Screening for cognitive and behavioural impairment in amyotrophic lateral sclerosis: Frequency of abnormality and effect on survival

Abstract

Abstract:Objective: To screen for cognitive and behavioral impairment in people with amyotrophic lateral sclerosis (ALS) and controls with neuromuscular disease and to correlate these with clinical features. Methods

Clinical and laboratory features of ALS

“…In addition, the levels in patients with cognitive impairment showed greater deviation from the healthy controls than those of patients without cognitive impairment, consistent with the suggestion that patients with cognitive impairment have more severe and widespread disease [269] . Between ALS with cognitive impairment and controls there was a significant difference in the expression of clusterin.…”

“…Because ALS is a heterogeneous disease, patients were subdivided on the basis of the presence of absence of cognitive impairment based on results of the ACE-III test. ALS participants with cognitive impairment measured with ACE-III had worse prognosis [239,269] , so this is a distinction that has clinical importance. Patients with cognitive impairment measured with these tools have more severe and widespread MRI changes [283] .…”

“…The basis for subdivision into cognitive and non-cognitive groups was that these subgroups have different survival [239,269] and patients with cognitive impairment have more widespread disease [270] . The basis for the study of carriers of C9orf72 mutations is that these represent a clear subgroup of ALS.…”

Energy metabolism in amyotrophic lateral sclerosis: assessment of body composition and energy expenditure

“…Patients also vary in the presence or absence of cognitive impairment (Xu et al, 2017) and in the presence or absence of other non-motor issues .…”

“…Altered metabolism (Ahmed et al, 2014a) and changes in energy expenditure (Ahmed et al, 2016c) have been reported in patients with frontotemporal dementia (FTD). Cognitive impairment similar to that seen in FTD occurs in some ALS patients (Xu et al, 2017), and ALS and FTD appear to exist at opposite ends of a common disease spectrum (Ahmed et al, 2016a, De Silva et al, 2016. However, compared to patients with ALS, patients with FTD display a distinct metabolic profile, characterized by a higher BMI (Ahmed et al, 2014b) and altered eating patterns (Ahmed et al, 2016b).…”