Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| University Hospital Muenster | OTHER |
| Medical University of Warsaw | OTHER |
| Hannover Medical School | OTHER |
| University of Ulm |
Not provided
Not provided
Not provided
Not provided
This is a non-interventional observational study designed to systematically record the results of routine laryngeal examinations and specific characteristics of dysphagia in patients with neurodegenerative disorders. The results of a fiberoptic / flexible endoscopic evaluation of swallowing (FEES) while performing a structured task protocol will be recorded. If available, laryngeal electromyography (EMG) results will also be recorded. In addition to the examination results, demographic and disease-specific data are collected, and two questionnaires, the Swallowing Disturbance Questionnaire for Parkinson's Disease (SDQ-PD) and the swallowing specific Quality Of Life Questionnaire (SWALQOL), are administered.
Neurodegenerative disorders are associated with a high prevalence of neurogenic dysphagia. Depending on the underlying disease, the prevalence can affect up to 80% of patients. Dysphagia is associated with dehydration, malnutrition, facilitates aspiration pneumonia and thereby determines the prognosis of neurodegenerative diseases. For most of them, dysphagia-associated complications are the leading cause of death.
Multiple system atrophy (MSA) is a sporadic progressive neurodegenerative disorder caused by oligodendroglial aggregation of α-synuclein affecting predominantly the nigrostriatal, olivo-ponto-cerebellar, and autonomic systems,resulting in a clinical presentation of dysautonomia combined with either predominantly parkinsonian (MSA-P) or cerebellar (MSA-C) symptoms of varying severity.In its early stage, the diagnosis of MSA according to the second consensus criteria can be challenging. Therefore, the Movement Disorders Society MSA study group recently addressed the importance of developing valuable diagnostic tools for securing an early diagnosis in patients with MSA not only to estimate disease prognosis but also to early initiate novel, potentially disease-modifying treatments in clinical trials. Despite laryngopharyngeal dysfunction being associated with decreased life expectancy and quality of life, systematic assessment of these functions in MSA is scarce. Previously, an easy-to-implement MSA-FEES task-protocol was suggested to systematically assess laryngopharyngeal function.
A pilot study on 8 patients with MSA not only showed that the task protocol was feasible and well tolerated, but also that laryngopharyngeal symptoms where highly prevalent despite the lack of clinical presentation (Warnecke et. al 2019). Moreover, irregular arytenoid cartilages movements where present in all MSA-patients when performing this task protocol, suggesting this symptom could serve as a clinical marker to identify MSA-patients. Following this pilot study, an observational two center study assessed 57 MSA patients with this protocol and compared findings to an age-matched cohort of PD-patients (Gandor et al. 2020). While only 43.9% of MSA patients had clinical symptoms of laryngeal dysfunction, 93% showed laryngeal abnormalities during FEES performing the task-protocol. 91.2% of MSA-patients showed irregular arytenoid cartilages movements. In contrast, only one PD patient showed laryngeal abnormalities with vocal fold motion impairment, but not irregular arytenoid cartilages movements. This study suggests that irregular arytenoid cartilages movements allow differentiating MSA from PD with a sensitivity of 0.9 and a specificity of 1.0.
4repeat tauopathies (4RT) are caused by an intraneuronal accumulation of 4repeat tau.
4RT, also known as progressive supranuclear palsy (PSP) with all its clinical subtypes, is a rapidly progressive neurodegenerative disease that leads to increasing impairment of cortical and subcortical function in the affected person due to the accumulation of tau protein in the brain (Höglinger 2017). Due to the clinical variability of the presentation, early diagnostic certainty is desirable. Depending on the affected area in the central nervous system, different clinical phenotypes result. The most commonly described and researched entity is Progressive Supranuclear P, also known as Richardson Syndrome, or PSP-RS. Further clinical presentations include a Parkinsonian variant (PSP-P), corticobasal syndrome (PSP-CBS), pure akinesia with gait freezing (PSP-PAGF), speech/language dominant presentations (PSP-SL), frontotemproal dementia variants (PSP-FTD), and cerebellar presentations (PSP-C) (Höglinger 2017).
4RT is also associated with swallowing and speech problems, and aspiration pneumonia is one of the most common causes of death in this disease entity (Tilley 2016). In addition, dystonic dysinnervation of laryngeal muscles can lead to laryngeal motion abnormalities (Panegyres 2007).
Equally, patients with motor neurone disease (MND) are affected by dysphagia early in the disease. In a FEES study by Steven B. Leder and colleagues (2004), which included 17 ALS patients subjectively complaining of dysphagia, fluid aspiration or an increased risk of fluid aspiration was found in almost 60% of cases, regardless of whether the disease initially mainly affected the bulbar musculature or the limb musculature. As the disease progressed, the depth of penetration of fluids into the hypopharynx prior to triggering the swallowing reflex increased, so these patients were advised to thicken fluids. Initially, residues of solid food consistencies were detected in the valleculae, piriform sinus and/or laryngeal inlet in three patients after swallowing. As a result of progressive paresis of the pharyngeal muscles, the residuals increased in the ALS patients examined during the course of the disease. If this disorder pattern was detected, it was advised to reduce the size of the food bolus and to clear the pharynx by swallowing water or reswallowing several times. In a further small case series (n = 11), the FEES showed that dysphagic ALS patients are particularly at risk of penetration/aspiration when swallowing liquids. Penetration and aspiration occur particularly frequently intradeglutitively (D'Ottaviano et al. 2013). A recent publication reports on FEES examinations in 202 ALS patients (w/m 95/107; bulbar/spinal onset 66/136; mean age 64.68 +/- 11.12 years). A close positive correlation with disease severity (measured using the ALSFRS-R) was found for all FEES parameters (leaking, aspiration, residuals), regardless of the three consistencies tested (liquid, semi-solid, solid) (Fattori et al. 2017).
The aim of this FEEMSA trial is to continue recruitment of patients with neurodegenerative diseases and systematically assess laryngopharyngeal function in large cohorts, to categorise the dysphagia phenotypes and better correlate them with the disease subtypes (MSA Parkinson vs cerebellar; PSP variants, MND variants, etc). If available, laryngeal EMG will also be recorded.
Not provided
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Multiple System Atrophy | Patients diagnosed will probable or possible MSA according to the 2nd criteria for the diagnosis of MSA (Gilman 2008) that received laryngopharyngeal assessment according to the systematic task protocol during FEES (Warnecke et al. 2019). | ||
| progressive supranuclear palsy | Patients diagnosed will probable or possible Progressive Supranuclear Palsy or (PSP) related 4repeat tauopathies according to the Movement Disorders Society diagnostic criteria (Höglinger 2017) that received laryngopharyngeal assessment according to the systematic task protocol during FEES (Warnecke et al. 2019). | ||
| Parkinson Disease | Patients diagnosed will Parkinson's disease according to the Movement Disorders Society diagnostic criteria (Postuma 2015) that received laryngopharyngeal assessment according to the systematic task protocol during FEES (Warnecke et al. 2019). | ||
| Motor Neuron Disease | Patients diagnosed with Motor Neuron Disease that received laryngopharyngeal assessment according to the systematic task protocol during FEES (Warnecke et al. 2019). | ||
| Neurodegenerative Diseases | Patients diagnosed with a neurodegenerative disease not specified above that received laryngopharyngeal assessment according to the systematic task protocol during FEES (Warnecke et al. 2019). |
Not provided
| Measure | Description | Time Frame |
|---|---|---|
| laryngeal movement disorders | occurrence of vocal fold motion impairment, paradoxical vocal fold motion, vocal fold fixation or involuntary irregular arytenoid cartilages movements when assessed with the task-protocol | 1 day |
| dysphagia | occurrence of dysphagic symptoms when assessed with the task-protocol | 1 day |
Not provided
| Measure | Description | Time Frame |
|---|---|---|
| laryngeal EMG findings | abnormalities recorded on laryngeal EMG showing denervation, dystonic co-activation or myoclonic discharges | 1 day |
Inclusion Criteria:
AND underwent laryngopharyngeal assessment according to the systematic task protocol during FEES (Warnecke et al. 2019).
Exclusion Criteria:
- Patients who do not sign the consent form
Not provided
Not provided
Not provided
Patients will be recruited at the participating sites from outpatient's clinics or admitted patients.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Florin Gandor, MD | Contact | +493320422781 | 22781 | gandor@kliniken-beelitz.de |
| Tobias Warnecke, MD | Contact | tobias.warnecke@klinikum-os.de |
| Name | Affiliation | Role |
|---|---|---|
| Florin Gandor, MD | Movement Disorders Hospital Beelitz-Heilstätten, Germany | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Department of Neurology and Department of ENT, Medical University Innsbruck | Recruiting | Innsbruck | Tyrol | 6020 | Austria |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 30972002 | Background | Warnecke T, Vogel A, Ahring S, Gruber D, Heinze HJ, Dziewas R, Ebersbach G, Gandor F. The Shaking Palsy of the Larynx-Potential Biomarker for Multiple System Atrophy: A Pilot Study and Literature Review. Front Neurol. 2019 Mar 26;10:241. doi: 10.3389/fneur.2019.00241. eCollection 2019. | |
| 32757231 | Background |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| OTHER |
| Medical University Innsbruck | OTHER |
| University Hospital Carl Gustav Carus | OTHER |
| University of Barcelona | OTHER |
| Seoul National University Hospital | OTHER |
| Gifu University Graduate School of Medicine | OTHER |
| University of Bologna | OTHER |
| Tel Aviv University | OTHER |
| Asklepios Kliniken Hamburg GmbH | OTHER |
| Asklepios Fachklinikum Stadtroda | OTHER |
| Klinikum OsnabrĂĽck | UNKNOWN |
Not provided
Not provided
Not provided
| Department of Neurology, Medical University of Ulm | Recruiting | Ulm | Baden-Wurttemberg | 89081 | Germany |
|
| Movement Disorders Hospital - Kliniken Beelitz | Recruiting | Beelitz-Heilstätten | Brandenburg | 14547 | Germany |
|
| Department of Neurology, Medical School Hannover | Recruiting | Hanover | Lower Saxony | 30625 | Germany |
|
| Department of Neurology, University Hospital MĂĽnster | Recruiting | MĂĽnster | North Rhine-Westphalia | 48149 | Germany |
|
| Department of Neurology, University Hospital Carl Gustav Carus | Recruiting | Dresden | Saxony | 01307 | Germany |
|
| Department of Neueology Asklepios Klinik Stadtroda | Recruiting | Stadtroda | Thuringia | 07646 | Germany |
|
| Asklepios Fachklinikum Stadtroda | Recruiting | Stadtroda | Thuringia | Germany |
|
| Asklepios Klinik Barmbek | Recruiting | Hamburg | 22291 | Germany |
|
| Department of Neurology Asklepios Klinik Barmbek | Recruiting | Hamburg | 22307 | Germany |
|
| Department of Neurology, Movement Disorders Unit, Medical Center Tel Aviv | Recruiting | Tel Aviv | 64239 | Israel |
|
| IRCCS Istituto delle Scienze Neurologiche, Azienda USL di Bologna | Recruiting | Bologna | 40124 | Italy |
|
| Department of Neurology, Gifu University Graduate School of Medicine | Recruiting | Gifu | 501-1194 | Japan |
|
| Department of Neurology, Medical University Warsaw | Recruiting | Warsaw | 02-091 | Poland |
|
| Department of Neurology SNUCM | Recruiting | Seoul | South Korea |
|
| Unidad de Parkinson y Trastornos del Movimiento Instituto ClĂnic de Neurociencias, Hospital Clinic de Barcelona | Recruiting | Barcelona | Catalonia | 08036 | Spain |
|
| Gandor F, Vogel A, Claus I, Ahring S, Gruber D, Heinze HJ, Dziewas R, Ebersbach G, Warnecke T. Laryngeal Movement Disorders in Multiple System Atrophy: A Diagnostic Biomarker? Mov Disord. 2020 Dec;35(12):2174-2183. doi: 10.1002/mds.28220. Epub 2020 Aug 5. |
| 34773420 | Background | Vogel A, Claus I, Ahring S, Gruber D, Haghikia A, Frank U, Dziewas R, Ebersbach G, Gandor F, Warnecke T. Endoscopic Characteristics of Dysphagia in Multiple System Atrophy Compared to Parkinson's Disease. Mov Disord. 2022 Mar;37(3):535-544. doi: 10.1002/mds.28854. Epub 2021 Nov 13. |
| ID | Term |
|---|---|
| D019578 | Multiple System Atrophy |
| D010300 | Parkinson Disease |
| D013494 | Supranuclear Palsy, Progressive |
| D016472 | Motor Neuron Disease |
| D019636 | Neurodegenerative Diseases |
| D003680 | Deglutition Disorders |
| ID | Term |
|---|---|
| D054969 | Primary Dysautonomias |
| D001342 | Autonomic Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D001480 | Basal Ganglia Diseases |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009069 | Movement Disorders |
| D000080874 | Synucleinopathies |
| D020734 | Parkinsonian Disorders |
| D009886 | Ophthalmoplegia |
| D015835 | Ocular Motility Disorders |
| D003389 | Cranial Nerve Diseases |
| D024801 | Tauopathies |
| D010243 | Paralysis |
| D009461 | Neurologic Manifestations |
| D005128 | Eye Diseases |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D009468 | Neuromuscular Diseases |
| D004935 | Esophageal Diseases |
| D005767 | Gastrointestinal Diseases |
| D004066 | Digestive System Diseases |
| D010608 | Pharyngeal Diseases |
| D010038 | Otorhinolaryngologic Diseases |
Not provided
Not provided