Behavioural changes predict poorer survival in amyotrophic lateral sclerosis

Area of Science:

• Neurology
• Neuroscience

Background:

• Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons.
• Behavioral and cognitive dysfunctions are increasingly recognized in ALS patients.
• Prognostic tools are crucial for managing ALS patient care.

Purpose of the Study:

• To evaluate behavioral impairment using the Motor Neuron Disease Behavioural Scale (MiND-B) in ALS patients.
• To assess cognitive dysfunction using the Addenbrooke's Cognitive Examination (ACE) in ALS patients.
• To determine the prognostic implications of these impairments on survival.

Main Methods:

• Prospective recruitment of 134 ALS patients from a specialized multidisciplinary clinic.
• Behavioral assessment using the Motor Neuron Disease Behavioural Scale (MiND-B).
• Cognitive evaluation using the Addenbrooke's Cognitive Examination (ACE).
• Survival analysis using Kaplan-Meier and Cox regression models.

Main Results:

• 59% of ALS patients exhibited behavioral dysfunction (MiND-B), associated with significantly shorter survival (HR 2.53, p=0.003).
• A 1-point decrease in MiND-B score correlated with a 3% increase in mortality risk.
• 33% of patients showed cognitive dysfunction (ACE), linked to significantly shorter survival (HR 2.0, p=0.042).

Conclusions:

• Behavioral and cognitive impairments are significant predictors of poor survival in ALS.
• The MiND-B and ACE are efficient clinical tools for prognostication in ALS.
• These assessments can aid multidisciplinary ALS clinics in patient management.