: 99%

ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS

Newton²,

et al. 2018

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ObjectiveTo elucidate the relationship between disease stage in amyotrophic lateral sclerosis (ALS), as measured with the King's Clinical Staging System, and cognitive and behavioral change, measured with the Edinburgh Cognitive and Behavioural ALS Screen (ECAS).MethodsA large multicenter observational cohort of 161 cross-sectional patients with ALS and 80 healthy matched controls were recruited across 3 research sites (Dublin, Edinburgh, and London). Participants were administered the ECAS and categorized into independent groups based on their King's clinical disease stage at time of testing.ResultsSignificant differences were observed between patients and controls on all subtests of the ECAS except for visuospatial functioning. A significant cross-sectional effect was observed across disease stages for ALS-specific functions (executive, language, letter fluency) and ECAS total score but not for ALS-nonspecific functions (memory, visuospatial). Rates of ALS-specific impairment and behavioral change were also related to disease stage. The relationship between cognitive function and disease stage may be due to letter fluency impairment, whereas higher rates of all behavioral domains were seen in later King's stage. The presence of bulbar signs, but not site of onset, was significantly related to ALS-specific, ECAS total, and behavioral scores.ConclusionALS-specific cognitive deficits and behavioral impairment are more frequent with more severe disease stage. By end-stage disease, only a small percentage of patients are free of neuropsychological impairment. The presence of bulbar symptoms exaggerates the differences observed between disease stages. These findings suggest that cognitive and behavioral change should be incorporated into ALS diagnostic criteria and should be included in future staging systems.

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Section: Discussionmentioning

confidence: 99%

ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS

Newton²,

et al. 2018

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“…The participants were asked to assemble a daily safe scheme for the administration of the medication. Despite correction for motor disability, MND patients performed worse than the control group [26].…”

Section: Executive Dysfunctionmentioning

confidence: 99%

Cognition and behaviour in motor neurone disease

Lillo

Hodges

2010

Current Opinion in Neurology

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“…Not only has the ECAS shown high sensitivity and specificity to cognitive impairment against a full neuropsychological battery (17)(18)(19)(20)(21), it has high clinical utility in describing the nature of these impairments. Monitoring progression of cognitive and behavioural symptoms may have important implications for patient management, treatment, prognosis, end-oflife decision making, and caregiver burden (22)(23)(24)(25)(26)(27)(28). The purpose of this study was to develop alternate forms of the ECAS to facilitate repeated assessment and longitudinal monitoring of cognition and behaviour in patients with ALS.…”

Section: Discussionmentioning

confidence: 99%

“…Cognitive dysfunction may have important implications for patient management, treatment fidelity, power of attorney, and endof-life decision making (22)(23)(24). Behaviour change has been linked to increased carer burden (25,26) and shortened survival (27,28).…”

Section: Introductionmentioning

confidence: 99%

ECAS A-B-C: alternate forms of the Edinburgh Cognitive and Behavioural ALS Screen

Crockford

Kleynhans

Wilton

et al. 2017

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Background: The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) is a short assessment by which neuropsychological symptoms can be detected and quantified in people with ALS. To avoid potential practice effects with repeated administration, here we present alternative versions of the ECAS suitable for measuring change over time. Objective: To develop two alternate versions of the ECAS: ECAS-B and ECAS-C. Method: One hundred and forty-nine healthy adult participants were recruited. Thirty participants completed a pilot study in developing the alternate versions. Two groups of 40 participants were administered the ECAS-B or ECAS-C and compared to published data of the original ECAS (ECAS-A) to determine equivalence. An additional 39 participants were administered the ECAS consecutively, either repeating the original version (ECAS-A-A-A) serially or the different versions (ECAS-A-B-C) to determine potential practice effects. Recordings of assessments were scored by a second researcher to determine inter-rater reliability. Results: No significant differences were found between versions (A, B, C) of the composite performance measures of ALS Specific, ALS Non-Specific, and ECAS Total scores. Repeated serial administration of ECAS-A (A-A-A) produced some practice effects for composite scores, whereas no such effects were found when alternate versions were administered serially (A-B-C). Exceptionally high intra-class correlations were found for all three versions of the ECAS suggesting a high degree of rater agreement. Conclusion: The newly developed alternate forms of the ECAS are both highly equitable to the original ECAS-A and enable avoidance of practice effects, thus supporting their use in measuring cognition and behaviour over time.

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Amyotrophic lateral sclerosis patients show executive impairments on standard neuropsychological measures and an ecologically valid motor-free test of executive functions

Cited by 24 publications

References 75 publications

ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS

ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS

Cognition and behaviour in motor neurone disease

ECAS A-B-C: alternate forms of the Edinburgh Cognitive and Behavioural ALS Screen

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