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

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

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

ObjectiveThe 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.MethodsTwo-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).ResultsAlthough below acceptable screening tool criterion, a score of ≤ 3 on the ALSFRS-R swallowing item optimized classification accuracy to detect global pharyngeal dysphagia (sensitivity: 68%, specificity: 64%, AUC: 0.68) and penetration/aspiration (sensitivity: 79%, specificity: 60%, AUC: 0.72). Depending on score selection, sensitivity and specificity of the ALSFRS-R bulbar subscale ranged between 34-94%. A score of ConclusionsThe ALSFRS-R bulbar subscale or swallowing item did not demonstrate adequate diagnostic accuracy to detect radiographically confirmed swallowing impairment. These results suggest the need for alternate screens for dysphagia in ALS

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

Effects Of Submental Surface Electrical Stimulation On Swallowing Kinematics In Healthy Adults: An Error-Based Learning Paradigm

Purpose: Hyoid bone and laryngeal approximation aid airway protection (laryngeal vestibule closure) while moving toward their peak superior and anterior positions during swallowing. Submental surface electrical stimulation (SES) is a therapeutic technique that targets the muscles that move the hyoid bone during swallowing. It is unknown whether submental SES only increases peak hyoid bone swallowing positions but not peak laryngeal swallowing positions, which could require faster or greater laryngeal movement to achieve adequate laryngeal vestibule closure. Method: We examined the effects of submental SES on hyo-laryngeal kinematics in 30 healthy adults who swallowed 50 times using an error-based learning paradigm. Results: Submental SES did not alter any hyo-laryngeal swallowing kinematic. However, submental SES significantly changed the starting position of the hyoid bone just prior to the swallow onset (more anterior; p = .003). On average, submental SES immediately prior to swallow onset can position the hyoid approximately 20% closer to its peak swallowing point. Conclusions: These findings indicate that electrical stimulation of the agonists for hyoid movement might not alter swallowing outcomes tested in this study. However, submental SES could have clinical utility by minimizing swallowing impairments related to reduced hyoid swallowing range of motion in individuals with dysphagia.Wo