title: The cognitive and behavioural profile of amyotrophic lateral sclerosis: application of the consensus criteria.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Clinical Neurosciences and Division of Neuroscience, San Raffaele Scientific Institute, Milan, Italy Vita-Salute University, Via Olgettina, Milan, Italy. consonni.monica@hsr.it
authors.forename: Monica
authors.initials: M
authors.lastname: Consonni
authors.forename: Sandro
authors.initials: S
authors.lastname: Iannaccone
authors.forename: Chiara
authors.initials: C
authors.lastname: Cerami
authors.forename: Paola
authors.initials: P
authors.lastname: Frasson
authors.forename: Marco
authors.initials: M
authors.lastname: Lacerenza
authors.forename: Christian
authors.initials: C
authors.lastname: Lunetta
authors.forename: Massimo
authors.initials: M
authors.lastname: Corbo
authors.forename: Stefano F
authors.initials: SF
authors.lastname: Cappa
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000523:psychology*; D003071:Cognition; D003072:Cognition Disorders / Q000150:complications / Q000175:diagnosis* / Q000523:psychology; D032921:Consensus; D003704:Dementia / Q000150:complications / Q000175:diagnosis* / Q000523:psychology; D056344:Executive Function; D005260:Female; D006801:Humans; D008297:Male; D008569:Memory Disorders / Q000150:complications / Q000175:diagnosis* / Q000523:psychology; D008570:Memory, Short-Term; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications / Q000523:psychology*; D009483:Neuropsychological Tests; D012720:Severity of Illness Index