Screening for cognition in amyotrophic lateral sclerosis: test characteristics of a new screen
Open Access
- 6 February 2021
- journal article
- research article
- Published by Springer Science and Business Media LLC in Zeitschrift für Neurologie
- Vol. 268 (7), 2533-2540
- https://doi.org/10.1007/s00415-021-10423-x
Abstract
Cognitive and behavioural impairment in amyotrophic lateral sclerosis (ALS) negatively influences the quality of life and survival, and, therefore, screening for these impairments is recommended. We developed a cognitive screening tool, the amyotrophic lateral sclerosis–frontotemporal dementia–cognitive screen (ALS–FTD–Cog) and aimed to validate it in patients with ALS. During the current study, the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was published and we, therefore, decided to compare these two cognitive screening methods. The ALS–FTD–Cog was administered to 72 patients with ALS, 21 patients with behavioural variant FTD (bvFTD) and 34 healthy controls. Twenty-nine patients with ALS underwent the ECAS. ROC curve analyses were performed and sensitivity and specificity of the ALS–FTD–Cog and ECAS were calculated, with a neuropsychological examination (NPE) as the gold standard. Cognitive impairment was present in 28% of patients with ALS. ROC curve analyses of the ALS–FTD–Cog and ECAS showed an area under the curve (AUC) of 0.72 (95% CI 0.58–0.86) and 0.95 (95% CI 0.86–1.03), respectively. Compared to a full NPE, sensitivity and specificity of the ALS–FTD–Cog were 65.0% and 63.5% and of the ECAS 83.3% and 91.3%, respectively. The sensitivity and specificity of the ALS–FTD–Cog in patients with bvFTD were 94.4% and 100%, respectively. Test characteristics of the ALS–FTD–Cog were moderate, suggesting restricted practical value, as compared to a comprehensive NPE. The ECAS had an excellent AUC and high sensitivity and specificity, indicating that it is a valid screening instrument for cognitive impairment in ALS.Funding Information
- Stichting ALS Nederland (2013-19)
- Amsterdam UMC
This publication has 37 references indexed in Scilit:
- Behavior Matters—Cognitive Predictors of Survival in Amyotrophic Lateral SclerosisPLOS ONE, 2013
- The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based studyJournal of Neurology, Neurosurgery & Psychiatry, 2011
- Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementiaBrain, 2011
- Cognitive and behavioural deficits associated with the orbitomedial prefrontal cortex in amyotrophic lateral sclerosisBrain, 2010
- Neurobehavioral symptoms in ALS are negatively related to caregivers’ burden and quality of lifeEuropean Journal of Neurology, 2010
- Detecting frontotemporal dysfunction in ALS: Utility of the ALS Cognitive Behavioral Screen (ALS-CBS™)Amyotrophic Lateral Sclerosis, 2009
- Penn State screen exam for the detection of frontal and temporal dysfunction syndromes: Application to ALSAmyotrophic Lateral Sclerosis, 2009
- The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory functionJournal of the Neurological Sciences, 1999
- A validation study of the Hospital Anxiety and Depression Scale (HADS) in different groups of Dutch subjectsPsychological Medicine, 1997
- Hippocampal and neocortical ubiquitin-immunoreactive inclusions in amyotrophic lateral sclerosis with dementiaNeuroscience Letters, 1992