Clinical Measures of Bulbar Dysfunction in ALS

Area of Science:

• Neurology
• Speech-Language Pathology

Background:

• Bulbar impairment is a critical feature of Amyotrophic Lateral Sclerosis (ALS), significantly affecting patient survival and quality of life.
• Dysfunction in speech and swallowing are primary contributors to ALS clinical heterogeneity, necessitating timely interventions.

Purpose of the Study:

• To comprehensively review and appraise commonly used clinical tools for assessing and monitoring bulbar dysfunction in ALS.
• To evaluate these instruments using the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) framework.

Main Methods:

• Systematic review of clinical assessment tools for bulbar dysfunction in ALS.
• Evaluation based on the COSMIN framework to assess measurement properties.

Main Results:

• A wide array of assessment tools exists, but significant geographical variations in practice and individual instrument limitations were identified.
• Current assessment practices reveal gaps in systematic evaluation and integrated protocols.

Conclusions:

• Optimization of existing and development of new assessment tools are crucial for both clinical and research applications in ALS.
• Multicenter validation and standardization of bulbar assessment instruments are essential for developing clinical guidelines and best practice recommendations.