Assessing behavioural changes in ALS: cross-validation of ALS-specific measures.

Area of Science:

• Neurology
• Behavioral Science

Background:

• Amyotrophic Lateral Sclerosis (ALS) is often accompanied by behavioral changes.
• Existing tools like the FrSBe lack ALS specificity.
• The Beaumont Behavioural Inventory (BBI) was developed as a proxy report for behavioral changes in ALS.

Purpose of the Study:

• To cross-validate the Beaumont Behavioural Inventory (BBI) against the ALS-Frontotemporal Degeneration Questionnaire (ALS-FTD-Q).
• To assess the construct validity, precision, sensitivity, specificity, and accuracy of the BBI in an ALS patient cohort.

Main Methods:

• Sixty ALS patients, including 8% with comorbid Frontotemporal Degeneration (FTD), were recruited.
• Patients were evaluated using both the BBI and the ALS-FTD-Q, administered by a carer.
• Correlational analysis, including precision, sensitivity, and specificity calculations, was performed.

Main Results:

• A significant large positive correlation (r=0.807, p<0.0001) was found between the BBI and ALS-FTD-Q scores.
• ALS-FTD patients scored significantly higher on the BBI than non-demented ALS patients (p<0.0001).
• Overall concordance was 72%, with adequate performance for severe impairment but lower for mild changes.

Conclusions:

• The BBI demonstrates good construct validity when compared to an ALS-specific tool (ALS-FTD-Q).
• The BBI is a more comprehensive behavioral assessment for ALS, capturing a wider spectrum of behavioral changes.
• The BBI shows higher sensitivity for mild behavioral changes potentially missed by the ALS-FTD-Q.