Abstract Details

To develop a valid scale that reliably and accurately identifies symptoms of bulbar dysfunction and tracks the progression of motor neuron impairment over time.

The purpose of the C-BAS is to provide an instrument for comprehensive assessment of bulbar function in patients with ALS, with the broader goal of establishing reliable data collection for clinical and research applications. This measure represents a compilation of evidence based clinical components  designed to facilitate early recognition and track rate and functional impacts of bulbar motor neuron disease, accurately identifying pre-symptomatic impairments to allow for timely application of clinical interventions. The C-BAS may therefore prove useful for documenting early bulbar impairment and successively monitoring ALS disease progression.

The C-BAS represents a collection of subjective and objective bulbar measures designed to identify speech and swallowing impairments, while tracking ALS progression. This scale employs quantitative measurement of speech and swallowing, including diadochokinetic rates, maximum phonation time, speaking rate, swallowing rate, sentence intelligibility, the S/Z ratio, reading/pause rate, swallowing tasks, UMN and LMN features and patient reported outcome measures.

Cumulative data collection will be presented, which will include:

1.      Content survey acquired validation data on individual C-BAS items.

2.      Results from an ALS clinical care C-BAS implementation study (n=20+ individuals).

3.      Comparison of C-BAS scores with existing bulbar scales (e.g., ALSFRS bulbar subscale, CNS-BFS), in order to achieve validation.

The C-BAS incorporates a novel means of systematic data collection for assessing early and longitudinal bulbar symptoms. Unlike existing ALS scales, C-BAS is constructed purely for assessment of bulbar dysfunction in a clinical setting. The designed focus is to evaluate impairment of body structures and function, activity limitation, and participation restrictions not routinely measured through existing ALS instruments. Once validation has been established, this scale should provide a standardized, comprehensive measure targeting bulbar features in ALS.