ABSTRACT
The Amyotrophic Lateral Sclerosis (ALS) is a multisystemic disorder. It is considered a neuromuscular disease but also involves cognitive (executive functions, social cognition, attention, memory and language), emotional or behavior changes in over 50% of the reported cases and to of frontotemporal diagnosis lobar degeneration of behavioral variant in up to 15% of the cases. For this reason, the presence of cognitive and motivational problems was analysed in a Spanish sample of ALS patients through the prefrontal symptoms inventory (PSI) to determine applicability in this disease STI. A sample of 31 patients with a potential ALS or definitive diagnostic criteria according to El Escorial was used. Obtained results ISP were compared with a sample of 31 healthy people in the same proportions of gender, age and education level. Obtained results showed a not significant difference between the two populations in the motivational factor problems, related to the depression symptomatology frequently associated with ALS. A significant positive correlation between age at diagnosis and the scale of the motivational problems was observed, with a not significant trend related to problems in the executive control and in social the behavior control and with the age at diagnosis, in the same sense with age at diagnosis. Therefore, it can be concluded that, despite the results obtained, emotional and behavioral deficits in ALS patients and symptoms related to frontotemporal dementia (among others, anosognosia or lack of consciousness symptoms) could have interfered in patient perception about their symptomatology