APPENDIX SEVEN
Bulbar Tests

Careful analysis of proper tests for management, clinical studies and treatment trials is recommended. In addition to precise bulbar measures, special attention should be given to measurement of early metabolic indicators of dehydration and malnutrition due to dysphagia, including urine osmolarity, serum pre-albumin, nitrogen balance and body mass index. The placement of a percutaneous endoscopic gastrostomy [PEG] or gastrostomy tube [GT] may be employed as a time-to-failure end point in clinical trials. Criteria for the proper timing of PEG or GT placement require further definition and development.

Advancing the evidence-based foundation of symptomatic treatment of bulbar dysfunction requires clear definition of clinical trial endpoints and trial design. Measurement tools could include bulbar and pseudobulbar affect clinimetric scales with attention to confounding effects of depression.

Use of specific adaptive equipment, such as communication aids, could be evaluated with quality of life measurement instruments.

Preferred: Precise measurement of bulbar function to be developed

Validated: Bulbar functional rating scales

Frenchay Scale

Hillel Scale

Norris ALS Scale Bulbar Subscale

Timed speech tests

Presently, phonetic feature analysis, videofluoroscopy, and orofacial strength measurement with bulbar force transducers are acceptable for small, single center studies.

Future Research: Further tailoring of established bulbar functional rating scales to address the special problems of ALS is identified as a clinical research priority. Timed speech tests should be further validated. Collaborative efforts with speech therapists and nutritionists are recommended.

In addition to developing valid tests, newer technologies need to be evaluated that may allow for monitoring the clinical course and effect of treatment.

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