Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen in Norway: A Validation Study

Cognitive Impairment in ALS: Screening Tools, Experiences and Prognosis

Cognitive impairment is present in about 30-50% of the patients with amyotrophic lateral sclerosis (ALS). Suitable screening tools are available, but none of these are evaluated in a Norwegian population.

Study Overview

• Status: Completed
• Conditions: Cognitive Impairment; Amyotrophic Lateral Sclerosis
• Intervention / Treatment: Diagnostic test: ECAS-N; Diagnostic test: MoCA

Detailed Description

Screening of cognitive and behavioral impairment is a distinct recommendation in ALS-specific health-care. Thus, a rapid screening tool valid for use in Norway is urgent. However, cognitive assessment for patients with ALS can be difficult due to the complexity of cognitive impairment, as well as motor challenges with writing, drawing and speaking. Therefore, only ALS-specific, multi domain screening instruments with integrated behavioral sections should be used. Internationally, the Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen (ECAS), is recommended for the purpose. Besides being quick and easy to administer, the ECAS is shown to be sensitive and have high specificity to ALS-specific dysfunction and behavioral changes. The introduction of ECAS has probably contributed to a more nuanced picture of cognitive impairment in ALS than previously assumed. Therefore the ECAS has been translated and culturally adapted into Norwegian (ECAS-N). Based on scores from healthy people, Norwegian age- and educational-adjusted norms for verbal fluency (n=277) and cut-off-scores (n=85) for abnormal findings are established. However, further investigation of psychometric properties of the ECAS-N is needed. The objectives of the study are: 1. To investigate if the ECAS-N reflect cognitive impairment (internal consistency), and is robust to measurement errors due to different times of testing (test-retest reliability) and different raters (interrater reliability) 2. To investigate if the ECAS-N can be used to distinguish between people with ALS-specific cognitive impairment, and those who do not have cognitive impairment, and those who have cognitive impairment due to other disorders (construct validity).

• Study Type: Observational
• Enrollment (Actual): 31

Contacts and Locations

Study Locations

• Norway
  • Bergen, Norway, 5021
    • Haukeland University Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 35 years to 85 years (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes

• Sampling Method: Non-Probability Sample

Study Population

Patients who fulfill the inclution criteria and attending to the ALS-unit at HUH, St.Olavs Hospital, Hospital of Southern Norway or Namsos Hospital, within 4 months after being diagnosed With ALS.

Healthy People and patients with dementia will constitute Control groups

Description

Inclusion Criteria:

• voluntary informed consent
• native Norwegian speaker
• aged between 35 and 85 years old (only for Controls)

Exclusion Criteria:

• great difficulties in writing og Reading
• comorbid Medical history
• neurological disorders others than ALS
• psychiatric history of importance to cognitive function

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

3

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Patients with ALS; Patients with ALS will be tested by two independent testers with the ECAS-N at 4 months (baseline) and 8 months (follow-up), and the MoCA at 4 months (baseline) by one tester	Diagnostic test: ECAS-N; All participants included will be tested with the ECAS-N; Other Names: Edinburgh cognitive and behavioral ALS screen (Norwegian); Diagnostic test: MoCA; All included patients With ALS will be tested With the MoCA; Other Names: Montreal cognitive assessment (MoCA)
Healthy controls; Persons with no cognitive impairment will be tested with the ECAS-N once, by one tester	Diagnostic test: ECAS-N; All participants included will be tested with the ECAS-N; Other Names: Edinburgh cognitive and behavioral ALS screen (Norwegian)
Controls with dementia; Persons with cognitive impairment due to other disorders will be tested with the ECAS-N once, by one tester	Diagnostic test: ECAS-N; All participants included will be tested with the ECAS-N; Other Names: Edinburgh cognitive and behavioral ALS screen (Norwegian)

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Edinburgh cognitive and behavioural amyotrophic lateral sclerosis screen - Norwegian Version (ECAS-N)	We will use the ALS-specific sub-score (minimum score = 0, maximum score = 100), the ALS non-specific sub-score (minimum score = 0, maximum score = 36), a summed total ECAS-N score (minimum score =0, maximum score =136), the sub score of behavioural changes (minimum score = 0, maximum score = 10) and the sub score of psychotic change (minimum score = 0, maximum score = 3). A dichotomized cut-off scores for normality will also be used for the ALS-specific cut-off score of 65 or over, the non ALS-specific cut-off score of 24 or over and the total ECAS-N cut-off score of 92 or over. For the ALS-specific scores, non ALS-specific scores and total ECAS-N scores, high scores indicate less problems than low scores. For the sub score of behavioural change and the sub score of psychotic change, high scores indicate more problems than low scores.	4 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Montreal cognitive assessment (MoCA) - Norwegian version	We will use the total MoCA score (minimum score = 0, maximum score = 30) and a dichotomized cut-off score for normality of 26 or over. High scores indicate less problems than low scores	4 months
Change from 4 months Edinburgh cognitive and behavioural amyotrophic lateral sclerosis screen - Norwegian Version (ECAS-N) at 8 months	We will use the changed ALS-specific sub-score (minimum score = 0, maximum score = 100), the changed ALS non-specific sub-score (minimum score = 0, maximum score = 36), a changed summed total ECAS-N score (minimum score =0, maximum score =136), the changed sub score of behavioural changes (minimum score = 0, maximum score = 10) and the changed sub score of psychotic change (minimum score = 0, maximum score = 3). A changed dichotomized cut-off scores for normality will also be used for the ALS-specific cut-off score of 65 or over, the non ALS-specific cut-off score of 24 or over and the total ECAS-N cut-off score of 92 or over. For the ALS-specific scores, non ALS-specific scores and total ECAS-N scores, high scores indicate less problems than low scores. For the sub score of behavioural change and the sub score of psychotic change, high scores indicate more problems than low scores.	8 months

Collaborators and Investigators

• Sponsor: Haukeland University Hospital
• Collaborators: Western Norway University of Applied Sciences
• Investigators: Principal Investigator: Tina Taule, PhD, Haukeland University Hospital

Study record dates

Study Major Dates

• Study Start (Actual): May 1, 2017
• Primary Completion (Actual): December 31, 2022
• Study Completion (Actual): May 4, 2023

Study Registration Dates

• First Submitted: June 1, 2018
• First Submitted That Met QC Criteria: July 5, 2018
• First Posted (Actual): July 6, 2018

Study Record Updates

• Last Update Posted (Actual): October 30, 2024
• Last Update Submitted That Met QC Criteria: October 28, 2024
• Last Verified: October 1, 2024

More Information

Terms related to this study

• Keywords: Validation; Screening; Amyotrophic lateral sclerosis; Cognitive
• Additional Relevant MeSH Terms: Central Nervous System Diseases; Nervous System Diseases; Mental Disorders; Pathologic Processes; Neuromuscular Diseases; Metabolic Diseases; Neurocognitive Disorders; Cognition Disorders; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Sclerosis; Cognitive Dysfunction; Motor Neuron Disease; Amyotrophic Lateral Sclerosis
• Other Study ID Numbers: 2016/3166

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No