A neuropsychological and cognitive insight in amyotrophic lateral sclerosis / motor neuron disease

A neuropsychological and cognitive insight into motor neuron disease/amyotrophic lateral sclerosis (MND/ALS) 1) We evaluated cognitive functioning in 128 MND patients (vs 113 healthy controls), with a comprehensive neuropsychological battery. Dysfunctions were significantly present in 40% of cases (mainly for executive functioning and short-term memory); 7% of them showed clear frontal or fronto-temporal dementia (chapter 2). 2) Nine ALS patients (vs 10 healthy controls) underwent the fMRI study: significant differences were displayed in lateralized activation between patients and controls during the attentional task, both for unpleasant and for neutral word stimuli (statistically greater activation in right middle frontal gyrus for patients, greater activation for frontal, parietal and cerebellar areas in controls) and lower activation was shown in patients in the posterior cingulate during mnesic recall, for both unpleasant and neutral stimuli (chapter 3). 3) We validated an Italian version of a specific quality of life questionnaire for MND/ALS: the ALSAQ-40, psychometric reliability in terms of internal reliability, construct validity, test-retest reliability and face validity were evaluated on the basis of 76 patients’ responses. Correlation with functional and clinical measures are discussed in the text (chapter 4). 5) Similarly, we validated an Italian version of the ELQ, (a questionnaire for detecting emotional lability) on 41 MND patients and 39 respective caregivers (vs 39 healthy controls and 39 pseudo-caregivers). The lack of correlation with neuropsychological profile and correlations with psychopathological indices, both in patients and in caregivers, are described in the text (chapter 5). 5) The Rorschach test (according to Exner’s guidelines) was administred to 21 ALS patients with early onset, 21 with onset longer than two years and 21 myasthenic controls. Among the numerous findings obtained, suicidal ideation was significantly more present in the ALS group with a recent diagnosis compared to those with a more remote one (chapter 6).