TY - JOUR
T1 - The cognitive and behavioural profile of Amyotrophic Lateral Sclerosis
T2 - Application of the consensus criteria
AU - Consonni, Monica
AU - Iannaccone, Sandro
AU - Cerami, Chiara
AU - Frasson, Paola
AU - Lacerenza, Marco
AU - Lunetta, Christian
AU - Corbo, Massimo
AU - Cappa, Stefano F.
PY - 2013
Y1 - 2013
N2 - 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.
AB - 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.
KW - Amyotrophic Lateral Sclerosis
KW - behavioural impairment
KW - cognitive impairment
KW - dysexecutive syndrome
KW - Lower Motor Neuron Disease
KW - motor disability
UR - http://www.scopus.com/inward/record.url?scp=84882424750&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84882424750&partnerID=8YFLogxK
U2 - 10.3233/BEN-2012-110202
DO - 10.3233/BEN-2012-110202
M3 - Article
C2 - 23001631
AN - SCOPUS:84882424750
SN - 0953-4180
VL - 27
SP - 143
EP - 153
JO - Behavioural Neurology
JF - Behavioural Neurology
IS - 2
ER -