Cognitive and Behavioral Rehabilitation in ALS, PMA and PLS

Summary of research:

Amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA), and primary lateral sclerosis (PLS) are characterized by motor impairments, such as respiratory issues, trouble walking and swallowing -and speech difficulties. However, up to 50% of patients also experience cognitive and behavioral symptoms, of which 5%-10% have ALS-frontotemporal dementia (FTD). These symptoms negatively impact patients and family carers. However, they are often under-appreciated, as motor impairments receive greater clinical attention. Dutch ALS guidelines recommend psycho-education and support in the case of these symptoms, but do not specify how to monitor symptoms or tailor interventions. As a result, cognitive and behavioral symptoms are not consistently monitored, and interventions are not well integrated into clinical practice for ALS patients.

Our aim is to improve management of cognitive and behavioral dysfunction in ALS, PMA, and PLS. We will first evaluate approaches for monitoring cognitive and behavioral changes and synthesize evidence on the effectiveness of interventions. This will be achieved through a systematic literature review, and a survey among Dutch and European ALS health care professionals. Subsequently, we will develop and implement a toolbox of targeted interventions and formulate recommendations for monitoring. Finally, we will evaluate the acceptability, adherence and experience of the toolbox in clinical practice.

The toolbox will be integrated into clinical practice. Healthcare professionals will receive guidance in how to monitor cognitive and behavioral changes, and to provide cognitive rehabilitation. By improving diagnosis and management of these symptoms, this project aims to enhance the quality of life for patients and carers.

Throughout the project, a working group consisting of patients, family carers and health care professionals is involved. The working group will actively contribute to all stages of the project, from preparation through execution to implementation.