Screening and Evaluation Tools of Dysphagia in Adults With Neuromuscular Diseases: A Systematic Review
Audag, N., Goubau, C., et al. (2019).
10, 2040622318821622.
This systematic review investigates the validity and reliability of dysphagia screening and assessment tools for mixed populations of individuals with neuromuscular diseases.
Fund Eliane Lagast and Association Belge Contre les Maladies Neuro-Musculaires Aide à la Recherche (Belgium); Fondation Saint-Luc (Belgium); Institut de Recherche Expérimentale et Clinique, Université Catholique de Louvain (Belgium)
From database inception to June 2018
Original research studies
19
<div>VFSS had a 92% sensitivity for swallowing impairment in individuals with ALS and spinal muscular atrophy patients. "[A]ll of the nondysphagic patients also showed radiological swallowing abnormalities indicating a specificity of 0% in this study" (p. 4).<br><br>In individuals with Duchenne muscular dystrophy, VFSS abnormalities were related to age, except for oral holding. VFSS was a better indicator of the oral phase of swallowing (p < 0.05) and pooling of contrast fluid in the vallecula (p < 0.05) when compared to the dysphagia questionnaire.</div>
<div>Pharyngo-esophageal manometry in patients with ALS and spinal muscular atrophy showed abnormal upper esophageal sphincter opening and hypotonia of the proximal pharynx had 80% sensitivity and 20% specificity as indicators of dysphagia.</div>
<div>FEES was 53% sensitive to swallowing alterations in individuals with ALS and spinal muscular atrophy, but had 85% specificity.<br><br>For individuals with myotonic dystrophy type 1, inter- and intra-rater reliability was sufficient for all FEES variables, with a weighted kappa > 0.61. Bolus consistency significantly influenced the likelihood of observing mild or severe swallowing impairment.</div>
<div>The dysphagia limit (DL; a non-instrumental method estimating the volume at which second or more swallows become necessary to swallow a single bolus) was used to compare the peak duration and relative timing of muscle activity of four muscle groups during swallowing. DL was significantly correlated with dysphagia (p < 0.001) for individuals with ALS, polymyositis/dermatomyositis and myasthenia gravis but not for individuals with myotonic dystrophy type 1 (p > 0.05).<br><br>Individuals with with Duchenne muscular dystrophy (DMD) had to use a greater maximal muscle activity to swallow compared to controls. However, no differences were found between individuals with DMD and dysphagia and those with intact swallowing function. Surface electromyography (sMEG) can be used to distinguish disordered swallowing function in individuals with DMD via physiological changes in the swallowing muscles activity. "The mean differences between the repeated sEMG assessments were amplitude: 29.12 ± 21.69% MVC, duration: 0.69 ± 0.62s, and relative timing: 0.40 ± 0.24s" (p.8).</div>
<div>In individuals with spinal and bulbar muscular atrophy (SBMA), the values of tongue pressure in those with laryngeal penetration were significantly smaller than those without (p = 0.018), when compared with VFSS. Test–retest reliability of tongue pressure for individuals with SBMA was excellent; the intraclass correlation coefficient was 0.986 (p < 0.001).</div>
<div>In individuals with inclusion body myositis, penetration was seen in 10% by real-time magnetic resonance imaging (RT-MRI) and in 30% with FEES or VFSS. Bolus retention in the pharyngeal tract, the most sensitive indicator of functional swallowing deficits, was identified by RT-MRI as well as FEES and VFSS. Inter-rater agreement was assessed for bolus transport and retention; the Krippendorff α for bolus transport was 0.39 (RT-MRI), 0.55 (VFSS), and 0.67 (FEES), and for retention was 0.51 (RT-MRI), 0.52 (VFSS), and 0.52 (FEES).</div>
<div>In patients with ALS, cough volume acceleration (CVA), peak expiratory flow rate (PEFR) and peak expiratory flow rise time (PEFRT) were significant predictors of penetration/aspiration status. "ALS patients with a CVA less than 45.3 liters per second squared were 5.6 times more likely to penetrate/aspirate. A PEFR lower than 4.0l and a PEFRT greater than 80ms increased 3.6 and 3.2 times the risk of penetration/aspiration, respectively. Those cutoffs for CVA, PEFR and PEFRT 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" (p. 8).</div>
<div>For individuals with ALS, the total eating assessment tool 10 (EAT-10) score demonstrated good ability to identify penetrators vs. aspirators using a cutoff score of 3 (with a sensitivity of 88% and specificity of 57%). It demonstrated excellent accuracy at identifying aspirators vs. safe swallowers utilizing a cutoff score of 8 (with a sensitivity of 86% and specificity of 72%).</div>
<div>The neuromuscular disease swallowing status scale (NdSSS) was significantly correlated with penetration aspiration score in individuals with ALS (r = −0.51) but not in individuals with Duchenne muscular dystrophy (DMD) (r = 0.22). "For inter- and intrarater reliabilities, the weighted kappas were 0.95 and 1.00, respectively, for DMD; and 0.98 and 0.98, respectively, for ALS" (p. 9). "Responsiveness was determined with the standardized response mean for the NdSSS and was large in ALS (1.21) and moderate in DMD (0.65)" (p. 9).</div>
<div>The volume-viscosity swallow test showed a high sensitivity (93%) and specificity (80%) for screening and diagnosing oropharyngeal dysphagia in individuals with ALS.</div>
<div>In individuals with Duchenne muscular dystrophy, the Sydney swallow questionnaire detected dysphagia with good sensitivity (78%) and specificity (83%) at a cutoff score of 234 (total maximum score = 1700).</div>
<div>For individuals with neuromuscular diseases, the 3-ounce water swallow test showed a higher specificity (86%) than clinical signs on a 25-item form identifying dysphagia but a lower sensitivity (52%) compared with VFSS.</div>
<div>For individuals with inclusion body myositis, a standard questionnaire was compared with VFSS results and showed a sensitivity of 70% and specificity of 44% for impaired food propulsion and a sensitivity of 63% and specificity of 58% for aspiration-related problems. "Two questions reliably predicted the presence of impaired food propulsion on VFSS, namely 'Does food get stuck in your throat?' and 'Do you have to swallow repeatedly in order to get rid of food?'" (p. 9).</div>
<div>For patients with ALS undergoing VFSS, the oral phase of swallowing seemed the most affected. Pharyngeal contraction was correlated with penetration aspiration score.<br><br>The VFSS had acceptable test-retest reliability in ALS patients when using the dysphagia outcome severity scale and the Aspiration-Penetration Rating Scale. Inter-rater reliability of 92% and 95% agreement, and intra-rater reliability of 98% and 100% agreement, respectively were found.</div>
<div>Abnormal laryngeal elevation during VFSS was significantly correlated with aspiration (p = 0.001) and may aid in predicting aspiration and pneumonia in individuals with myasthenia gravis.</div>
<div>In individuals with dysphagia and ALS, reduced submental/suprahyoid activity and laryngeal-pharyngeal movements were detected. "A strong to moderate correlation was observed between these two indices and the penetration aspiration score and dysphagia outcome severity score, respectively. Moreover, a decrease in swallowing reproducibility could be a preclinical sign of dysphagia and, beyond a certain threshold, a pathological hallmark of oropharyngeal dysphagia" (p. 8).</div>
<div>Maximum tongue pressure (MTP) was significantly lower in the individuals "with ALS with reduced tongue function (p = 0.002) or with pharyngeal residue (p = 0.006) than in the patients with normal characteristics. Bolus formation and oral and pharyngeal transit time observed in VFSS were significantly prolonged among those with reduced MTP (p < 0.01)" (p. 8).</div>
