The cognitive and behavioural profile of Amyotrophic Lateral Sclerosis: Application of the consensus criteria

Monica Consonni, Sandro Iannaccone, Chiara Cerami, Paola Frasson, Marco Lacerenza, Christian Lunetta, Massimo Corbo, Stefano F. Cappa

Research output: Contribution to journalArticlepeer-review


OBJECTIVE: The study aims to assess the spectrum of cognitive and behavioural disorders in patients affected by Amyotrophic Lateral Sclerosis (ALS) according to the recent consensus criteria [9]. The study also intends to assess the impact of physical disability on cognitive and behavioural abnormalities. METHODS: Detailed neurological, neuropsychological and neurobehavioral evaluations were administered to 23 ALS patients, 11 Lower Motor Neuron Disease (LMND) patients and 39 healthy controls. Strong et al.'s criteria [9] were applied to diagnose the presence of cognitive/behavioural impairment. Clinical and neuropsychological scores were used for group comparisons and correlation analyses. RESULTS: In comparison with LMND and controls, a subgroup of ALS patients (∼30%) manifested executive dysfunction, which was severe enough to classify them as cognitively impaired. Action naming difficulties and short-term memory deficits were also observed. Aspontaneity, disorganization and mental rigidity reached clinical relevance in 20% of ALS patients. A small percentage of ALS patients (13%) also had comorbid dementia. The cognitive or behavioural status was not related to the clinical features of ALS. CONCLUSION: The use of consensus criteria for cognitive and behavioural impairment and the comparison with the LMND group proved useful in defining the spectrum of non-motor manifestations of ALS.

Original languageEnglish
Pages (from-to)143-153
Number of pages11
JournalBehavioural Neurology
Issue number2
Publication statusPublished - 2013


  • Amyotrophic Lateral Sclerosis
  • behavioural impairment
  • cognitive impairment
  • dysexecutive syndrome
  • Lower Motor Neuron Disease
  • motor disability

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Clinical Neurology
  • Neurology


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