Diagnosis and treatment of neurogenic dysphagia – S1 guideline of the German Society of Neurology

Dziewas et al. Neurological Research and Practice https://doi.org/10.1186/s42466-021-00122-3 (2021) 3:23 Neurological Research and Practice GUIDELINES Open Access Diagnosis and treatment of neurogenic dysphagia – S1 guideline of the German Society of Neurology Rainer Dziewas1,2* , Hans-Dieter Allescher3, Ilia Aroyo4, Gudrun Bartolome5, Ulrike Beilenhoff6, Jörg Bohlender7, Helga Breitbach-Snowdon8, Klemens Fheodoroff9, Jörg Glahn10, Hans-Jürgen Heppner11, Karl Hörmann12, Christian Ledl13, Christoph Lücking14, Peter Pokieser15, Joerg C. Schefold16, Heidrun Schröter-Morasch17, Kathi Schweikert18, Roland Sparing19, Michaela Trapl-Grundschober20, Claus Wallesch21, Tobias Warnecke1, Cornelius J. Werner22, Johannes Weßling23, Rainer Wirth24 and Christina Pflug25 Abstract Introduction: Neurogenic dysphagia defines swallowing disorders caused by diseases of the central and peripheral nervous system, neuromuscular transmission, or muscles. Neurogenic dysphagia is one of the most common and at the same time most dangerous symptoms of many neurological diseases. Its most important sequelae include aspiration pneumonia, malnutrition and dehydration, and affected patients more often require long-term care and are exposed to an increased mortality. Based on a systematic pubmed research of related original papers, review articles, international guidelines and surveys about the diagnostics and treatment of neurogenic dysphagia, a consensus process was initiated, which included dysphagia experts from 27 medical societies. Recommendations: This guideline consists of 53 recommendations covering in its first part the whole diagnostic spectrum from the dysphagia specific medical history, initial dysphagia screening and clinical assessment, to more refined instrumental procedures, such as flexible endoscopic evaluation of swallowing, the videofluoroscopic swallowing study and high-resolution manometry. In addition, specific clinical scenarios are captured, among others the management of patients with nasogastric and tracheotomy tubes. The second part of this guideline is dedicated to the treatment of neurogenic dysphagia. Apart from dietary interventions and behavioral swallowing treatment, interventions to improve oral hygiene, pharmacological treatment options, different modalities of neurostimulation as well as minimally invasive and surgical therapies are dealt with. Conclusions: The diagnosis and treatment of neurogenic dysphagia is challenging and requires a joined effort of different medical professions. While the evidence supporting the implementation of dysphagia screening is rather convincing, further trials are needed to improve the quality of evidence for more refined methods of dysphagia diagnostics and, in particular, the different treatment options of neurogenic dysphagia. The present article is an abridged and translated version of the guideline recently published online (https://www.awmf.org/uploads/tx_ szleitlinien/030-111l_Neurogene-Dysphagie_2020-05.pdf). * Correspondence: 1 Klinik für Neurologie, Universitätsklinik Münster, 48149 Münster, Germany 2 Klinik für Neurologie und Neurologische Frührehabilitation, Klinikum Osnabrück, Am Finkenhügel 1, 49076 Osnabrück, Germany Full list of author information is available at the end of the article © The Author(s). 2021 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. Dziewas et al. Neurological Research and Practice (2021) 3:23 Introduction The present article is an abridged and translated version of the guideline recently published online (https://www. awmf.org/uploads/tx_szleitlinien/030-111l_NeurogeneDysphagie_2020-05.pdf). The act of swallowing is a highly complex neuromuscular process that requires precise bilateral coordination of more than 25 muscle pairs. Using different imaging techniques, numerous physiological studies have consistently demonstrated that apart from the well-established role of the brain stem, different cortical areas are involved in the modulation of swallowing. Based on these findings, reorganization mechanisms have been further explored and form the neuroscientific basis for treatment approaches using different neurostimulation modalities. Neurogenic dysphagia defines swallowing disorders caused by diseases of the CNS, PNS, neuromuscular transmission, or muscles. In contrast to this uniformity suggestive term, swallowing disorders caused by specific diseases differ considerably in terms of their clinical presentation, the respective therapeutic options, and the prognosis. Dysphagia is one of the most common and at the same time most dangerous symptoms of many neurological diseases. Impaired deglutition is initially found in at least 50% of all patients with ischemic or hemorrhagic stroke [1]. Affected patients have a 4 times increased risk of aspiration pneumonia, suffer more often from a long-lasting severe disability, are more often discharged to nursing homes, and also show significantly increased mortality [2]. Comparable numbers have been published for traumatic brain injury with a reported incidence of clinically relevant dysphagia in about 60% of patients [3]. In this patient collective, the presence of dysphagia is associated with a significantly extended time on mechanical ventilation and a longer need for artificial nutrition. In all Parkinson syndromes, neurogenic dysphagia is also a major risk factor for pneumonia, which is the leading cause of death in these patients [4, 5]. Furthermore, swallowing disorders in these patients are associated with a reduced quality of life, insufficient drug effects, and malnutrition [6, 7]. 2030% of patients with dementia have severe dysphagia with silent aspiration that goes unnoticed by the patients [8]. Dysphagia is also a prominent clinical feature in various neuromuscular diseases. Up to 30% of patients with amyotrophic lateral sclerosis present with impaired swallowing at diagnosis [9] and practically all of them develop dysphagia as the disease progresses. Myasthenia Gravis manifests itself in 15% of cases with swallowing impairments. As the illness progresses, over 50 % of all patients are affected, and in more than 50 % of cases, a myasthenic crisi (...truncated)