Paul Muhle

Isolated dysphagia as initial sign of anti-IgLON5 syndrome

Objective: To report on dysphagia as initial sign in a case of anti-IgLON5 syndrome and provide an overview of the current literature. Methods: The diagnostic workup included cerebral MRI, fiber optic endoscopic evaluation of swallowing (FEES) with the FEES tensilon test, a videofluoroscopic swallowing study, evoked potentials and peripheral nerve conduction studies, polysomnography, lumbar puncture, and screening for neural autoantibodies. A systematic review of all published cases of IgLON5 syndrome is provided. Results: We report a case of anti-IgLON5 syndrome presenting with slowly progressive neurogenic dysphagia. FEES revealed severe neurogenic dysphagia and bilateral palsy of the vocal cords. Autoantibody screening was positive for IgLON5 IgG (+++, 1:1,000) serum levels but no other known neural autoantibody. Polysomnography was highly suggestive of non-REM parasomnia. Symptoms were partially responsive to immunotherapy. Conclusions: Slowly progressive neurogenic dysphagia may occur as initial sign of anti-IgLON5 syndrome highlighting another clinical presentation of this rare disease.

[Age-related changes in swallowing. Physiology and pathophysiology].

The term presbyphagia refers to all changes of swallowing physiology that are manifested with increasing age. Alterations in the pattern of deglutition that are part of healthy aging are called primary presbyphagia. Primary presbyphagia is not an illness in itself but contributes to a more pervasive naturally diminished functional reserve, making older adults more susceptible to dysphagia. If disorders in swallowing occur in the elderly as a comorbidity of a specific disease, for example stroke or neurodegenerative disorders, this is called secondary presbyphagia. Increasing age has an impact on each stage of deglutition. In the oral preparatory phase a diminished input for smell and taste as well as a usually multifactorial cause of dry mouth are the most important influencing factors. Sarcopenia, the degenerative loss of skeletal muscle mass, strength and quality associated with aging, interferes in particular with the oropharyngeal phase. A decreased sensory feedback from the oropharyngeal mucosa leads to a delayed triggering of the swallowing reflex. Finally, a reduction in connective tissue elasticity and changes of the axial skeleton lead to various modifications of the swallowing pattern with advanced age.

Schluckstörungen im Alter

The term presbyphagia refers to all changes of swallowing physiology that are manifested with increasing age. Alterations in the pattern of deglutition that are part of healthy aging are called primary presbyphagia. Primary presbyphagia is not an illness in itself but contributes to a more pervasive naturally diminished functional reserve, making older adults more susceptible to dysphagia. If disorders in swallowing occur in the elderly as a comorbidity of a specific disease, for example stroke or neurodegenerative disorders, this is called secondary presbyphagia. Increasing age has an impact on each stage of deglutition. In the oral preparatory phase a diminished input for smell and taste as well as a usually multifactorial cause of dry mouth are the most important influencing factors. Sarcopenia, the degenerative loss of skeletal muscle mass, strength and quality associated with aging, interferes in particular with the oropharyngeal phase. A decreased sensory feedback from the oropharyngeal mucosa leads to a delayed triggering of the swallowing reflex. Finally, a reduction in connective tissue elasticity and changes of the axial skeleton lead to various modifications of the swallowing pattern with advanced age.

Randomized trial of transcranial direct current stimulation for poststroke dysphagia

We evaluated whether transcranial direct current stimulation (tDCS) is able to enhance dysphagia rehabilitation following stroke. Besides relating clinical effects with neuroplastic changes in cortical swallowing processing, we aimed to identify factors influencing treatment success.

Increase of Substance P Concentration in Saliva after Pharyngeal Electrical Stimulation in Severely Dysphagic Stroke Patients – an Indicator of Decannulation Success?

Background/Aims: Substance P (SP) is a neuropeptide, likely acting as a neurotransmitter in the pharyngeal mucosa enhancing the swallow and cough reflex. Pharyngeal Electrical Stimulation (PES) induces a temporary increase of salivary SP levels in healthy adults. Previous evidence suggests that post-stroke dysphagia is related to reduced SP levels. Here, we investigated the effects of PES on SP levels in severely dysphagic stroke patients and a possible link between increase of SP and treatment success. Methods: 23 tracheotomized stroke patients who could not be decannulated due to severe and persisting dysphagia according to endoscopic evaluation received PES for 10 minutes a day over three consecutive days in this prospective single-center study. If initial treatment failed, repetitive stimulation cycles were provided. Saliva samples were collected before and directly after each PES. Results: 61% of participants were decannulated after the first treatment cycle. Increase of SP levels post-stimulation was closely related to treatment success, i.e. decannulation with 79% of successfully treated patients showing increase of SP, whereas 89% of unsuccessfully treated patients had stable or decreased SP levels. Applying logistic regression analysis, increase of SP level remained the only independent predictor of decannulation after PES. All 3 repetitively treated patients showed increased SP levels when progressing from the 1st to the 2nd cycle, two of whom were decannulated hereafter. Conclusions: The physiological mechanism of PES may consist in restoration of sensory feedback, which is known to be crucial for the execution of a safe swallow. SP possibly acts as a biomarker for indicating response to PES.

Measurement of Oxygen Desaturation Is Not Useful for the Detection of Aspiration in Dysphagic Stroke Patients

Background: Dysphagia is one of the most dangerous symptoms of acute stroke. Various screening tools have been suggested for the early detection of this condition. In spite of conflicting results, measurement of oxygen saturation (SpO2) during clinical swallowing assessment is still recommended by different national guidelines as a screening tool with a decline in SpO2 ≥2% usually being regarded as a marker of aspiration. This paper assesses the sensitivity of SpO2 measurements for the evaluation of aspiration risk in acute stroke patients. Methods: Fifty acute stroke patients with moderate to severe dysphagia were included in this study. In all patients, fiberoptic endoscopic evaluation of swallowing (FEES) was performed according to a standardised protocol. Blinded to the results of FEES, SpO2 was monitored simultaneously. The degree of desaturation during/after swallows with aspiration was compared to the degree of desaturation during/after swallows without aspiration in a swallow-to-swallow analysis of each patient. To minimise potential confounders, every patient served as their control. Results: In each subject, a swallow with and a swallow without aspiration were analysed. Overall, aspiration seen in FEES was related to a minor decline in SpO2 (mean SpO2 without aspiration 95.54 ± 2.7% vs. mean SpO2 with aspiration 95.28 ± 2.7%). However, a significant desaturation ≥2% occurred only in 5 patients during/after aspiration. There was no correlation between aspiration/dysphagia severity or the amount of aspirated material and SpO2 levels. Conclusions: According to this study, measurement of oxygen desaturation is not a suitable screening tool for the detection of aspiration in stroke patients.

Introducing a Virtual Lesion Model of Dysphagia Resulting from Pharyngeal Sensory Impairment

Background/Aims: Performing neurophysiological and functional imaging studies in severely affected patients to investigate novel neurostimulation techniques for the treatment of neurogenic dysphagia is difficult. Therefore, basic research needs to be conducted in healthy subjects. Swallowing is a motor function highly dependent on sensory afferent input. Here we propose a virtual peripheral sensory lesion model to mimic pharyngeal sensory impairment, which is known as a major contributor to dysphagia in neurological disease. Methods: In this randomized crossover study on 11 healthy volunteers, cortical activation during pneumatic pharyngeal stimulation was measured applying magnetoencephalography in two separate sessions, with and without pharyngeal surface anesthesia. Results: Stimulation evoked bilateral event-related desynchronization (ERD) mainly in the caudolateral pericentral cortex. In comparison to the no-anesthesia condition, topical anesthesia led to a reduction of ERD in beta (13-30 Hz) and low gamma (30-60 Hz) frequency ranges (p<0.05) in sensory but also motor cortical areas. Conclusions: Withdrawal of sensory afferent information by topical anesthesia leads to reduced response to pneumatic pharyngeal stimulation in a distributed cortical sensorimotor network in healthy subjects. The proposed paradigm may serve to investigate the effect of neuromodulatory treatments specifically on pharyngeal sensory impairment as relevant cause of neurogenic dysphagia.

Subtle Esophageal Motility Alterations in Parkinsonian Syndromes: Synucleinopathies vs. Tauopathies: Parkinsonian Esophageal Motility Alterations

Esophageal dysfunction is a frequent phenomenon in Parkinsons disease during all disease stages, but data about esophageal involvement in atypical parkinsonian syndromes as well as possible differences between alpha‐synucleinopathies and tauopathies, including causative links to the origin of the dysfunction, are lacking so far.

Predicting Dysphagia with National Institute of Health Stroke Scale: Distinction between Infra- and Supratentorial Region is Essential

Background: For the early detection of post-stroke dysphagia (PSD), valid screening parameters are crucial as part of a step-wise diagnostic procedure. This study examines the role of the National Institute of Health Stroke Scale (NIH-SS) as a potential low-threshold screening parameter. Methods: During a ten-year period, 687 newly admitted patients at University Hospital Muenster were included in a retrospective analysis, if they had ischemic or haemorrhagic stroke confirmed by neuroimaging and had received NIH-SS scoring and endoscopic swallowing evaluation upon admission. The NIH-SS score was correlated with dysphagia severity as measured by the validated 6-point fiberoptic endoscopic dysphagia severity score (FEDSS), and the ideal cut-off score to predict PSD, defined as FEDSS > 1, was calculated. Supra- and infratentorial strokes were analysed separately due to their differing role in the pathophysiology of neurogenic dysphagia. Results: NIH-SS and dysphagia severity show a significant positive correlation in the whole study population (R2 = 0.745) as well as in both analysed subgroups (R2 = 0.494 for supra- and R2 = 0.646 for infratentorial strokes, p < 0.0005, respectively). For supratentorial strokes, the ideal NIH-SS cut-off is > 9 (sensitivity 68.3%, specificity 61.5%, positive predictive value 89.7%, negative predictive value 28.4%). For infratentorial strokes, a lower ideal cut-off > 5 was calculated (sensitivity 67.4%, specificity 85.0%, positive predictive value 95.1%, negative predictive value 37.8%). Conclusions: NIH-SS may be used as an adjunct to predict dysphagia in acute stroke patients with moderate sensitivity and specificity. Differentiation between supra- and infratentorial regions is essential not to miss dysphagia in infratentorial stroke.

The Effect of Improved Dysphagia Care on Outcome in Patients with Acute Stroke: Trends from 8-Year Data of a Large Stroke Register

Background: Early dysphagia screening and appropriate management are recommended by current guidelines to reduce complications and case fatality in acute stroke. However, data on the potential benefit of changes in dysphagia care on patient outcome are limited. Our objective was to assess the degree of implementation of dysphagia guidelines and determine the impact of modifications in dysphagia screening and treatment practices on disease complications and outcome in stroke patients over time. Methods: In this prospective register-based study (“Stroke Register of Northwestern Germany”), all adult stroke patients admitted to 157 participating hospitals between January, 2008 and December, 2015 were included (n = 674,423). Dysphagia incidence upon admission, the proportion of patients receiving a standardized swallowing screening, and the percentage of dysphagic patients being referred to a speech language therapist (SLT) for treatment were obtained per year. Pneumonia rate, modified Rankin Scale (mRS) at discharge, and in-hospital mortality were compared between groups of dysphagic vs. non-dysphagic patients over time. Results: Screening proportions continuously increased from 47.2% in 2008 to 86.6% in 2015. But the proportion diagnosed with dysphagia remained stable with about 19%. The number of dysphagic patients receiving SLT treatment grew from 81.6 up to 87.0%. Pneumonia incidence was higher in dysphagic stroke cases (adjusted OR 5.4 [5.2–5.5], p < 0.001), accompanied by a worse mRS at discharge (adjusted OR for mRS ≥3: 3.1 [3.0–3.1], p < 0.001) and higher mortality (adjusted OR 3.1 [3.0–3.2], p < 0.001). The order of magnitude of these end points did not change over time. Conclusion: Although advances have been made in dysphagia care, prevalent screening and treatment practices remain insufficient to reduce pneumonia rate, improve functional outcome, and decrease case fatality in dysphagic stroke patients. More research is urgently needed to develop more effective swallowing therapies.