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

Study Overview
=================
Brief Summary
-----------------
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.

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).

Official Title
-----------------
Cognitive Impairment in ALS: Screening Tools, Experiences and Prognosis

Conditions
-----------------
Amyotrophic Lateral Sclerosis, Cognitive Impairment

Intervention / Treatment
-----------------
* Diagnostic Test: ECAS-N
* Diagnostic Test: MoCA


Participation Criteria
=================
Eligibility Criteria
-----------------
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

Ages Eligible for Study
-----------------
Minimum Age: 35 Years
Maximum Age: 85 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
Accepts Healthy Volunteers

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients with ALS<br>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<br>* All participants included will be tested with the ECAS-N<br>* Other names: Edinburgh cognitive and behavioral ALS screen (Norwegian);Diagnostic Test: MoCA<br>* All included patients With ALS will be tested With the MoCA<br>* Other names: Montreal cognitive assessment (MoCA);|
| Healthy controls<br>Persons with no cognitive impairment will be tested with the ECAS-N once, by one tester | Diagnostic Test: ECAS-N<br>* All participants included will be tested with the ECAS-N<br>* Other names: Edinburgh cognitive and behavioral ALS screen (Norwegian);|
| Controls with dementia<br>Persons with cognitive impairment due to other disorders will be tested with the ECAS-N once, by one tester | Diagnostic Test: ECAS-N<br>* All participants included will be tested with the ECAS-N<br>* 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 | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Amyotrophic lateral sclerosis, Cognitive, Screening, Validation