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

Objective: Cognitive and behavioural 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 rigour 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 behavioural 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 Neurone 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 behavioural 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 behavioural 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.