Abstract
Cognitive impairment (CI) in amyotrophic lateral sclerosis (ALS) may present a serious barrier to a patient’s wellbeing and significantly decrease quality of life. Although reports of CI in ALS without frank dementia are becoming quite common, questions remain regarding the specific cognitive domains affected, as well as how other psychological and medical factors may impact cognitive functioning in these patients. Additionally, the influence of depressive symptoms on disease processes is not known. We aimed to address these questions by completing extensive neuropsychological tests with 22 patients with ALS and 17 healthy volunteers. A subgroup of these patients also completed questionnaires to measure depressive and vegetative symptoms. We tested for overall cognitive differences between groups, the influence of physical (e.g., bulbar and limb), vegetative (e.g., fatigue), and depressive symptoms on cognitive performance, and the relationship between depressive symptoms and disease severity in ALS. Overall, patients performed more poorly than healthy controls (HCs), most notably on tests of executive functioning and learning and memory. Results suggest that true cognitive performance differences exist between patients with ALS and HCs, as these differences were not changed by the presence of vegetative or depressive symptoms. There was no effect of limb or bulbar symptoms on cognitive functioning. Also, patients were not any more depressed than HCs, however increased depressive scores correlated with faster disease progression and decreased limb function. Collectively, it is suggested that translational advances in psychological intervention for those with CI and depression become emphasized in future research.
Highlights
Until recently, amyotrophic lateral sclerosis (ALS) was still considered by many a pure motor neuron disease (Pongratz, 2000), and the notion of cognitive impairment (CI) in ALS was met with much resistance until just the past several years (Bak and Chandran, 2012)
RELATIONSHIP BETWEEN DEPRESSIVE SYMPTOMS, PHYSICAL SYMPTOMS, AND COGNITION IN ALS Depression contributes to decreased quality of life in patients with ALS (Tramonti et al, 2012), our study indicated that as a group patients with ALS were no more likely to be depressed than a healthy controls (HCs) population
Overall, this study supported previous findings that CI is a symptom in approximately 35% of patients with ALS, and that patients overall perform worse than HCs in tests of executive dysfunction and learning and memory
Summary
Amyotrophic lateral sclerosis (ALS) was still considered by many a pure motor neuron disease (Pongratz, 2000), and the notion of cognitive impairment (CI) in ALS was met with much resistance until just the past several years (Bak and Chandran, 2012). Some experts propose that ALS is not a categorical disease, but rather a spectrum disorder between pure ALS and Frontotemporal Lobar Degenerative diseases, such as Frontotemporal dementia (Abrahams et al, 2000; Grossman et al, 2007; Murphy et al, 2007; Woolley and Katz, 2008; Merrilees et al, 2010). One critical reason for this research is that cognitive difficulties in patients with ALS negatively impact quality of life, and appear to be even more detrimental to quality of life than physical impairment (Goldstein et al, 2002). CI can influence decision-making (Girardi et al, 2011), treatment compliance (Olney et al, 2005), survival (Olney et al, 2005; Flaherty-Craig et al, 2011), and even the wellbeing of caregivers (Merrilees et al, 2010)
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