Cognitive Impairment in ALS: Screening Tools, Experiences and Prognosis

Study Purpose:

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 Status:



Amyotrophic Lateral Sclerosis , Cognitive Impairment

Study Type:


Type of Intervention:


Intervention Name:






Study Chair(s)/Principal Investigator(s):

Tina Taule, PhD, Haukeland University Hospital ID:


Neals Affiliated?


Coordinating Center Contact Information

Tina Taule, PhD / email hidden; JavaScript is required / +47 41694143

Full Study Summary:

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 Sponsor:

Haukeland University Hospital

Estimated Enrollment:


Estimated Study Start Date:

05 / 01 / 2017

Estimated Study Completion Date:

12 / 31 / 2022

Posting Last Modified Date:

08 / 09 / 2022

Date Study Added to

07 / 06 / 2018

Minimum Age:

35 Years

Maximum Age:

85 Years

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

Haukeland University Hospital | Recruiting

Tina Taule, PhD / +47 41694143 / email hidden; JavaScript is required

Tiina Rekand, Professor / email hidden; JavaScript is required

Bergen 5021