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.
Highlights
Amyotrophic Lateral Sclerosis (ALS) is a multisystem disorder, in which the core pathology is the degeneration of motor neurons, giving rise to progressive and diffuse muscle wasting, weakness and spasticity
Based on clinical evaluations performed by two expert neurologists (S.I. and C.C.), 3 ALS patients were found to meet the criteria for dementia
As for the mean percentage of pathological neuropsychological scores, we found that cognitively normal ALS, ALSci/ALS cases satisfied criteria for behavioural impairment (ALSbi), Lower Motor Neuron Disease (LMND) and control groups differed in the domains of executive function (X3 = 27.185, p < 0.001), and language (X3 = 9.136, p = 0.028)
Summary
Amyotrophic Lateral Sclerosis (ALS) is a multisystem disorder, in which the core pathology is the degeneration of motor neurons, giving rise to progressive and diffuse muscle wasting, weakness and spasticity. A spectrum of cognitive and/or behavioural dysfunctions constitutes the non-motor manifestations of ALS. A subset of patients, ranging from 1 to 40% [1,2,3,4] fulfils the criteria for dementia, the cognitive impairment in ALS is most commonly characterised by a mild or moderate dysfunction in the domain of executive functions (e.g. word generation, problem solving, attentional control and reasoning). The estimated prevalence of cognitive dysfunction ranges from 10% to 75% [1,3,6,7,8]. This wide variation can be attributed to the selection of patients and
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