Alessio Maranzano1,2, Barbara Poletti1, Federica Solca2, Silvia Torre1, Eleonora Colombo1,2, Matteo Faré3,4, Roberta Ferrucci5,6,7, Laura Carelli1, Federico Verde1,2, Claudia Morelli1, Vincenzo Silani1,2, Nicola Ticozzi1,2. 1. Department of Neurology, Istituto Auxologico Italiano IRCCS, Milan, Italy. 2. Department of Pathophysiology and Transplantation, 'Dino Ferrari' Center, Università degli Studi di Milano, Milan, Italy. 3. Department of Neurology, Ospedale San Gerardo ASST, Monza, Italy. 4. School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy. 5. 'Aldo Ravelli' Center for Neurotechnology and Experimental Brain Therapeutics, Department of Health Sciences, International Medical School, Università Degli Studi di Milano, Milan, Italy. 6. ASST Santi Paolo e Carlo, Neurology Clinic III, Milan, Italy. 7. Department of Neurology, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.
Abstract
BACKGROUND AND PURPOSE: Increasing evidence shows that approximately half of patients with amyotrophic lateral sclerosis (ALS) display cognitive (ALSci) or behavioural (ALSbi) impairment, or both (ALScbi). The aim of our study was to assess whether the burden of upper and lower motor neuron involvement is associated with the presence of cognitive and behavioural impairment. METHODS: A single-centre retrospective cohort of 110 Italian ALS patients was evaluated to assess correlations between motor and cognitive/behavioural phenotypes. Upper motor neuron regional involvement was measured with the Penn Upper Motor Neuron Score (PUMNS), whilst lower motor neuron signs were assessed using the Lower Motor Neuron Score. The Edinburgh Cognitive and Behavioural ALS Screen-Italian version and the Frontal Behaviour Inventory were administered to evaluate patients' cognitive and behavioural profiles. RESULTS: The PUMNS at first visit was significantly higher in behaviourally impaired ALS patients (ALSbi and ALScbi) compared to behaviourally unimpaired individuals (ALS and ALSci) (9.9 vs. 6.9, p = 0.014). Concerning the different Frontal Behaviour Inventory subdomains, higher PUMNS correlated with the presence of apathy, emotive indifference, inflexibility, inattention, perseveration and aggressiveness. CONCLUSION: To our knowledge, this is the first study showing that a clinical prominent upper motor neuron dysfunction is associated with a more significant behavioural impairment in ALS patients, suggesting the hypothesis of a preferential spreading of the pathology from the motor cortex to the ventromedial prefrontal and orbitofrontal cortex in this group of patients.
BACKGROUND AND PURPOSE: Increasing evidence shows that approximately half of patients with amyotrophic lateral sclerosis (ALS) display cognitive (ALSci) or behavioural (ALSbi) impairment, or both (ALScbi). The aim of our study was to assess whether the burden of upper and lower motor neuron involvement is associated with the presence of cognitive and behavioural impairment. METHODS: A single-centre retrospective cohort of 110 Italian ALS patients was evaluated to assess correlations between motor and cognitive/behavioural phenotypes. Upper motor neuron regional involvement was measured with the Penn Upper Motor Neuron Score (PUMNS), whilst lower motor neuron signs were assessed using the Lower Motor Neuron Score. The Edinburgh Cognitive and Behavioural ALS Screen-Italian version and the Frontal Behaviour Inventory were administered to evaluate patients' cognitive and behavioural profiles. RESULTS: The PUMNS at first visit was significantly higher in behaviourally impaired ALS patients (ALSbi and ALScbi) compared to behaviourally unimpaired individuals (ALS and ALSci) (9.9 vs. 6.9, p = 0.014). Concerning the different Frontal Behaviour Inventory subdomains, higher PUMNS correlated with the presence of apathy, emotive indifference, inflexibility, inattention, perseveration and aggressiveness. CONCLUSION: To our knowledge, this is the first study showing that a clinical prominent upper motor neuron dysfunction is associated with a more significant behavioural impairment in ALS patients, suggesting the hypothesis of a preferential spreading of the pathology from the motor cortex to the ventromedial prefrontal and orbitofrontal cortex in this group of patients.