Relationship between pulmonary, cough, and swallowing functions in individuals with amyotrophic lateral sclerosis.

INTRODUCTION/AIMS: Evaluations of pulmonary, cough, and swallow function are frequently performed to assess disease progression in amyotrophic lateral sclerosis (ALS), yet the relationship between these functions remains unknown. We therefore aimed to determine relationships between these measures in individuals with ALS. METHODS: One hundred individuals with ALS underwent standardized tests: forced vital capacity (FVC), maximum expiratory/inspiratory pressure (MEP, MIP), voluntary cough peak expiratory flow (PEF), and videofluoroscopic swallow evaluation (VF). Duplicate raters completed independent, blinded ratings using the Dynamic Imaging Grade of Swallowing Toxicity (DIGEST) scale. Descriptives, Spearman's Rho correlations, Kruskal-Wallis analyses, and Pearson's chi-squared tests were completed. RESULTS: Mean and standard deviation across pulmonary and cough measures were FVC: 74.2% predicted (± 22.6), MEP: 91.6 cmH2O (± 46.4), MIP cmH2O: 61.1 (± 28.9), voluntary PEF: 352.7 L/min (± 141.6). DIGEST grades included: 0 (normal swallowing): 31%, 1 (mild dysphagia): 48%, 2 (moderate dysphagia): 10%, 3 (severe dysphagia): 10%, and 4 (life-threatening dysphagia): 1%. Positive correlations were observed: MEP-MIP: r = .76, MIP-PEF: r = .68, MEP-PEF: r = .61, MIP-FVC: r = .60, PEF-FVC: r = .49, and MEP-FVC: r = .46, p < .0001. MEP (p = .009) and PEF (p = .04) differed across DIGEST safety grades. Post hoc analyses revealed significant between group differences in MEP and PEF across DIGEST safety grades 0 versus 1 and grades 0 versus 3, (p < .05). DISCUSSION: In this cohort of individuals with ALS, pulmonary function, and voluntary cough were associated. Expiratory metrics (MEP, PEF) were diminished in individuals with unsafe swallowing, increasing their risk for effectively defending the airway.

Sensitivity and specificity of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised to detect dysarthria in individuals with amyotrophic lateral sclerosis.

INTRODUCTION/AIMS: Given the widespread use of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) to measure disease progression in ALS and recent reports demonstrating its poor sensitivity, we aimed to determine the sensitivity and specificity of the ALSFRS-R bulbar subscale and speech item to detect validated clinical ratings of dysarthria in individuals with ALS. METHODS: Paired ALSFRS-R and validated Speech Intelligibility Test (SIT) data from individuals with ALS were analyzed. Trained raters completed duplicate, independent, and blinded ratings of audio recordings to obtain speech intelligibility (%) and speaking rate (words per minute, WPM). Binary dysarthria profiles were derived (dysarthria ≤96% intelligible and/or <150 WPM). Data were obtained using the Kruskal-Wallis test, receiver-operating characteristic (ROC) curve, area under the curve (AUC), sensitivity and specificity percentages, and positive/negative predictive values (PPV/NPV). RESULTS: A total of 250 paired SIT and ALSFRS-R data points were analyzed. Dysarthria was confirmed in 72.4% (n = 181). Dysarthric speakers demonstrated lower ALSFRS-R bulbar subscale (8.9 vs. 11.2) and speech item (2.7 vs. 3.7) scores (P < .0001). The ALSFRS-R bulbar subscale score had an AUC of 0.81 (95% confidence interval [CI] 0.75 to 0.86). A subscale score of ≤11 yielded a sensitivity of 86%, specificity of 57%, PPV of 84%, and NPV of 60% to correctly identify dysarthria status. The ALSFRS-R speech item score demonstrated an AUC of 0.81 to detect dysarthria (95% CI 0.76 to 0.85), with sensitivity of 79%, specificity of 75%, PPV of 89%, and NPV of 58% for a speech item cutpoint of ≤3. DISCUSSION: The ALSFRS-R bulbar and speech item subscale scores may be useful, inexpensive, and quick tools for monitoring dysarthria status in ALS.

Functional Lingual Pressure Thresholds for Swallowing Safety and Efficiency Impairments in Amyotrophic Lateral Sclerosis.

Although reductions in lingual strength are reported in individuals with amyotrophic lateral sclerosis (ALS) that are associated with dysphagia; determination of a functional lingual pressure threshold (FLPT) has not yet been established. The present study therefore sought to identify an FLPT for impaired swallowing safety and efficiency in individuals with ALS.Thirty individuals with ALS completed a standardized videofluoroscopic swallowing examination and maximum anterior isometric lingual pressure testing using the Iowa Oral Performance Instrument. Duplicate, blinded ratings of the validated Penetration-Aspiration Scale (PAS) scores and Analysis of Swallowing Physiology: Events, Kinematics and Timing (ASPEKT) were performed. Binary classifications of safety (unsafe: PAS: ≥ 3) and efficiency (inefficient: ≥ 3% worst total pharyngeal residue) were derived. Descriptives and receiver operating characteristic curve analyses (AUC, sensitivity, specificity) were performed.Unsafe and inefficient swallowing were instrumentally confirmed in 57% and 70% of ALS patients, respectively. Across the entire cohort, the mean maximum lingual physiologic capacity was 32.1 kilopascals ('kPa'; SD: 18.1 kPa). The identified FLPT for radiographically confirmed unsafe swallowing was 43 kPa (sensitivity: 94%, specificity: 62%, AUC 0.82, p = 0.003). FLPT for inefficient swallowing was 46 kPa (sensitivity: 86%, specificity: 56%, AUC = 0.77, p = 0.02).These data provide preliminary FLPT data in a small cohort of individuals with ALS that need to be further investigated in larger cohorts to inform clinical screening practices.

Predictors of Peak Expiratory Cough Flow in Individuals with Amyotrophic Lateral Sclerosis.

Dystussia is prevalent in individuals with amyotrophic lateral sclerosis (ALS), leading to a diminished physiologic capacity to effectively defend the airway. We aimed to identify predictors of peak expiratory cough flow rate in individuals with ALS. One hundred and thirty-four individuals with a confirmed diagnosis of ALS (El-Escorial criteria revised) completed the ALS Functional Rating Scale-Revised (ALSFRS-R) and underwent pulmonary function and cough spirometry testing. Pearson's correlation coefficients and hierarchical multiple regression modeling were conducted to determine predictors of voluntary cough peak expiratory flow rate (p < 0.05). The full model including age, bulbar disease, cough spirometry metrics, and respiratory parameters had a marginal R2 = 0.635, F (7, 126) = 30.241, p < 0.0005, adjusted R2 = 0.61. Maximum expiratory pressure, compression phase, and vital capacity did not contribute and were therefore removed (p < 0.05). The most parsimonious predictive model included age, bulbar disease, peak inspiratory flow rate and duration, peak expiratory rise time, and inspiratory pressure generation with a marginal R2 = 0.543. Although expiratory pressure generation has historically served as the therapeutic target to improve dystussia in ALS, the current dataset highlighted that the inability to quickly and forcefully inspire during the inspiratory phase of voluntary cough places patients at a mechanical disadvantage to generate subsequent high-velocity expiratory airflow to clear the airway. Thus, therapeutic training programs that include both inspiratory and expiratory strength targets may optimize airway clearance capacity in this challenging patient population.

Swallowing Safety and Efficiency Impairment Profiles in Individuals with Amyotrophic Lateral Sclerosis.

Dysphagia is common in individuals with amyotrophic lateral sclerosis (ALS) and associated with reductions in quality of life and health-related outcomes. Despite the high prevalence of dysphagia in ALS, functional impairment profiles of swallowing safety and efficiency have not been comprehensively examined. We therefore aimed to determine the relative prevalence of unsafe and inefficient swallowing in a large cohort of individuals with ALS. We further sought to examine the impact of global and bulbar disease progression (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised "ALSFRS-R" total and bulbar scores, respectively), disease duration, and onset type on swallowing impairment profiles. One hundred individuals with ALS completed a standardized videofluoroscopic swallowing examination Two independent and blinded raters performed validated ratings of safety (Penetration-Aspiration Scale, PAS) and efficiency (Analysis of Swallowing Physiology: Events, Kinematics, and Timing, ASPEKT % residue). Binary classifications of safety (unsafe: PAS ≥ 3), efficiency (inefficient: total residue ≥ 3% of C2-42) and global swallowing status were derived. The ALSFRS-R was completed to derive ALSFRS-R total and bulbar subscale scores. Demographic data (disease duration and onset type) for each participant was also recorded.  Descriptives, 2 × 2 contingency tables with Fishers exact test, and independent samples t-tests were performed (α = 0.05). Prevalence of unsafe and inefficient swallowing was 48% and 73%, respectively. Global swallowing profiles were, in rank order: unsafe and inefficient (39%), inefficient but safe (34%), safe and efficient (18%), and unsafe but efficient (9%). There were no differences in global disease progression or disease duration across swallowing impairment profiles. ALSFRS-R bulbar subscale scores were significantly lower in unsafe versus safe swallowers, p < 0.05. Spinal onset patients had a greater proportion of safe swallowers as compared to bulbar onset patients (p = 0.000, Fisher's exact test). Both spinal and bulbar onset patients demonstrated a higher prevalence of inefficient swallowers as compared to efficient swallowers (p = 0.04, Fisher's exact test). Dysphagia was prevalent in this group of individuals with ALS. Approximately half demonstrated safety impairments and two-thirds had impairments in swallowing efficiency. Inefficient swallowing was approximately four times more likely to be the initial functional impairment in patients with one pathophysiologic functional impairment. A longitudinal study is needed to examine the temporal evolution of dysphagia in ALS.

Discriminant Ability of the 3-Ounce Water Swallow Test to Detect Aspiration in Acute Postoperative Cardiac Surgical Patients.

Dysphagia is a common complication of cardiac surgery (CS) contributing to morbidity and mortality. Although early dysphagia detection is important, no current screening guidelines or validated tools exist in the cardiac intensive care setting. We therefore aimed to examine the discriminant ability of the 3-ounce water swallow test (3 oz. WST) to detect aspiration in acute postoperative CS patients. 196 postoperative CS patients were enrolled in this prospective single-center study. Participants completed the 3 oz. WST and a standardized Flexible Endoscopic Evaluation of Swallowing. Independent duplicate ratings of the penetration aspiration scale (PAS) were performed in a blinded fashion (100% agreement criteria). Receiver operating characteristic curve and area under the curve (AUC) analyses were performed with sensitivity, specificity, positive, and negative predictive values (PPV, NPV) derived. Fifty-four CS patients (28%) were confirmed aspirators (PAS ≥ 6), of whom 48% (n = 26) were silent aspirators (PAS = 8). Both the sensitivity and specificity of the 3 oz. WST to identify instrumentally confirmed aspiration was 63% (AUC: 0.63, 95% CI: 0.54, 0.72), and PPV was 39% and NPV 82%. The 3 oz. WST demonstrated fair discriminant ability to detect aspiration in acute postoperative CS patients. The high rate of silent aspiration may explain, in part, these findings given that the screening fail criteria include an overt cough response. In isolation, the 3 oz. WST does not represent a sensitive screen of aspiration in postoperative CS patients with a need to identify alternative screening tools for this setting.

Reflexive Airway Sensorimotor Responses in Individuals with Amyotrophic Lateral Sclerosis.

BACKGROUND: Progressive motor denervation in amyotrophic lateral sclerosis (ALS) leads to reduced expiratory cough flow and diminished airway clearance physiologic capacity. Although ALS is thought to primarily impact motor systems, preliminary data from our laboratory suggest degradation of afferent pathways that regulate reflexive cough responses to radiographically confirmed aspiration. We, therefore, aimed to delineate both sensory and motor responses to a tussigenic airway irritant in individuals with ALS compared to healthy controls. METHODS: Thirty-two individuals with ALS and 34 healthy age and gender-matched controls completed reflex cough testing. Capsaicin stimuli (0, 50, 100, 150, 200 µM) were presented in a randomized three-block design and motor (cough spirometry metrics) and sensory (patient-rated urge to cough, UtC) ratings collected. ALS patients underwent videofluoroscopy with penetration-aspiration ratings completed. Descriptives, Mann-Whitney U, and mixed models ANOVAs were performed. RESULTS: Sensory: Individuals with ALS demonstrated greater UtC sensitivity slopes (i.e., increased stimulus sensitivity) vs. healthy controls (p = 0.036). Within the ALS group, however, silent aspirators (PAS = 8) demonstrated blunted UtC sensitivity slopes compared to ALS patients who did not (PAS ≤ 7, p = 0.0001). Motor: Compared to healthy controls, ALS individuals demonstrated reduced peak expiratory flow rates (p = 0.004), longer peak expiratory rise time (p = 0.017), and lower cough volume acceleration (p = 0.000). CONCLUSIONS: ALS individuals demonstrated increased sensitivity to an upper airway irritant; however, they demonstrated slower and weaker expiratory cough motor output compared to healthy controls. In ALS silent aspirators, blunted sensorimotor responses were observed, suggesting that sensory degradation may occur at the final or most severe stage of bulbar disease progression.

Biomechanical Biomarkers of Tongue Impairment During Swallowing in Persons Diagnosed with Amyotrophic Lateral Sclerosis.

BACKGROUND: The impact of tongue dysfunction on deglutition in persons diagnosed with amyotrophic lateral sclerosis (ALS) is not well understood. This information is needed to improve our understanding of the mechanisms of swallowing impairment, for identifying risk factors of dysphagia, and for establishing impairment-specific treatments aimed at slowing the loss of swallow function. OBJECTIVES: The goals of this study were to determine the relation between biomechanical measures of oral tongue movements using electromagnetic articulography (EMA) and measures of swallow physiology, swallow safety and efficiency, and self-reported swallowing function. METHODS: Participants were diagnosed with ALS by a neurologist following the El Escorial Criteria from the World Federation of Neurology. Twelve participants underwent (1) EMA to derive biomechanical measures of the tongue, (2) videofluoroscopic evaluation to measure swallow physiology, safety, and efficiency, and (3) maximal tongue strength testing using the Iowa Oral Pressure Instrument (IOPI). Participants completed self-reported functional assessments. Spearman's rank correlations assessed for associations between lingual biomechanics and swallowing physiology, swallow safety and efficiency, and self-reported bulbar function. RESULTS: Results demonstrated strong associations between biomechanical and swallowing physiology, swallow safety, and self-reported measures. Notably, swallowing safety during thin liquid intake was associated with tongue speed (r = - 0.7, p < 0.05) and range of motion (r = - 0.71, p < 0.05), and swallowing safety during puree intake was associated with tongue strength (r = - 0.69, p < 0.05). CONCLUSIONS: Our findings underscore the importance of tongue movements on swallowing physiology and safety, help improve our understanding of mechanisms of swallowing impairment, and highlight a potential clinical tool to index bulbar impairment.

Differences in voluntary and reflexive cough strength in individuals with amyotrophic lateral sclerosis and healthy adults.

INTRODUCTION: Dystussia impacts the ability to protect the airway. Voluntary cough provides a metric of airway defense but differs from the reflexive response to aspiration during swallowing. Therefore, we evaluated relationships between voluntary and reflexive cough among individuals with amyotrophic lateral sclerosis (ALS) and a healthy cohort. METHODS: Twenty-eight individuals with ALS and 26 healthy individuals completed voluntary and reflexive cough testing. Descriptive statistics, reliability, and paired t tests were conducted to evaluate differences in cough volume acceleration (CVA) and peak expiratory flow rate (PEFR) in voluntary vs reflexive cough. RESULTS: Compared with reflexive cough, voluntary CVA and PEFR were greater in individuals with ALS [t(27) = 4.83, P < .001 and t(27) = 8.69, P < .001] and the healthy cohort [t(21) = 5.91, P < .001 and t(21) = 12.26, P < .001], respectively. DISCUSSION: These findings hold important implications for the use and interpretation of voluntary peak cough flow during clinical swallowing evaluations.

Swallowing screening practice patterns for nurses in the cardiac surgery intensive care unit.

AIMS AND OBJECTIVES: The current study surveyed nurse practice patterns for performing swallowing screens in an academic cardiac intensive care unit (ICU). It aimed to index: training and levels of confidence in conducting dysphagia screens; screening methods employed; timing and frequency of implement; and subsequent plan of care in identified high-risk patients. BACKGROUND: Swallowing impairment (dysphagia) is common following cardiac surgery and associated with significant morbidity and mortality. Early and accurate detection of dysphagia is therefore critical to afford implementation of interventions to optimise patient care. Currently, no validated instruments or guidelines exist for nursing screening of dysphagia in this setting. METHODS: An anonymous and voluntary 10-item mixed-methods online survey was conducted using Qualtrics software. Nonprobability purposive sampling was utilised to recruit nurses working in an academic 24-bed cardiac ICU. Thematic analysis using operationally defined coding, SRQR checklist and descriptive statistics were employed. RESULTS: Sixty-nine nurses completed the survey during a 1-month period, representing an 84% response rate. Formal training in performing swallowing screens was reported in 18.6% of nurses. In rank order, reported level of confidence was the following: "moderately" (49%); "somewhat" (35%); "not" (13%); and "very" (3%). The majority of nurses performed screens within 1 hr (40.6%) or between 1-4 hr (43.8%) of extubation. Fifteen different methods were utilised to screen swallowing function by nurses who reported a total of 31 different clinical signs indicative of dysphagia. CONCLUSIONS: Survey data of practicing nurses in an academic cardiac ICU revealed limited formal training in swallowing screening methodology, a high degree of variability in screening methods employed and low levels of agreement for dysphagia signs. RELEVANCE TO CLINICAL PRACTICE: Data highlight a knowledge gap and need for the development of formal education and validated rapid nursing dysphagia screening tools for standardised implementation in the cardiac surgery ICU setting.

Impact of expiratory strength training in amyotrophic lateral sclerosis: Results of a randomized, sham-controlled trial.

INTRODUCTION: The purpose of this study was to determine the impact of an in-home expiratory muscle strength training (EMST) program on pulmonary, swallow, and cough function in individuals with amyotrophic lateral sclerosis (ALS). METHODS: EMST was tested in a prospective, single-center, double-blind, randomized, controlled trial of 48 ALS individuals who completed 8 weeks of either active EMST (n = 24) or sham EMST (n = 24). The primary outcome to assess treatment efficacy was change in maximum expiratory pressure (MEP). Secondary outcomes included: cough spirometry; swallowing; forced vital capacity; and scoring on the ALS Functional Rating Scale-Revised. RESULTS: Treatment was well tolerated with 96% of patients completing the protocol. Significant differences in group change scores were noted for MEP and Dynamic Imaging Grade of Swallowing Toxicity scores (P < 0.02). No differences were noted for other secondary measures. DISCUSSION: This respiratory training program was well-tolerated and led to improvements in respiratory and bulbar function in ALS. Muscle Nerve 59:40-46, 2019.

Provisional best practices guidelines for the evaluation of bulbar dysfunction in amyotrophic lateral sclerosis.

INTRODUCTION: Universally established comprehensive clinical bulbar scales objectively assessing disease progression in amyotrophic lateral sclerosis (ALS) are currently lacking. The goal of this working group project is to design a best practice set of provisional bulbar ALS guidelines, available for immediate implementation within all ALS clinics. METHODS: ALS specialists across multiple related disciplines participated in a series of clinical bulbar symposia, intending to identify and summarize the currently accepted best practices for the assessment and management of bulbar dysfunction in ALS Results: Summary group recommendations for individual speech, Augmentative and Alternative Communication (AAC), and swallowing sections were achieved, focusing on the optimal proposed level of care within each domain. DISCUSSION: We have identified specific clinical recommendations for each of the 3 domains of bulbar functioning, available for incorporation within all ALS clinics. Future directions will be to establish a formal set of bulbar guidelines through a methodological and evidence-based approach. Muscle Nerve 59:531-531, 2019.

Lingual and Jaw Kinematic Abnormalities Precede Speech and Swallowing Impairments in ALS.

Early identification of bulbar involvement in persons with ALS is critical for improving diagnosis and prognosis; however, efficacious diagnostic markers have not yet been identified. The purpose of this study was to determine whether kinematic changes of the tongue and jaw during swallowing, measured using 3D electromagnetic articulography (EMA), predate clinically identifiable symptoms of speech and swallowing impairment in persons diagnosed with ALS. Data were collected from 16 adults diagnosed with ALS and 18 neurotypical controls. Groups were aged matched. Eligible participants with ALS were tolerating an unrestricted diet (FOIS = 7), produced intelligible speech (> 97%), and had a speaking rate greater than 150 words per minute. Participants completed a 3-mL water swallow task, during which EMA recorded kinematic measures of the anterior and posterior regions of tongue including lingual speed, range of motion, duration, coordination, and efficiency. Jaw speed and range of motion were also recorded. Persons diagnosed with ALS demonstrated reduced posterior lingual range of motion (11.40 mm ± 4.01 vs. 16.07 mm ± 5.27), slower posterior lingual speeds (83.67 mm/s ± 47.96 vs. 141.35 mm/s ± 66.54), increased lingual movement duration (13.46 s ± 6.75 vs. 9.21 s ± 3.28), and reduced lingual coordination (0.04 s ± 0.11 vs. 17 s ± 0.19) during the 3-oz water swallow task compared to controls. Persons diagnosed with ALS demonstrated increased range of motion (9.86 mm ± 5.38 vs. 6 mm ± 3.78) and increased jaw speed (68.62 mm/s ± 50.13 vs. 34.72 mm/s ± 17.75) during swallowing compared to controls. The current findings suggest that changes in lingual and jaw motor performance during a simple water swallow task are present in persons with ALS who are pre-symptomatic of clinically detectable bulbar impairment.

Relationship Between the Eating Assessment Tool-10 and Objective Clinical Ratings of Swallowing Function in Individuals with Head and Neck Cancer.

The Eating Assessment Tool-10 (EAT-10) represents a validated, easy to administer patient report dysphagia severity scale. Although its ability to detect swallowing impairment has been investigated in other patient populations, the utility of this instrument in individuals with head and neck cancer (HNC) has not been studied. The aim of the current investigation was to determine the relationship between patient ratings of swallowing impairment (EAT-10) and objective clinical ratings of swallow physiology in individuals with HNC. Forty-four HNC participants completed the EAT-10 and a standardized videofluoroscopy swallow study. Blinded raters determined airway safety using the penetration-aspiration scale (PAS) and swallowing function using the modified barium swallow impairment profile (MBSImP™©). Participants were stratified into three groups (pre-treatment through 1 year post-treatment, 1-5 years post-treatment, and >5 years post-treatment). Independent t tests, Pearson's and Spearman's Rho correlations, and a Bonferroni correction for multiple comparisons were performed. EAT-10 scores were significantly higher in HNC patients with unsafe swallowing (M 24.45, SD 8.32) compared to those with safe swallowing (M 16.20, SD 12.14), t(21) = -2.36, p  < 0.04. Significant correlations were revealed between EAT-10 scores and the MBSImP™© (pharyngeal composite), and PAS scores (p < 0.05) for the pre-treatment to within 1 year post-treatment group. No associations, however, were observed for HNC patients in the time groups representing greater than 1-year post cancer treatment.

Impact of expiratory strength training in amyotrophic lateral sclerosis.

INTRODUCTION: We evaluated the feasibility and impact of expiratory muscle strength training (EMST) on respiratory and bulbar function in persons with amyotrophic lateral sclerosis (ALS). METHODS: Twenty-five ALS patients participated in this delayed intervention open-label clinical trial. Following a lead-in period, patients completed a 5-week EMST protocol. Outcome measures included: maximum expiratory pressure (MEP); physiologic measures of swallow and cough; and penetration-aspiration scale (PAS) scores. RESULTS: Of participants who entered the active phase of the study (n = 15), EMST was well tolerated and led to significant increases in MEPs and maximum hyoid displacement during swallowing post-EMST (P < 0.05). No significant differences were observed for PAS scores or cough spirometry measures. CONCLUSIONS: EMST was feasible and well tolerated in this small cohort of ALS patients and led to improvements in expiratory force-generating pressures and swallow kinematics. Further investigation is warranted to confirm these preliminary findings. Muscle Nerve 54: 48-53, 2016.

Defining Swallowing-Related Quality of Life Profiles in Individuals with Amyotrophic Lateral Sclerosis.

Although it is known that dysphagia contributes to significant malnutrition, pneumonia, and mortality in amyotrophic lateral sclerosis (ALS), it remains unclear how swallowing impairment impacts quality of life in this vulnerable patient population. The aim of the current study was to (1) delineate swallow-related quality of life (SR-QOL) profiles in individuals with ALS and (2) evaluate relationships between SR-QOL, degree of swallowing impairment, and ALS global disease progression. Eighty-one ALS patients underwent a standardized videofluoroscopic swallow study and completed the swallowing quality of life (SWAL-QOL) instrument and ALS functional rating scale-revised (ALSFRS-R). Penetration Aspiration Scale (PAS) scores were derived by a blinded rater. Correlation analyses and a between groups ANOVA (safe vs. penetrators vs. aspirators) were performed. Mean SWAL-QOL score for this cohort was 75.94 indicating a moderate degree of SR-QOL impairment with fatigue, eating duration, and communication representing the most affected domains. Correlations were revealed between the SWAL-QOL and (1) PAS (r = -0.39, p < 0.001) and (2) ALSFRS-R (r = 0.23, p < 0.05). Mean (SD) SWAL-QOL scores for safe versus penetrator versus aspirator groups were 81.2 (2.3) versus 77 (3.4) versus 58.7 (5.9), respectively, with a main effect observed [F(2,78) = 9.71, p < 0.001]. Post hoc testing revealed lower SWAL-QOL scores for aspirators versus safe swallowers (p < 0.001) and aspirators versus penetrators (p < 0.001). Overall, SR-QOL was moderately reduced in this cohort of ALS patients and profoundly impacted in ALS aspirators and individuals with advanced disease. These findings highlight the importance of early multidisciplinary intervention to not only avoid malnutrition, weight loss, and pulmonary sequelae but also the associated reduced QOL seen in these individuals.

Voluntary Cough Airflow Differentiates Safe Versus Unsafe Swallowing in Amyotrophic Lateral Sclerosis.

Dysphagia and aspiration are prevalent in amyotrophic lateral sclerosis (ALS) and contribute to malnutrition, aspiration pneumonia, and death. Early detection of at risk individuals is critical to ensure maintenance of safe oral intake and optimal pulmonary function. We therefore aimed to determine the discriminant ability of voluntary cough airflow measures in detecting penetration/aspiration status in ALS patients. Seventy individuals with ALS (El-Escorial criteria) completed voluntary cough spirometry testing and underwent a standardized videofluoroscopic swallowing evaluation (VFSE). A rater blinded to aspiration status derived six objective measures of voluntary cough airflow and evaluated airway safety using the penetration-aspiration scale (PAS). A between groups ANOVA (safe vs. unsafe swallowers) was conducted and sensitivity, specificity, area under the curve (AUC) and likelihood ratios were calculated. VFSE analysis revealed 24 penetrator/aspirators (PAS ≥3) and 46 non-penetrator/aspirators (PAS ≤2). Cough volume acceleration (CVA), peak expiratory flow rise time (PEFRT), and peak expiratory flow rate (PEFR) were significantly different between airway safety groups (p < 0.05) and demonstrated significant discriminant ability to detect the presence of penetration/aspiration with AUC values of: 0.85, 0.81, and 0.78, respectively. CVA <45.28 L/s/s, PEFR <3.97 L/s, and PEFRT >76 ms had sensitivities of 91.3, 82.6, and 73.9 %, respectively, and specificities of 82.2, 73.9, and 78.3 % for identifying ALS penetrator/aspirators. Voluntary cough airflow measures identified ALS patients at risk for penetration/aspiration and may be a valuable screening tool with high clinical utility.

Longitudinal Examination of Swallowing Safety and Vocal Fold Mobility in Cardiac Surgical Patients.

BACKGROUND: Aspiration and vocal fold mobility impairment (VFMI) are frequently reported in adults after cardiac surgery (CS) and impede recovery. Preoperative and postoperative laryngoscopic evaluations have not been undertaken, masking the incidence and evolution of dysphagia and VFMI in CS patients. We therefore sought to determine frequency of unsafe swallowing and VFMI before and after CS. METHODS: Thirty-five adults undergoing elective CS enrolled. Participants underwent fiberoptic endoscopic evaluations of swallowing and VFMI before and after surgical procedure. Trained raters performed duplicate, blinded ratings with the validated Penetration-Aspiration Scale, and a laryngologist performed blinded ratings of VFMI. Descriptive, Wilcoxon signed rank, and McNemar tests were performed. RESULTS: Preoperative swallowing safety profiles were 60% safe, 34% penetration, and 6% aspiration. Postoperative swallowing safety profiles were 14% safe, 63% penetration, and 23% aspiration. Significant differences in preoperative to postoperative swallowing outcomes were noted for Penetration-Aspiration Scale scores (P < .0001), unsafe swallowing (40% vs 86%; χ2 = 12.8; P = .0003), and aspiration (6% vs 23%; χ2 =6; P = .01). No differences in VFMI were noted preoperatively to postoperatively (partial VFMI, 9% vs 23%; P > .05). CONCLUSIONS: A 4-fold increase in aspiration was observed in CS patients. No cases of vocal fold paralysis were observed across time points. These data highlight the utility of instrumental laryngoscopic evaluations during the acute postoperative phase.

Longitudinal changes of swallowing safety and efficiency in infants with spinal muscular atrophy who received disease modifying therapies.

BACKGROUND: Dysphagia is a common feature of the natural history of patients with spinal muscular atrophy (SMA). Literature regarding swallowing safety and efficiency is scarce in patients with SMA, particularly in the era of newborn screening programs and disease-modifying therapies. OBJECTIVE: To describe the longitudinal changes of swallowing safety and efficiency in children with SMA who received one or more disease modifying therapies METHODS: Case series of patients with SMA followed at the University of Florida from 1 May 2019 to 31 December 2022 who had two or more videofluoroscopy swallowing studies (VFSS), with the first being within 30 days of their first treatment. Data extracted from the electronic health record included: neuromotor outcomes, VFSS penetration aspiration scores (PAS), presence of abrnormal oral or pharyngeal residue, clinical history, and timing of disease-modifying therapies administration. RESULTS: Seven subjects were included (five male); three were diagnosed via newborn screen. Median age at diagnosis was 10 days (range: 4-250). Median age at initial VFSS was 29 days (range: 9-246), and age at the last VFSS was 26.1 months (range: 18.2-36.2). All subjects received onasemnogene-abeparvovec (OA); four received additional therapies. PAS at diagnosis was abnormal in four subjects. Six subjects required feeding modifications after VFSS results. Of these, three had silent aspiration (PAS 8) and three of them improved after treatment. CONCLUSIONS: Swallowing safety and efficiency can be impaired in patients with SMA despite early treatment. Larger, prospective studies are needed to define optimal timiing of longitudinal instrumental evaluations.

Causes of dysphagia in a tertiary-care swallowing center.

OBJECTIVES: Dysphagia can be caused by a myriad of disease processes, and it has significant impacts on patients' quality of life, life expectancy, and economic burden. To date, the most common causes of dysphagia in outpatient tertiary-care swallowing centers are unknown. We undertook this study to determine these prevalences. We also describe the diagnostic techniques utilized to establish the diagnosis. METHODS: The electronic charts of 100 consecutive patients who presented to an outpatient tertiary-care university swallowing center between January 2010 and April 2011 were retrospectively reviewed. Information regarding patient demographics, validated symptom surveys, diagnostic workups, and ultimate diagnoses was abstracted and tabulated into a central database. Descriptive statistics were used to evaluate the association between patient symptoms and diagnoses. RESULTS: The mean age of the entire cohort was 62 +/- 13.5 years, and 58% of the cohort was male. The most common identified causes of dysphagia were reflux (27%), postirradiation dysphagia (14%), and cricopharyngeus muscle dysfunction (11%). In 13% of cases, the cause of dysphagia was undetermined. The diagnostic tests utilized included flexible laryngoscopy (71%; 17% with endoscopic swallow evaluation), modified barium swallow study (45%), esophagoscopy (35%), barium esophagography (21%), manometry (10%), and ambulatory pH testing (2%). CONCLUSIONS: The most common causes of dysphagia in a tertiary-care swallowing center are reflux, postirradiation dysphagia, and cricopharyngeus muscle dysfunction. A precise cause for the symptom could not be identified in 13% of our cohort. Endoscopic visualization (laryngoscopy, flexible endoscopic evaluation of swallowing, and transnasal esophagoscopy) and fluoroscopic swallow studies were the investigations most often utilized. These techniques can be used to arrive at a diagnosis in 80% of cases.