Screening for cognitive and behavioral change in amyotrophic lateral sclerosis/motor neuron disease: a systematic review of validated screening methods

Abstract

Objectives: Cognitive and behavioral change in Amyotrophic Lateral Sclerosis (ALS) is well-accepted. Several screening tools have been developed to detect such changes. Further guidance on their use may come from a consideration of the rigor with which they were validated. This systematic review set out to critically appraise and present published data pertaining to the validation of six screening tools used to diagnose cognitive and/or behavioral change in patients with ALS. Methods: The screening tools considered in this search included: The Edinburgh Cognitive and Behavioural ALS Screen (ECAS), The ALS Cognitive Behavioural Screen (ALS-CBS), The Motor Neuron Disease Behavioural Scale (MiND-B), The Frontal Behavioural Inventory ALS Version, The ALS Frontotemporal Dementia Questionnaire (ALS-FTD-Q), and The Beaumont Behavioural Inventory (BBI). MEDLINE, EMBASE, and PsycINFO were searched until 4th week of June 2017. Results: Fourteen eligible studies were included in the review. Papers either reported data concerning convergent validity or clinical validity. Validation data concerning the ECAS showed this screening tool to have strong clinical validity, although further work needs to consider how its use will affect diagnosis rates according to current diagnostic guidelines. When screening for behavioral change only, more limited information is available; the BBI may offer greater potential than the ALS-FTD-Q for detecting mild impairment as it assesses a wider range of behavioral changes. Conclusions: Scores of sensitivity, specificity, positive predictive values, and negative predictive values should be given considerable importance when considering which screening tools to incorporate into current clinical practice.

Keywords:

• Amyotrophic Lateral Sclerosis
• cognition
• behavior
• screening tests
• validity

Ethical approval

This article does not contain any studies with human participants performed by either of the authors.

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.

Additional information

Funding

This work represents independent research part-funded (LHG) by the NIHR Maudsley Biomedical Research Centre at the South London and Maudsley NHS Foundation Trust and the Institute of Psychiatry, Psychology and Neuroscience, King’s College London, UK. The views expressed are those of the author and not necessarily those of the NHS, the NIHR or the Department of Health