Validation of the revised classification of cognitive and behavioural impairment in ALS

Abstract

ObjectiveIn 2017, the diagnostic criteria for cognitive and behavioural impairment in amyotrophic lateral sclerosis (ALS) with frontotemporal dementia (ALSFTD-1) have been modified (ALSFTD-2) with the inclusion of a novel category (ALS with combined cognitive and behavioural impairment, ALScbi) and with changes of operational criteria of the other categories (ALS with cognitive impairment (ALSci), ALS with behavioural impairment (ALSbi) and ALS with frontotemporal dementia (ALS-FTD)). We compared the two sets of criteria to assess the effect of the revised criteria on the cognitive classification of patients with ALS.MethodsTwo cohorts of patients with ALS were included in this study: a population-based cohort including patients identified through the Piemonte/Valle d’Aosta register for ALS in the 2014–2017 period (n=321), and a referral cohort recruited at the Turin ALS centre and at the ALS centre of the Maugeri Institute in Milan in the same period (n=205). Cognitive function was classified in blind by two neuropsychologists expert in ALS.ResultsALSFTD-2 criteria determined a shift of about 15% of patients from their original category to a new one. In both cohorts, about 9% of patients were reclassified to the novel category ALScbi. Among patients previously classified as cognitively normal, 14 (4.3%, population-based cohort) and 19 (9.3%, referral cohort) were reclassified as ALSbi or ALSci. The median survival of the different categories was significantly different with both with sets of criteria.ConclusionsThe new ALSFTD-2 criteria, compared with the old ones, have positive effects on the clinical practice being more sensitive to the early cognitive impairment and having a better prognostic yield.

Plateaus in amyotrophic lateral sclerosis progression: results from a population‐based cohort

“…Such a finding is in line with the shorter survival found in patients with cognitive impairment, also because of a poor compliance with treatment [18]. Furthermore, it confirms our previous study on a different sample showing that survival of behaviourally impaired patients was intermediate between that of normal and cognitively impaired patients [19]. Notably, none of the considered variables was related to the timing of plateaus during the disease course.…”

Cognitive impairment across ALS clinical stages in a population-based cohort

“…16 The battery was administered following the same sequence in order to avoid the possible differential interference of the answers of one test over the others. The administration of the battery required about 1.5 hours, 17 and was performed in the morning. If the participant felt too tired, a further session was scheduled to complete the battery within 2 weeks of the first session.…”

The Characteristics of Cognitive Impairment in ALS Patients Depend on the Lateralization of Motor Damage

“…The battery included a series of tests aimed at evaluating executive function, memory, visuospatial function, and language, selected according to the diagnostic criteria for the behavioral variant of frontotemporal dementia [11] and the amyotrophic lateral sclerosis frontotemporal dementia (ALS-FTD) consensus criteria [12]. Specifically for this study we considered mini-mental state examination (MMSE), Wisconsin card sorting test (WCST), trail-making test A (TMT-A) and B (TMT-B) and (TMT-B-A), digit span forward (digit span FW) and backward (digit span BW), letter (FAS) and category (CAT) fluency test, Rey auditory verbal learning test (RAVLT), Babcock story recall test (BSRT), Rey-Osterrieth complex figure test (ROCFT), Raven's colored progressive matrices (CPM47), and frontal assessment battery (FAB) [13]. The study design was approved by the Ethical Committee of the Azienda Ospedaliero-Universitaria Città della Salute (prot.…”