Endoscopic characteristics of dysphagia in multiple system atrophy compared to Parkinson's disease

Background: Dysphagia is a major clinical concern in multiple system atrophy (MSA). A detailed evaluation of its major endoscopic features compared with Parkinson’s disease (PD) is lacking. Objective: This study systematically assessed dysphagia in MSA compared with PD and correlated subjective dysphagia to objective endoscopic findings. Methods: Fifty-seven patients with MSA (median, 64 [interquartile range (IQR): 59–71] years; 35 women) underwent flexible endoscopic evaluation of swallowing using a specific MSA–flexible endoscopic evaluation of swallowing task protocol. Findings were compared with an age-matched cohort of 57 patients with PD (median, 67 [interquartile range: 60–73] years; 28 women). In a subcohort, subjective dysphagia was assessed using the Swallowing Disturbance Questionnaire and correlated to endoscopy findings. Results: Patients with MSA predominantly showed symptoms suggestive of oral-phase disturbance (premature spillage, 75.4%, piecemeal deglutition, 75.4%). Pharyngealphase symptoms occurred less often (pharyngeal residues, 50.9%; penetration/aspiration, 28.1%). In contrast, pharyngeal symptoms were the most common finding in PD (pharyngeal residues, 47.4%). Oral symptoms occurred less frequently in PD (premature spillage, 15.8%, P < 0.001; piecemeal deglutition, 1.8%, P < 0.01). Patients with MSA had a greater risk for oral-phase disturbances with increased disease severity (P < 0.05; odds ratio, 3.15). Patients with MSA showed a significantly higher intraindividual interswallow variability compared with PD. When correlating Swallowing Disturbance Questionnaire scores with endoscopy results, its cutoff, validated for PD, was not sensitive enough to identify patients with MSA with dysphagia. We developed a subscore for identifying dysphagia in MSA and calculated a new cutoff (sensitivity 85%, specificity 100%). Conclusions: In contrast with patients with PD, patients with dysphagic MSA more frequently present with oralphase symptoms and a significantly higher intraindividual interswallow variability. A novel Swallowing Disturbance Questionnaire MSA subscore may be a valuable tool to identify patients with MSA with early oropharyngeal dysphagia.Projekt DEAL 202

The Shaking Palsy of the Larynx—Potential Biomarker for Multiple System Atrophy: A Pilot Study and Literature Review

In its early stages multiple system atrophy (MSA), a neurodegenerative movement disorder, can be difficult to differentiate from idiopathic Parkinson's disease (PD), and emphasis has been put on identifying premotor symptoms to allow for its early identification. The occurrence of vegetative symptoms in addition to motor impairment, such as orthostatic hypotension and neurogenic bladder dysfunction, enable the clinical diagnosis in the advanced stages of the disease. Usually with further disease progression, laryngeal abnormalities become clinically evident and can manifest in laryngeal stridor due to impaired vocal fold motion, such as vocal fold abduction restriction, mostly referred to as vocal fold paresis, or paradoxical vocal fold adduction during inspiration. While the pathogenesis of laryngeal stridor is discussed controversially, its occurrence is clearly associated with reduced life expectancy. Before the clinical manifestation of laryngeal dysfunction however, abnormal vocal fold motion can already be seen in patients that might not yet fulfill the diagnostic criteria of MSA. In this article we summarize the current literature on pharyngolaryngeal findings in MSA and report preliminary findings from a pilot study investigating eight consecutive MSA patients. Patients showed varying speech abnormalities. Only 2/8 patients exhibited laryngeal stridor. However, during FEES, all patients presented with irregular arytenoid cartilages movements and vocal fold abduction restriction. 3/8 showed vocal fold fixation and 1/8 paradoxical vocal fold motion. All patients presented with oropharyngeal dysphagia, 5/8 with penetration or aspiration events. We suggest that specific abnormal vocal fold motion can help identifying MSA patients and may allow for delimiting this disorder from idiopathic PD. These findings therefore may serve as a novel clinical biomarker for MSA. Based on the available data and our preliminary clinical experience we developed a standardized easy-to-implement task-protocol to be performed during flexible endoscopic evaluation of swallowing (FEES) for detection of MSA-related pharyngolaryngeal movement disorders. Furthermore, we initiated a prospective study to evaluate the diagnostic utility of this protocol

Validation of the DIGEST-FEES as a Global Outcome Measure for Pharyngeal Dysphagia in Parkinson’s Disease

&lt;jats:title&gt;Abstract&lt;/jats:title&gt;&lt;jats:p&gt;Flexible Endoscopic Evaluation of Swallowing (FEES) is one of two diagnostic gold standards for pharyngeal dysphagia in Parkinson's disease (PD), however, validated global outcome measures at the patient level are widely lacking. The Dynamic Imaging Grade of Swallowing Toxicity for Flexible Endoscopic Evaluation of Swallowing (DIGEST-FEES) represents such an outcome measure but has been validated primarily for head and neck cancer collectives. The objective of this study was, therefore, to investigate the validity of the DIGEST-FEES in patients with PD. Content validity was evaluated with a modified Delphi expert survey. Subsequently, 66 FEES videos in PD patients were scored with the DIGEST-FEES. Criterion validity was determined using Spearman's correlation coefficient between the DIGEST-FEES and the Penetration-Aspiration Scale (PAS), the Yale-Residue-Rating-Scale, the Functional-Oral-Intake-Scale (FOIS), and the swallowing-related Unified-Parkinson-Disease-Rating-Scale (UPDRS) items. Inter-rater reliability was determined using 10 randomly selected FEES-videos examined by a second rater. As a result, the overall DIGEST-FEES-rating exhibited significant correlations with the Yale-Valleculae-Residue-Scale (&lt;jats:italic&gt;r&lt;/jats:italic&gt; = 0.84; &lt;jats:italic&gt;p&lt;/jats:italic&gt; &amp;lt; 0.001), the Yale-Pyriform-Sinus-Residue-Scale (&lt;jats:italic&gt;r&lt;/jats:italic&gt; = 0.70; &lt;jats:italic&gt;p&lt;/jats:italic&gt; &amp;lt; 0.001), the FOIS (&lt;jats:italic&gt;r&lt;/jats:italic&gt; =  − 0.55, &lt;jats:italic&gt;p&lt;/jats:italic&gt; &amp;lt; 0.001), and the UPDRS-Swallowing-Item-Score (&lt;jats:italic&gt;r&lt;/jats:italic&gt; = 0.42, &lt;jats:italic&gt;p&lt;/jats:italic&gt; &amp;lt; 0.001). Further, the DIGEST-FEES-safety subscore correlated with the PAS (&lt;jats:italic&gt;r&lt;/jats:italic&gt; = 0.63, &lt;jats:italic&gt;p&lt;/jats:italic&gt; &amp;lt; 0.001). Inter-rater reliability was high for the overall DIGEST-FEES rating (quadratic weighted kappa of 0.82). Therefore, DIGEST-FEES is a valid and reliable score to evaluate overall pharyngeal dysphagia severity in PD. Nevertheless, the modified Delphi survey identified domains where DIGEST-FEES may need to be specifically adapted to PD or neurological collectives in the future.&lt;/jats:p&gt

Dysphagia as Isolated Manifestation of Jo-1 Associated Myositis?

Dysphagia can be predominant or sole symptom of myositis. However, diagnostic evaluation is difficult in such cases. Here, we present evidence for dysphagia as sole manifestation of Jo-1 associated myositis. A 77-year-old patient suffering from isolated dysphagia was assessed by flexible endoscopic evaluation of swallowing, videofluoroscopy, high resolution esophageal manometry, whole body muscle MRI, electroneurographic and electromyographic examination, cerebrospinal fluid analysis, screening for autoantibodies, and body plethysmography. We detected isolated oropharyngeal dysphagia including a decreased pressure of the upper esophageal sphincter leading to cachexia in an anti-Jo-1 positive patient without any abnormalities in the other diagnostics. Immunosuppressive therapy with cortisone and azathioprine led to long-term improvement of dysphagia. This is the first report of isolated dysphagia as manifestation of Jo-1 associated myositis. Therefore, Jo-1 associated myositis should be considered as a possible differential diagnosis for isolated dysphagia. Typical signs for myositis in instrumental dysphagia assessment are presented

Standardized Endoscopic Swallowing Evaluation for Tracheostomy Decannulation in Critically Ill Neurologic Patients – a prospective evaluation

Abstract Background Removal of a tracheostomy tube in critically ill neurologic patients is a critical issue during intensive care treatment, particularly due to severe dysphagia and insufficient airway protection. The “Standardized Endoscopic Evaluation for Tracheostomy Decannulation in Critically Ill Neurologic Patients” (SESETD) is an objective measure of readiness for decannulation. This protocol includes the stepwise evaluation of secretion management, spontaneous swallowing, and laryngeal sensitivity during fiberoptic endoscopic evaluation of swallowing (FEES). Here, we first evaluated safety and secondly effectiveness of the protocol and sought to identify predictors of decannulation success and decannulation failure. Methods A prospective observational study was conducted in the neurological intensive care unit at Münster University Hospital, Germany between January 2013 and December 2017. Three hundred and seventy-seven tracheostomized patients with an acute neurologic disease completely weaned from mechanical ventilation were included, all of whom were examined by FEES within 72 h from end of mechanical ventilation. Using regression analysis, predictors of successful decannulation, as well as decannulation failure were investigated. Results Two hundred and twenty-seven patients (60.2%) could be decannulated during their stay according to the protocol, 59 of whom within 24 h from the initial FEES after completed weaning. 3.5% of patients had to be recannulated due to severe dysphagia or related complications. Prolonged mechanical ventilation showed to be a significant predictor of decannulation failure. Lower age was identified to be a significant predictor of early decannulation after end of weaning. Transforming the binary SESETD into a 4-point scale helped predicting decannulation success in patients not immediately ready for decannulation after the end of respiratory weaning (optimal cutoff ≥1; sensitivity: 64%, specifity: 66%). Conclusions The SESETD showed to be a safe and efficient tool to evaluate readiness for decannulation in our patient collective of critically ill neurologic patients. </jats:sec