Affiliations: Department of Clinical Neurosciences and Division of
Neuroscience, San Raffaele Scientific Institute, Milan, Italy | Vita-Salute University, Via Olgettina, Milan,
Italy | NEuroMuscular Omnicentre – Fondazione Serena
Onlus, Niguarda Ca' Granda Hospital, Milan, Italy
Note: [] Corresponding author: Monica Consonni, PhD, Department of
Clinical Neurosciences, San Raffaele Turro Hospital, Via Stamira d'Ancona 20,
Milan 20127, Italy. Tel.: +39 02 2643 3305; Fax: +39 02 2643 3394 (5798);
E-mail: consonni.monica@hsr.it
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. 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.
Keywords: Amyotrophic Lateral Sclerosis, Lower Motor Neuron Disease, cognitive impairment, behavioural impairment, dysexecutive syndrome, motor disability
