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DYSPHAGIA ASSESSMENT AND TREATMENT PLANNING: A TEAM APPROACH
Myotonic Dystrophy Patient Registry evaluated 556 DM1 patients and their symptoms, identifying 48% with dys­phagia complaints. Those with myo­tonic symptoms reported dysphagia at more than twice the rate of those with­out myotonia (55.3% vs. 21.9%) (Wood et al., 2017). The pharynx and UES are the most affected, and intra-degluti­tive and post-deglutitive aspiration of bolus lead to pneumonia. Aspiration was detected in greater than 50% of 169 patients with MD (Willaert et al.,
2015). Delayed swallowing gestures and increase transit time have been associated with increased risk of aspi­ration (Leonard et al., 2001). In children, labial weakness also impairs bolus con­trol (Berggren et al., 2018). Pharyngeal muscle weakness contributes to poor bolus transit and can be seen on DSS as an increasing pharyngeal constric­tion ratio (Leonard et al., 2011). If pha­ryngeal outlet obstruction is suspected, then early DSS examination may be useful to direct treatment and prevent pharyngeal deterioration.
Myasthenia Gravis
Myasthenia gravis (MG) is an autoim­mune disorder of the neuromuscular junction (NMJ). It is caused by anti­bodies against the muscle membrane of the NMJ, resulting in inadequate acetylcholine in the junction and poor muscle contraction. In 85% of MG patients, anti-AChR (nicotinic acetyl­choline receptor) antibodies are the cause, but in 5% to 8% of cases, these are absent, and instead muscle-specific kinase (MuSK) antibodies are pres­ent (Stathopoulos & Dalakas, 2022). MuSK-positive patients appear to have greater prevalence of bulbar symptoms with dysphagia, dysphonia, and dysar-
thria (Stathopoulos & Dalakas, 2022). In fact, dysphagia may be the present­ing complaint in a quarter of patients with MG (Berrih-Aknin et al., 2014; Colton-Hudson et al., 2002; Klair et al.,
2014).
Dysphagia is a source of significant morbidity and mortality in MG, par­ticularly if the underlying disorder is unrecognized, which is common par­ticularly if nontypical antibodies are present. Aspiration may occur and be silent, risking pulmonary health. DSS findings include abnormal oral control of bolus with early spill, residue in the oral and oropharyngeal cavities, slow pharyngeal transit, and reduced pha­ryngeal constriction ratio, penetration, and aspiration (Colton-Hudson et al., 2002; Yamamoto et al., 2012). In 20 MG patients complaining of dysphagia, 13 demonstrated penetration during swal­low and 7 demonstrated aspiration, of whom 4 had silent aspiration (Colton­Hudson et al., 2002). Manometry dem­onstrates esophageal hypomotility and abnormal UES contraction (Stathopou­los & Dalakas, 2022).
Normal rehabilitative strategies such as exercises are not effective in MG due to muscle fatigue, and therefore posi­tioning and dietary modifications may be more appropriate and should be guided by DSS examination. Medical therapy is indicated in MG (typically pyridostigmine, steroids, azathioprine, or mycophenylate), and assessment on medication should be considered so that diet may be adapted for optimal function. DSS with edrophonium chlo­ride treatment may assist in making the diagnosis of MG by demonstrating changes in pharyngeal parameters with treatment on board (Yamamoto et al.,
2012). Rituximab therapy for MuSK­positive patients is highly effective and
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indicated as primary treatment in this group because it depletes B cells in cir­culation that are producing the autoan­tibody (Stathopoulos & Dalakas, 2022). Additional new immune therapies are in trial and may provide additional benefit for swallowing.
SUMMARY
Many neurological disorders impact swallowing safety, efficiency, and vigor. DSS is a crucial tool in unravelling the specific abnormalities present and should be a key feature of full swallow­ing assessment in these patients.
STUDY QUESTIONS
1. What types of stroke produce dys­phagia?
2. Which is more likely to be affected by stroke: oropharynx or hypo­pharynx function for swallowing?
DSS in an acute stroke patient? Should DSS be repeated in acute stroke patients, and if so, when?
4. What are typical features of dys­phagia in head-injured patients?
5. Have significant features of swal­lowing difficulty been identified for patients with muscular dystrophy? Multiple sclerosis? Polymyositis or dermatomyositis?
6. Do patients with neurological causes of oropharyngeal dysphagia respond to swallowing therapy?
7. Do patients with neurological causes of oropharyngeal dysphagia respond to surgical therapy?
8. Can DSS help identify who might benefit most from surgery to the upper esophageal sphincter
in patients with inclusion body myositis?
9. If a DSS is recommended for a patient with Parkinson’s disease, what might the clinician want to consider in scheduling it?
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Dysphagia in Head and
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Neck Cancer Patients
Katherine A. Kendall
Successful deglutition depends on sen­sory input from the muscles and the mucosal surfaces of the oropharynx to regulate and fine-tune the sequence of muscular contractions that results in a swallow. It makes sense that dis­ruption of the sensory, muscular, or structural integrity of the oral cavity, pharynx, and larynx causes dysphagia. In patients with head and neck cancer, tumor growth, changes in tissue char­acteristics secondary to radiation with or without chemotherapy, and any surgical procedure involving the head and neck region, therefore, have the potential to cause dysphagia. Indeed, xerostomia and dysphagia are the most often cited contributors to a decrease in quality of life for head and neck cancer patients after the completion of treat­ment (Wang & Eisbruch, 2016). This chapter will focus on the swallowing difficulty experienced by head and neck cancer patients.
INTRODUCTION
In patients with head and neck can­cer, interference with normal swal­lowing may result from the growth of the tumor-invading structures, impair­ing their functioning, or from the obstructive effects of the tumor itself, which interfere with bolus move­ment. Surgery to excise the tumor with a margin of normal tissue typically results in a defect with loss of struc­tures needed for normal deglutition. The method chosen for reconstruction of the defect will subsequently influ­ence the restoration of normal ana­tomic contours and function. Thus, the reconstruction affects the character and the severity of the resultant dysphagia. When postoperative radiation therapy is added to the regimen, dysphagia may worsen secondary to xerostomia and fibrosis of soft tissues in the field of radiation exposure, especially if
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