Upper motor neuron dysfunction is associated with the presence of behavioural impairment in patients with amyotrophic lateral sclerosis.
Eur J Neurol
; 29(5): 1402-1409, 2022 05.
Article
en En
| MEDLINE
| ID: mdl-34989063
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.Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Esclerosis Amiotrófica Lateral
Tipo de estudio:
Diagnostic_studies
/
Observational_studies
/
Risk_factors_studies
Límite:
Humans
Idioma:
En
Revista:
Eur J Neurol
Asunto de la revista:
NEUROLOGIA
Año:
2022
Tipo del documento:
Article
País de afiliación:
Italia