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

Study Overview
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Brief Summary
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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.

Detailed Description
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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

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

Conditions
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Amyotrophic Lateral Sclerosis, Cognitive Impairment

Intervention / Treatment
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* Diagnostic Test: ECAS-N
* Diagnostic Test: MoCA
* Diagnostic Test: CDR
* Other: Questionnaire


Participation Criteria
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Eligibility Criteria
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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
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Persons with ALS<br>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<br>* assessing ALS-specific cognitive impairment<br>* Other names: Edinburgh cognitive and behavioural ALS screen;Diagnostic Test: MoCA<br>* assessing cognitive impairment<br>* Other names: Montreal cognitive assessment;Diagnostic Test: CDR<br>* assessing global cognitive impairment, as well as possible diagnosis- and Level of dementia<br>* Other names: Clinical dementia rating;Other: Questionnaire<br>* Questions related to work situation and car driving<br>|

What is the study measuring?
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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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Amyotrophic Lateral Sclerosis, Cognitive, Predictor