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

Cognitive Impairment in ALS: Screening Tools, Experiences and Prognosis

This study evaluate use of a translated Norwegian version of the Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen (ECAS-N) as an early predictor in car-driving, working and use of advanced life-prolonging therapy.

Study Overview

• Status: Active, not recruiting
• Conditions: Cognitive Impairment; Amyotrophic Lateral Sclerosis
• Intervention / Treatment: Diagnostic test: ECAS-N; Diagnostic test: MoCA; Diagnostic test: CDR; Other: Questionnaire

Detailed Description

Cognitive impairment is present in about 30-50% of the patients with amyotrophic lateral sclerosis (ALS). Screening of cognitive and behavioral impairment is a distinct recommendation in ALS-specific health care. However, knowledge in how cognitive impairment shall influence health-care professionals' information given to patients and in decision making is lacking.

One of the major challenges in ALS management is the decision-making on advanced therapy. There is a lack of knowledge in how cognitive impairment in ALS shall be interfere on complex medical treatment that will affect quality of life or life itself. This means significant implications not only to the ALS patient and the community, but also the family and especially the spouse. Thus, further investigation of the ECAS-N and its potential in clinical use is needed. The scale may contribute a more proactive treatment better tailored to individual needs. The objective is to evaluate if the ECAS-N can be applied as an early predictor in car-driving, working and use of advanced life-prolonging therapy

• 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: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Patients who fullfill the inclusion criteria and attending to the ALS-clinic at HUH within 4 months after being diagnosed with ALS.

Description

Inclusion Criteria:

• Voluntary informed consent
• Native Norwegian speaker

Exclusion Criteria:

• Great difficulties in writing or 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

1

Cohorts and Interventions

Group / Cohort

Intervention / Treatment

Persons with ALS; Persons With possible ALS-specific cognitive impairment will be tested With ECAS-N, MoCA, CDR and a questionnaire at 4 months (baseline) and 8 months (1. follow-up). Further evaluation will be with the questionnaire and CDR at each follow-up until 3 years or use of permanent ventilation support or Death. Information about use of advanced life-prolonging therapy will be collected from patient journal.

Diagnostic test: ECAS-N; assessing ALS-specific cognitive impairment; Other Names: Edinburgh cognitive and behavioural ALS screen; Diagnostic test: MoCA; assessing cognitive impairment; Other Names: Montreal cognitive assessment; Diagnostic test: CDR; assessing global cognitive impairment, as well as possible diagnosis- and Level of dementia; Other Names: Clinical dementia rating; Other: Questionnaire; Questions related to work situation and car driving

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Clinical Dementia Rating (CDR)	We will use the total CDR score (minimum score = 0, maximum score = 18). Low scores indicate less problems than high scores.	8 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Clinical Dementia Rating (CDR)	We will use the total CDR score (minimum score = 0, maximum score = 18). Low scores indicate less problems than high scores.	4 months
Clinical Dementia Rating (CDR)	We will use the total CDR score (minimum score = 0, maximum score = 18). Low scores indicate less problems than high scores.	3 years or until death
Ability in car-driving	We will use a categorical variable (yes or no) and time of change to reduced function.	8 months
Ability in car-driving	We will use a categorical variable (yes or no) and time of change to reduced function.	4 months
Ability in car-driving	We will use a categorical variable (yes or no) and time of change to reduced function.	3 years or until death
Working ability	We will use a categorical variable (yes or no) and time of change to reduced function.	8 months
Working ability	We will use a categorical variable (yes or no) and time of change to reduced function.	4 months
Working ability	We will use a categorical variable (yes or no) and time of change to reduced function.	3 years or until death
Use of Advanced life-prolonging therapy	We will use a categorical variable (yes or no) and time of change to reduced function.	8 months
Use of Advanced life-prolonging therapy	We will use a categorical variable (yes or no) and time of change to reduced function.	4 months
Use of Advanced life-prolonging therapy	We will use a categorical variable (yes or no) and time of change to reduced function.	3 years or until death

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
Edinburgh cognitive and behavioral 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
Change from 4 months Edinburgh cognitive and behavioral 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
Montreal Cognitive Assessment (MoCA)	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 Montreal Cognitive Assessment (MoCA) at 8 months	We will use the changed total MoCA score (minimum score = 0, maximum score = 30) and the changed dichotomized cut-off score for normality of 26 or over. High scores indicate less 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 (Estimated): December 31, 2026

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: Amyotrophic Lateral Sclerosis; Cognitive; Predictor
• 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: 2016/2187-1

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

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