Screening for cognition and behaviour changes in ALS.

Area of Science:

• Neurology
• Neuropsychology
• Clinical Assessment

Background:

• Amyotrophic Lateral Sclerosis (ALS) often presents with cognitive and behavioral impairments.
• Existing screening tools may not be suitable for ALS patients with physical disabilities.
• A specific assessment is needed to differentiate ALS-related changes from other neurological disorders.

Purpose of the Study:

• To introduce the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) for assessing cognitive and behavioral changes in ALS.
• To validate the ECAS in a cohort of ALS patients and healthy controls.
• To establish the utility of ECAS in differentiating ALS-specific cognitive profiles.

Main Methods:

• Development of the ECAS, a 15-20 minute screen with ALS-Specific, ALS Non-specific, and carer behavior components.
• Recruitment of 48 ALS patients (without dementia), 40 healthy controls, and 20 carers.
• Establishment of abnormality cut-offs using data from healthy controls.

Main Results:

• Abnormality cut-offs established: 77/100 (ALS-Specific), 24/36 (ALS Non-specific), 105/136 (ECAS Total).
• 29% of ALS patients exhibited abnormal ALS-Specific scores; 6% had abnormal ALS Non-specific scores.
• Most common deficits: language (35%), executive functions and fluency (23% each). 40% of carers reported behavioral changes, 15% met criteria for possible frontotemporal dementia (FTD).

Conclusions:

• The ECAS is an effective in-clinic tool for assessing cognitive and behavioral changes in ALS patients with physical limitations.
• ECAS aids in determining the presence, severity, and type of cognitive/behavioral symptoms.
• This assessment is a crucial first step in managing neurological symptoms in ALS.