Matteo Faré (Italy)
San Gerardo Hospital ASST Monza Italy Department of NeurologyAuthor Of 1 Presentation
PROMINENT UPPER MOTOR NEURON DYSFUNCTION CORRELATES WITH A MORE SIGNIFICANT BEHAVIORAL IMPAIRMENT IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
- Alessio Maranzano (Italy)
Abstract
Background and Aims:
Increasing evidence indicates that up to 50 % of patients affected by amyotrophic lateral sclerosis (ALS) displays cognitive (ALSci) or behavioral (ALSbi) impairment, or both (ALScbi). The aim of our study is to assess whether the burden of upper (UMN) and lower motor neuron (LMN) involvement is associated to the presence of cognitive and behavioral impairment.
Methods:
A retrospective cohort of 110 Italian ALS patients has been evaluated to assess correlations between motor and cognitive/behavioral phenotypes. UMN regional involvement was measured with the Penn Upper Motor Neuron Score (PUMNS), while LMN signs were assessed using the Lower Motor Neuron Score (LMNS). The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) - Italian version and Frontal Behavior Inventory (FBI) were used to perform an evaluation of both cognitive and behavioral profile.
Results:
PUMNS scores at first visit were significantly higher in behaviorally impaired patients (ALSbi and ALScbi) compared to unimpaired individuals (ALS and ALSci) (9.90 vs 6.97, p=0.014). With regard to the different FBI subdomains, higher PUMNS scores correlated with the presence of Apathy, Emotive Indifference, Inflexibility, Inattention, Perseveration and Aggressiveness.
Conclusions:
To our knowledge, this is the first study suggesting that a prominent UMN dysfunction is associated with a more significant behavioral impairment in ALS patients, rising 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