Diagnostic utility of the amyotrophic lateral sclerosis Functional Rating Scale—Revised to detect pharyngeal dysphagia in individuals with amyotrophic lateral sclerosis

Abstract

The ALS Functional Rating Scale-Revised (ALSFRS-R) is the most commonly utilized instrument to index bulbar function in both clinical and research settings. We therefore aimed to evaluate the diagnostic utility of the ALSFRS-R bulbar subscale and swallowing item to detect radiographically confirmed impairments in swallowing safety (penetration or aspiration) and global pharyngeal swallowing function in individuals with ALS. Methods Two-hundred and one individuals with ALS completed the ALSFRS-R and the gold standard videofluoroscopic swallowing exam (VFSE). Validated outcomes including the Penetration-Aspiration Scale (PAS) and Dynamic Imaging Grade of Swallowing Toxicity (DIGEST) were assessed in duplicate by independent and blinded raters. Receiver operator characteristic curve analyses were performed to assess accuracy of the ALSFRS-R bulbar subscale and swallowing item to detect radiographically confirmed unsafe swallowing (PAS > 3) and global pharyngeal dysphagia (DIGEST >1). Results Although below acceptable screening tool criterion, a score of � 3 on the ALSFRS-R swallowing item optimized classification accuracy to detect global pharyngeal dysphagia (

Cardiac Autonomic Modulation in Subjects with Amyotrophic Lateral Sclerosis (ALS) during an Upper Limb Virtual Reality Task: A Prospective Control Trial

“…For the participants' clinical features, three scales referring to functional assessment, fatigue, and quality of life were necessary: As a functional assessment tool, the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) was enforced, validated in Brazilian individuals with ALS which permits monitoring of symptoms and limitations of daily living activities. The scale has 12 questions, with scores ranging between 0 and 4 and a maximum score of 48 (where the participant is in his or her optimum state) [ 23 – 26 ] To assess fatigue during the task implementation, we enforced the Fatigue Severity Scale (FSS). The FSS contains nine declarations, and for each item, subjects are instructed to choose a score ranging from 1 to 7, with 7 representing the highest level of agreement with a given statement.…”

“…As a functional assessment tool, the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) was enforced, validated in Brazilian individuals with ALS which permits monitoring of symptoms and limitations of daily living activities. The scale has 12 questions, with scores ranging between 0 and 4 and a maximum score of 48 (where the participant is in his or her optimum state) [ 23 – 26 ]…”

Qualitative measures that assess functional disability and quality of life in ALS

“…Early detection and consistent monitoring of dysphagia provides the opportunity to improve survival and QOL with timely interventions. The ALSFRS-R has reported poor discrimination in patients with milder disability for the items, “speech”, “salivation”, and “swallowing” on the bulbar domain [ 19 ] as well as inadequate diagnostic accuracy of the swallowing item to detect radiographically confirmed swallowing impairment, suggesting the need for alternate measures to assess dysphagia in ALS [ 44 ].…”

Gastrointestinal motility disorders in neurologic disease

“…Physical examination reveals cranial nerve palsies, muscle fasciculations, and an exaggerated jaw jerk (pseudobulbar palsy). Videofluoroscopic swallow tests with liquids and solids are superior to a symptom-based functional rating scale to evaluate swallowing (48), assess aspiration, and guide nutritional support (oral or percutaneous gastrostomy; ref. 49).…”