Cortical correlates of behavioural change in amyotrophic lateral sclerosis: Journal of Neurology, Neurosurgery and Psychiatry

M. Consonni, S.F. Cappa, E. Dalla Bella, V.E. Contarino, G. Lauria

Research output: Contribution to journalArticlepeer-review


Background Behavioural changes in amyotrophic lateral sclerosis (ALS) are heterogeneous. The study aim was to identify the behavioural profiles of non-demented patients with ALS and their neuroimaging correlates and to elucidate if they are comparable to those reported in studies of the behavioural-variant of frontotemporal dementia (bvFTD). Methods Behavioural changes of 102 non-demented patients with ALS were assessed through the Frontal Behavioural Inventory (FBI), a 24-item scale assessing different behavioural modifications, mainly chosen from the core clinical features of FTD. Principal component analysis (PCA) was used to detect distinct clusters of behavioural changes based on FBI subscores. The cortical thinning related to each behavioural profile was analysed in 29 patients with ALS. Cronbach's α was used to test the reliability of bvFTD-related FBI clustering in our cohort. Results Sixty patients with ALS had FBI score≥1. PCA identified three phenotypic clusters loading on disinhibited/hostile, dysexecutive and apathetic FBI subscores. Imaging analyses revealed that the thinning of bilateral orbitofrontal cortex was related to apathy, the right frontotemporal and cingular cortex to the disinhibited/hostile profile and the left precuneus cortex to the dysexecutive behaviours. The bvFTD-associated aggressive profile reliably applied to our cohort. Conclusions In non-demented patients with ALS, different behavioural profiles could be identified. The right frontotemporal and cingular cortex thinning was the hallmark of the behavioural profile mostly overlapping that described in bvFTD. Our findings provide the unbiased identification of determinants relevant for a novel stratification of patients with ALS based on their behavioural impairment, which might be useful as proxy of cognitive decline. © Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.
Original languageEnglish
Pages (from-to)380-386
Number of pages7
JournalJ. Neurol. Neurosurg. Psychiatry
Issue number4
Publication statusPublished - 2019


  • amyotrophic lateral sclerosis
  • behaviour
  • cortical thickness
  • frontotemporal dementia
  • principal component analysis
  • adult
  • aggression
  • Article
  • behavior change
  • behavior modification
  • brain size
  • cingulate gyrus
  • clinical feature
  • controlled study
  • female
  • Frontal Behavioral Inventory
  • hostility
  • human
  • major clinical study
  • male
  • neuroimaging
  • neuropsychological test
  • orbital cortex
  • precuneus
  • priority journal
  • rating scale
  • aged
  • apathy
  • brain cortex
  • diagnostic imaging
  • executive function
  • frontal lobe
  • middle aged
  • organ size
  • parietal lobe
  • pathology
  • prefrontal cortex
  • psychology
  • temporal lobe
  • Aged
  • Aggression
  • Amyotrophic Lateral Sclerosis
  • Apathy
  • Cerebral Cortex
  • Executive Function
  • Female
  • Frontal Lobe
  • Frontotemporal Dementia
  • Gyrus Cinguli
  • Hostility
  • Humans
  • Inhibition, Psychological
  • Male
  • Middle Aged
  • Organ Size
  • Parietal Lobe
  • Prefrontal Cortex
  • Principal Component Analysis
  • Temporal Lobe


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