The Impact of Lingual Resistance Training in Two Individuals with Amyotrophic Lateral Sclerosis: A Case Series

Introduction: Amyotrophic Lateral Sclerosis (ALS) is a fatal and progressive disease characterized by the deterioration of motor neurons within the body. This degeneration leads to bulbar dysfunction in the form of an impaired ability to communicate and swallow. Currently, bulbar dysfunction is treated via compensatory methods aimed at maximizing comfort and safety that include environmental adaptations, augmentative and assistive communication and gastrostomy tube placement to replace speech and oral feeding. The role of active intervention is controversial in this patient population and no investigations have examined the potential role lingual exercise might play in ALS bulbar management. The purpose of this study was to explore the impact of an eight week lingual resistance training program on lingual strength and lingual endurance, speech, swallowing, global disease progression and patient-reported outcomes in two individuals with ALS. Methods: Two individuals with ALS (El Escorial criteria) were enrolled across three different time points, each separated by eight weeks (Baseline 1, Baseline 2, Post-Treatment) with a delayed intervention design utilized to benchmark bulbar disease progression. At each evaluation, tongue strength, endurance, swallowing, speech and patient-reported outcomes were collected. Following Baseline 2, participants completed lingual resistance training five days a week for eight weeks (40 sessions). Outcome measures included maximum anterior isometric tongue pressure (MIP), maximum endurance hold time MHT), speech intelligibility, airway safety and patient-reported outcomes. Results: On average, MIPs decreased by 2% during the lead in period and increased by 13% across both participants. On average, MHT declined by 25% between baseline 1 and 2 and increased by 144% following lingual resistance training. No improvements were noted for speech intelligibility or airway safety during swallowing. Patient-perceptions of communicative effectiveness, swallowing impairment and quality of life remained relatively stable over the four-month period. Conclusion: Although no improvements were noted in lingual strength, improvements in lingual endurance hold times were noted in both participants. Further investigation is warranted to validate these preliminary findings in two cases of ALS

The Impact of Lingual Resistance Training in Two Individuals with Amyotrophic Lateral Sclerosis: A Case Series

Introduction: Amyotrophic Lateral Sclerosis (ALS) is a fatal and progressive disease characterized by the deterioration of motor neurons within the body. This degeneration leads to bulbar dysfunction in the form of an impaired ability to communicate and swallow. Currently, bulbar dysfunction is treated via compensatory methods aimed at maximizing comfort and safety that include environmental adaptations, augmentative and assistive communication and gastrostomy tube placement to replace speech and oral feeding. The role of active intervention is controversial in this patient population and no investigations have examined the potential role lingual exercise might play in ALS bulbar management. The purpose of this study was to explore the impact of an eight week lingual resistance training program on lingual strength and lingual endurance, speech, swallowing, global disease progression and patient-reported outcomes in two individuals with ALS. Methods: Two individuals with ALS (El Escorial criteria) were enrolled across three different time points, each separated by eight weeks (Baseline 1, Baseline 2, Post-Treatment) with a delayed intervention design utilized to benchmark bulbar disease progression. At each evaluation, tongue strength, endurance, swallowing, speech and patient-reported outcomes were collected. Following Baseline 2, participants completed lingual resistance training five days a week for eight weeks (40 sessions). Outcome measures included maximum anterior isometric tongue pressure (MIP), maximum endurance hold time MHT), speech intelligibility, airway safety and patient-reported outcomes. Results: On average, MIPs decreased by 2% during the lead in period and increased by 13% across both participants. On average, MHT declined by 25% between baseline 1 and 2 and increased by 144% following lingual resistance training. No improvements were noted for speech intelligibility or airway safety during swallowing. Patient-perceptions of communicative effectiveness, swallowing impairment and quality of life remained relatively stable over the four-month period. Conclusion: Although no improvements were noted in lingual strength, improvements in lingual endurance hold times were noted in both participants. Further investigation is warranted to validate these preliminary findings in two cases of ALS

Comparison of Validated Videofluoroscopic Outcomes of Pharyngeal Residue: Concordance Between a Perceptual, Ordinal, and Bolus-Based Rating Scale and a Normalized Pixel-Based Quantitative Outcome

Purpose: This study compared the concordance between two validated videofluoroscopic pharyngeal residue outcome scales used in clinical and research settings: the Dynamic Imaging Grade of Swallowing Toxicity (DIGEST) and the Analysis of Swallowing Physiology: Events, Kinematics and Timing (ASPEKT) percent residue metric. Method: Two trained raters completed independent and blinded videofluoroscopic ratings of both DIGEST efficiency and ASPEKT percent pharyngeal residue outcomes for bolus trials of 5 cc Varibar thin liquid ( n = 223), thin liquid comfortable cup sips ( n = 223), and 5 cc Varibar thin honey ( n = 223). Spearman's rho and Kruskal–Wallis analyses were performed for each bolus type. Results: Significant associations between DIGEST and ASPEKT pharyngeal residue outcomes were noted for 5 cc thin ( r = .54, p &lt; .001), cup sip thin ( r = .41, p &lt; .001), and 5 cc thin honey ( r = .60, p &lt; .001) bolus trials. ASPEKT percent residue increased across worsening DIGEST efficiency ordinal scale levels, with a main effect for each bolus type. Post hoc analysis revealed significant differences in the ASPEKT percent residue values between each DIGEST pairwise comparison for the thin cup sip bolus trial and for each pairwise comparison except between the moderate-to-severe (less than half vs. majority) ordinal levels for the 5 cc thin and 5 cc honey bolus trials, p &lt; .05. Conclusions: Perceptual, ordinal (DIGEST) and quantitative, pixel-based (ASPEKT) videofluoroscopic pharyngeal residue outcomes were associated in this data set of 669 bolus trials. Future research is warranted to confirm these results. </jats:sec

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 \u3c 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, 201

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 \u3c 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, 201

New horizons in understanding oral health and swallowing function within the context of frailty

AbstractFrailty is a complex and multidimensional condition wherein declines in physiologic reserve and function place individuals in a state of heightened vulnerability and decreased resiliency. There has been growing interest in both research and clinical settings to understand how to best define, assess and characterise frailty in older adults. To this end, various models and clinical assessment tools have been used to define and measure frailty. While differences exist among these models and tools, a common unifying theme is a focus on physical function and activity. Notably absent across many available conceptual models and clinical tools are items directly related to oral and swallowing function. This is an important oversight as widespread changes to both oral and swallowing function are evident in older adults. Indeed, emerging evidence suggests many of the functional domains affected in frail older adults, such as nutrition and sarcopenia, have cyclical relationships with impairments in oral (oral hypofunction) and swallowing function (dysphagia) as well. The increasing appreciation for the interrelationships among oral hypofunction, dysphagia and frailty provides an opportunity for refinement of frailty assessment and characterisation in older adults to incorporate metrics specific to oral and swallowing function.</jats:p

A Cross-Sectional, Quantitative Videofluoroscopic Analysis of Swallowing Physiology and Function in Individuals With Amyotrophic Lateral Sclerosis

Purpose To date, research characterizing swallowing changes in individuals with amyotrophic lateral sclerosis (ALS) has primarily relied on subjective descriptions. Thus, the degree to which swallowing physiology is altered in ALS, and relationships between such alterations and swallow safety and/or efficiency are not well characterized. This study provides a quantitative representation of swallow physiology, safety, and efficiency in a sample of individuals with ALS, to estimate the degree of difference in comparison to published healthy reference data and identify parameters that pose risk to swallow safety and efficiency. Secondary analyses explored the therapeutic effect of thickened liquids on swallowing safety and efficiency. Method Nineteen adults with a diagnosis of probable-definite ALS (El-Escorial Criteria–Revised) underwent a videofluoroscopic swallowing study, involving up to 15 sips of barium liquid (20% w/v), ranging in thickness from thin to extremely thick. Blinded frame-by-frame videofluoroscopy analysis yielded the following measures: Penetration–Aspiration Scale, number of swallows per bolus, amount of pharyngeal residue, degree of laryngeal vestibule closure (LVC), time-to-LVC, duration of LVC (LVCdur), pharyngeal area at maximum constriction, diameter of upper esophageal sphincter opening, and duration of UES opening (UESOdur). Measures of swallow physiology obtained from thin liquid trials were compared against published healthy reference data using unpaired t tests, chi-squared tests, and Cohen’s d effect sizes (adjusted p &lt; .008). Preliminary relationships between parameters of swallowing physiology, safety, and efficiency were explored using nonparametric Cochrane’s Q, Friedman’s test, and generalized estimating equations ( p &lt; .05). Results Compared to healthy reference data, this sample of individuals with ALS displayed a higher proportion of swallows with partial or incomplete LVC (24% vs. &lt; 1%), increased time-to-LVC ( d = 1.09), reduced UESwidth ( d = 0.59), enlarged pharyngeal area at maximum constriction, prolonged LVCdur ( d = 0.64), and prolonged UESOdur ( d = 1.34). Unsafe swallowing (i.e., PAS ≥ 3) occurred more frequently when LVC was partial/incomplete or time-to-LVC was prolonged. Pharyngeal residue was associated with larger pharyngeal areas at maximum constriction. Unsafe swallowing occurred less frequently with extremely thick liquids, compared to thin liquids. No significant differences in pharyngeal residue were observed based on liquid thickness. Conclusions Quantitative videofluoroscopic measurements revealed moderate-to-large differences in swallow physiology between this sample of individuals with ALS and healthy reference data. Increased time-to-LVC, noncomplete LVC, and enlarged pharyngeal area at maximum constriction were associated with impaired swallow safety or efficiency. Thickened liquids may mitigate the risk of acute episodes of aspiration in individuals with ALS. Further work is needed to corroborate these preliminary findings and explore how swallowing profiles evolve throughout disease progression. </jats:sec