Concordant Validity of a Digital Peak Cough Flow Meter to Assess Voluntary Cough Strength in Individuals with ALS.

Peak cough flow represents an important metric directly related to the physiologic ability of an individual to defend the airway or expel tracheal aspirate. Given the high prevalence of dysphagia and dystussia in individuals with amyotrophic lateral sclerosis (ALS) and recent findings that the expiratory phase of voluntary cough is significantly impaired in ALS individuals, we aimed to determine the reproducibility of an affordable, portable peak cough flow (PCF) meter for the assessment of cough production in individuals with ALS. 109 individuals with ALS completed voluntary cough testing using both the research cough spirometry equipment and a digital peak cough flow meter. Maximum peak expiratory cough flow rates were obtained from each device. Analyses included paired t test, Pearson's correlation, and Lin's concordance correlation to determine the degree of agreement and reproducibility between cough measurement devices (alpha = 0.05). Mean differences between peak cough flow test values (L/min) across instruments were not statistically significant (mean difference = - 2.93; 95% CI - 18.67, 12.82; p = 0.713). PCF values obtained from the digital peak cough flow meter and the research cough spirometry equipment were strongly associated (r = 0.826, p < 0.000) and demonstrated a high level of agreement and reproducibility (ρc = 0.824, 95% CI 0.754, 0.876). These data validate the use of an inexpensive and portable digital peak cough flow device to index peak cough flow strength in individuals with ALS. This assessment could easily be incorporated into a multidisciplinary ALS clinical setting to index the physiologic ability of an individual to protect the airway.

Oropharyngeal dysphagia profiles in individuals with oculopharyngeal muscular dystrophy.

BACKGROUND: Although dysphagia represents a hallmark manifestation of oculopharyngeal muscular dystrophy (OPMD), limited knowledge exists regarding the underlying nature of oropharyngeal swallowing impairments in this patient population. We aimed to delineate global pharyngeal dysphagia profiles in OPMD and identify the prevalence and physiologic associations of impairments in swallowing safety and efficiency. METHODS: Twenty-two individuals with OPMD completed a videofluoroscopic swallowing evaluation. Blinded raters completed validated scales of global dysphagia (dynamic imaging grade of swallowing toxicity, DIGEST), efficiency (normalized residue ratio scale, NRRS), and safety (penetration-aspiration scale, PAS). Degree of laryngeal vestibule closure and aspiration events were described. Descriptives and chi-squared analyses were conducted with alpha set at P < .05. KEY RESULTS: One hundred and thirty-four swallowing trials were analyzed. DIGEST scores revealed that 96% (n = 21) of participants demonstrated pharyngeal dysphagia (score >1). Presence of a cricopharyngeal bar was noted in 10 individuals. The predominant swallowing categorization across patients was safe and inefficient (51%) followed by unsafe and inefficient (32%). 77.3% demonstrated vallecular residue (NRRSv>0.07) and 90.1% piriform sinus residue (NRRSp > .20). 33% (n = 54) of swallows were unsafe (PAS>3) with 45 episodes of penetration and 9 episodes of aspiration. Aspiration occurred during the swallow in 100% of identified occurrences. Incomplete epiglottic inversion was associated with airway compromise and postswallow residue (P < .05). CONCLUSIONS & INFERENCES: These findings highlight the high prevalence of oropharyngeal swallowing impairments in both swallowing efficiency and safety. A high proportion of physiologic impairments in epiglottic inversion and laryngeal vestibule closure were noted that related to functional impairments in swallow safety and inefficiency.

Discriminant ability of the Eating Assessment Tool-10 to detect aspiration in individuals with amyotrophic lateral sclerosis.

BACKGROUND: Oropharyngeal dysphagia is prevalent in individuals with amyotrophic lateral sclerosis (ALS) leading to malnutrition, aspiration pneumonia, and death. These factors necessitate early detection of at-risk patients to prolong maintenance of safe oral intake and pulmonary function. This study aimed to evaluate the discriminant ability of the Eating Assessment Tool (EAT-10) to identify ALS patients with unsafe airway protection during swallowing. METHODS: Seventy ALS patients completed the EAT-10 survey and underwent a standardized videofluoroscopic evaluation of swallowing. Two blinded raters determined airway safety using the Penetration Aspiration Scale (PAS). A between groups anova (safe vs penetrators vs aspirators) was conducted and sensitivity, specificity, area under the curve (AUC), and likelihood ratios calculated. KEY RESULTS: Mean EAT-10 scores for safe swallowers, penetrators, and aspirators (SEM) were: 4.28 (0.79) vs 7.10 (1.79) vs 20.50 (3.19), respectively, with significant differences noted for aspirators vs safe swallowers and aspirators vs penetrators (p < 0.001). The EAT-10 demonstrated good discriminant ability to accurately identify ALS penetrator/aspirators (PAS ≥3) with a cut off score of 3 (AUC: 0.77, sensitivity: 88%, specificity: 57%). The EAT-10 demonstrated excellent accuracy at identifying aspirators (PAS ≥6) utilizing a cut off score of 8 (AUC: 0.88, sensitivity: 86%, specificity: 72%, likelihood ratio: 3.1, negative predictive value: 95.5%). CONCLUSIONS & INFERENCES: The EAT-10 differentiated safe vs unsafe swallowing in ALS patients. This patient self-report scale could represent a quick and meaningful aide to dysphagia screening in busy ALS clinics for the identification and referral of dysphagic patients for further instrumental evaluation.