Mario Prosiegel

Bulbar symptoms such as impaired swallowing and speech impairment are frequent features of amyotrophic lateral sclerosis and can markedly reduce life expectancy and quality of life. In this article, Kühnlein et al. highlight the importance of early detection, as well as appropriate treatment and management, of bulbar symptoms in patients with amyotrophic lateral sclerosis, emphasizing the benefits of a multidisciplinary approach. Amyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disease of the motor system. Bulbar symptoms such as dysphagia and dysarthria are frequent features of ALS and can result in reductions in life expectancy and quality of life. These dysfunctions are assessed by clinical examination and by use of instrumented methods such as fiberendoscopic evaluation of swallowing and videofluoroscopy. Laryngospasm, another well-known complication of ALS, commonly comes to light during intubation and extubation procedures in patients undergoing surgery. Laryngeal and pharyngeal complications are treated by use of an array of measures, including body positioning, compensatory techniques, voice and breathing exercises, communication devices, dietary modifications, various safety strategies, and neuropsychological assistance. Meticulous monitoring of clinical symptoms and close cooperation within a multidisciplinary team (physicians, speech and language therapists, occupational therapists, dietitians, caregivers, the patients and their relatives) are vital.

Stroke is the most frequent cause of neurogenic oropharyngeal dysphagia (NOD). In the acute phase of stroke, the frequency of NOD is greater than 50% and, half of this patient population return to good swallowing within 14 days while the other half develop chronic dysphagia. Because dysphagia leads to aspiration pneumonia, malnutrition, and in-hospital mortality, it is important to pay attention to swallowing problems. The question arises if a prediction of severe chronic dysphagia is possible within the first 72 hours of acute stroke.On admission to the stroke unit, all stroke patients were screened for swallowing problems by the nursing staff within 2 hours. Patients showing signs of aspiration were included in the study (n = 114) and were given a clinical swallowing examination (CSE) by the swallowing/speech therapist within 24 hours and a swallowing endoscopy within 72 hours by the physician. The primary outcome of the study was the functional communication measure (FCM) of swallowing (score 1-3, tube feeding dependency) on day 90.The grading system with the FCM swallowing and the penetration-aspiration scale (PAS) in the first 72 hours was tested in a multivariate analysis for its predictive value for tube feeding-dependency on day 90. For the FCM level 1 to 3 (P < .0022) and PAS level 5 to 8 (P < .00001), the area under the curve (AUC) was 72.8% and showed an odds ratio of 11.8 (P < .00001; 95% confidence interval 0.036-0.096), achieving for the patient a 12 times less chance of being orally fed on day 90 and therefore still being tube feeding-dependent.We conclude that signs of aspiration in the first 72 hours of acute stroke can predict severe swallowing problems on day 90. Consequently, patients should be tested on admission to a stroke unit and evaluated with established dysphagia scales to prevent aspiration pneumonia and malnutrition. A dysphagia program can lead to better communication within the stroke unit team to initiate the appropriate diagnostics and swallowing therapy as soon as possible.

The World Health Organization's International Classification of Functioning, Disability and Health (ICF) is gaining recognition in physical therapy. The Extended ICF Core Set for Stroke is a practical tool that represents a selection of categories from the whole classification and can be used along with the ICF qualifier scale to describe patients' functioning and disability following stroke. The application of the ICF qualifier scale poses the question of interrater reliability. The primary objective of this investigation was to study the agreement between physical therapists' ratings of subjects' functioning and disability with the Extended ICF Core Set for Stroke and with the ICF qualifier scale. Further objectives were to explore the relationships between agreement and rater confidence and between agreement and physical therapists' areas of core competence.A monocentric, cross-sectional reliability study was conducted. A consecutive sample of 30 subjects after stroke participated. Two physical therapists rated the subjects' functioning in 166 ICF categories.The interrater agreement of the 2 physical therapists was moderate across all judgments (observed agreement=51%, kappa=.41). Interrater reliability was not related to rater confidence or to the physical therapists' areas of core competence.The present study suggests potential improvements to enhance the implementation of the ICF and the Extended ICF Core Set for Stroke in practice. The results hint at the importance of the operationalization of the ICF categories and the standardization of the rating process, which might be useful in controlling for rater effects and increasing reliability.

Neuroacanthocytosis syndromes are a group of rare disorders characterized by neurodegeneration and thorny erythrocytes.1 Chorea-acanthocytosis (ChAc, OMIM 200150) is the main entity within this group and is one differential diagnosis of Huntington's disease (HD).1 Besides VPS13A sequencing2 demonstration of absence of its protein product (chorein) on Western blot3 can be used alternatively. In early stages, the phenotype often comprises dysphagia and tongue dystonia4 and appears quite indicative of ChAc. Here, we report two cases in which action-induced tongue protrusion was a striking and early finding. Case 1: This 30-year-old woman presented with orofacial chorea, dysphagia, dysarthria, and vocalizations. At age 28, after repeatedly biting her cheeks and tongue, she was diagnosed with borderline personality disorder. She had two generalized tonic-clonic seizures at age 29. On examination, she showed chorea of the limbs and reduced ankle reflexes. Increasing orofacial and limb chorea, chronically elevated creatine kinase (CK) levels and acanthocytosis (20%) suggested a diagnosis of ChAc. Absence of chorein in erythrocyte membranes4 was found. Case 2: This 30-year-old woman developed throat clicking and bruxism at age 24. Dysphagia and feeding dystonia were first noted at the age of 25, along with dysarthria, memory lapses, and vocal tics. She subsequently began biting her tongue and developed involuntary stereotypic movements, seizures, cognitive decline, and gait difficulties. On examination, she showed dysarthria, chorea, dystonia, ataxia, and hyporeflexia. Laboratory testing was significant for acanthocytosis and elevated CK levels. Heterozygous mutations were found in VPS13A (Case 23).5 Especially while eating, both patients demonstrate striking involuntary tongue movements and accompanying jaw opening. Forceful protrusion of the tongue only occurs during this action (see video), following which the patients are able to voluntarily retract the tongue. In a videofluoroscopic swallow study (VFSS) of Case 2, the pharyngeal phase was intact, whereas her oral phase was impaired by the tongue protrusion. Once the food bolus passed the upper sphincter of the esophagus, deglutition was carried out normally. Both patients tried to bypass the oral phase by strongly pressing their lips together to close their mouths while chewing, by extending the head if the tongue protruded, or else by extending the neck so that food fell into the pharynx posterior to the tongue, greatly increasing the risk of aspiration. Swallowing was effortful as suggested by the accompanying eye closure. In the VFSS, the tongue is not protruding against occluded teeth but is combined with wide opening of the jaw. The second patient's video shows that dysarthria in ChAc is marked by syllable iterations at the beginning of words and by impaired initiation of speech. When talking, the tongue of neither patient showed marked protrusion. However, since jaw closure occasionally was incomplete, it was possible to observe slight dystonic movements of the tongue, moving anterior to the teeth. Prominent tongue protrusion dystonia in ChAc occurs when placing solid food into the mouth, with chewing and swallowing food or with manipulations in the orolingual region but was absent at rest, in contrast to the spontaneous tongue protrusion described in other diseases.4 Dysphagia in ChAc differs from that observed in Huntington's disease (HD) who encounter difficulties mainly because of head and neck hyperextension, tachyphagia, and nonprotruding lingual chorea during the oral phase.6 Furthermore, movement disorder in HD directly affects also the pharyngeal and esophageal phases, while these appear to be intact in our ChAc patients. Action-induced tongue protrusion dystonia as seen in ChAc is not described in HD. Nonfood objects like toothpicks or cloth do not provoke tongue dystonia but may be used as a sensory trick, or as a mechanical obstruction to avoid involuntary jaw closure. The combination of masseter muscle and tongue protrusion dystonia appears to be the cause of the common mutilations of tongue, lips, and cheeks in ChAc and may result in significant weight loss and lethal infection.7 Mild tongue dystonia was observed on talking, swallowing saliva, and drinking. Being aware of these involuntary movements, patients sometimes incorporate them into parakinesias such as lip smacking. We suggest that action-induced tongue protrusion and feeding dystonia is a highly specific hallmark for ChAc, often seen early in the course of disease. Particularly in combination with chronically elevated CK levels in young adults, this symptom has high diagnostic value. Sequence 1. Patient 1 shows slight speech impairment such as iterations at the beginning of words. Tongue protrusion and jaw opening occur when she places a morsel of food in her mouth. Please, note the absence of further abnormal movements. Sequence 2. Patient 2 displays an almost identical action-induced tongue protrusion while eating. Sequence 3. VFSS sequence of Patient 2 shows feeding and chewing interrupted by tongue protrusion dystonia, with subsequent normal esophageal phase. Sequence 4. Patient 2 displays dysarthria and iterations while talking about her tongue movements and eating difficulties.1 While eating a pretzel, Patient 1 with ChAc demonstrates action-induced tongue protrusion. The authors thank Mrs. Andrea Hofmayer (Asklepios, Bad Tölz, Germany) and Mrs. Doro Kühnau (Rehabilitation Center, Nittenau, Germany) for their advice on speech pathology. Financial Disclosure: Benedikt Bader: Supported by the Advocacy for Neuroacanthocytosis Patients, supported by the Bayerische Forschungsallianz, received travel funding from the Bundesministerium für Bildung und Forschung; Ruth H. Walker: Supported by Intellyst Medical Communication, for speaking on Huntington's disease, supported by Scienta Healthcare education for advice on construction of a Huntington's disease case study for CME-accredited on-line program; Mathias Vogel: none; Mario Prosiegel: none; Jacqueline McIntosh: none; Adrian Danek: Receives travel funding from Pfizer, Editorial board of “Zeitschrift für Gerontoneurologie,” receives royalties from publishing “Klinische Untersuchung der höheren Hirnleistungen” (Kohlhammer Verlag, 2008), has received speaker honoraria from Merz, Pfizer, and Neuro-Update, receives research support as Principal Investigator from Bayerische Forschungsstiftung (of Federal Government of Bavaria), Münchner Universitätsgesellschaft, Deutsch-Französische Hochschulstiftung, and Advocacy for Neuroacanthocytosis Patients, has served as an expert witness for German courts of justice in medico- legal cases, supported by the Advocacy for Neuroacanthocytosis Patients. B. Bader: Conception, organization, and execution of research project, design of data analysis and patients, writing of the first draft of the manuscript; R.H. Walker: Review and critique of data analysis and patients, review and critique of manuscript; M. Vogel: Data interpretation and review and critique of data analysis and patients, review and critique of manuscript; M. Prosiegel: Data interpretation and review and critique of data analysis and patients, review and critique of manuscript; J. McIntosh, Design, data interpretation, and review and critique of data analysis and patients, review and critique of manuscript; A. Danek: Conception of research project, review and critique of manuscript. Additional Supporting Information may be found in the online version of this article. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article. Benedikt Bader MD*, Ruth H. Walker MB, ChB, PhD , Mathias Vogel PhD , Mario Prosiegel MD§, Jacqueline McIntosh¶, Adrian Danek MD*, * Neurologische Klinik und Poliklinik Ludwig-Maximilians-Universität München, Germany, Department of Neurology James J. Peters Veterans Affairs Medical Center Bronx, New York Mount Sinai School of Medicine New York, New York, Clinic of Neuropsychology Klinikum Bogenhausen München, Germany, § Center for Dysphagia Fachklinik Bad Heilbrunn Bad Heilbrunn, Germany, ¶ Wolfson Neurorehabilitation Centre London, United Kingdom.

Neurogenic dysphagia is one of the most frequent and prognostically relevant neurological deficits in a variety of disorders, such as stroke, parkinsonism and advanced neuromuscular diseases. Flexible endoscopic evaluation of swallowing (FEES) is now probably the most frequently used tool for objective dysphagia assessment in Germany. It allows evaluation of the efficacy and safety of swallowing, determination of appropriate feeding strategies and assessment of the efficacy of different swallowing manoeuvres. The literature furthermore indicates that FEES is a safe and well-tolerated procedure. In spite of the huge demand for qualified dysphagia diagnostics in neurology, a systematic FEES education has not yet been established.The structured training curriculum presented in this article aims to close this gap and intends to enforce a robust and qualified FEES service. As management of neurogenic dysphagia is not confined to neurologists, this educational programme is applicable to other clinicians and speech-language therapists with expertise in dysphagia as well.The systematic education in carrying out FEES across a variety of different professions proposed by this curriculum will help to spread this instrumental approach and to improve dysphagia management.

Oropharyngeal sensitivity plays a vital role in the initiation of the swallowing reflex and is thought to decline as part of the aging-process. Taste and smell functions appear to decline with age as well. The aim of our study was to generate data of oral sensitivity in healthy participants for future studies and to analyse age-related changes and their interdependence by measuring oral sensitivity, taste, and smell function. The experiment involved 30 participants younger than and 30 participants older than 60. Sensitivity threshold as a surrogate of oral sensitivity was measured at the anterior faucial pillar by electrical stimulation using commercially available pudendal electrode mounted on a gloved finger. Smell and taste were evaluated using commercially available test kits. Mean sensitivity was lower in young participants compared to older participants (1.9 ± 0.59 mA vs. 2.42 ± 1.03 mA; p = 0.021). Young participants also performed better in smell (Score 11.13 ± 0.86 vs 9.3 ± 1.93; p < 0.001) and taste examinations (Score 11.83 ± 1.86 vs 8.53 ± 3.18; p < 0.001). ANCOVA revealed a statistical association between sensitivity and smell (p = 0.08) that was moderated by age (p = 0.044). Electrical threshold testing at the anterior faucial pillar is a simple, safe, and accurate diagnostic measure of oral sensitivity. We detected a decline of oral sensitivity, taste, and smell in older adults.Trial registration: Clinicaltrials.gov, NCT03240965. Registered 7th August 2017- https://clinicaltrials.gov/ct2/show/NCT03240965 .

To determine: (1) patterns of cognitive and psychiatric dysfunction; (2) those neurobehavioural parameters which mostly influence disability in activities of daily living (ADL) and handicap in occupational and psychosocial activities, and (3) underlying neuroanatomical pathology in patients after rupture and repair of anterior communicating artery (ACoA) aneurysm.30 patients were extensively examined by means of a comprehensive battery of neuropsychological tests, by rating of psychopathological symptoms, and by use of the functional independence measure (FIM), Glasgow outcome scale (GOS), and MRI.(1) Three main groups were characterised by primary impairment of memory, executive functions, or of attentional performance. Within these main groups, specific patterns were identified relating to extent of primary dysfunction and associated disorders. The variety of neuropsychological disturbances is in contradiction to the existence of an "ACoA syndrome" as an entity. (2) Rehabilitation outcome proved to be mostly associated with both memory and attentional performance. (3) In neuropathological terms, lesions of the medial septum and nucleus of the diagonal band of Broca (MS/ndbB) were closely associated with memory deficits and prefrontal lesions were associated with attentional, executive, and psychopathological dysfunctions. At the same time, bilateral lesions were associated with severe disturbances. The type and severity of the above mentioned deficits were independent of the side of lesion in unilateral cases, of rectus gyrus resection, and of the Hunt and Hess grading system.

The aim of our work was to develop a step-wise concept for investigating neurogenic oropharyngeal dysphagia (NOD) that could be used by both trained nursing staff as well as swallowing therapists and physicians to identify patients with NOD at an early stage and so enable an appropriate therapy to be started. To achieve this objective, we assessed uniform terminology and standard operating procedures (SOP) in a new NOD step-wise concept. In-house stroke mortality rates and rates of pneumonia were measured over time (2003-2009) in order to show improvements in quality of care. In addition, outcome measures in a stroke-unit monitoring system were studied after neurorehabilitation (day 90) assessing quality of life (QL) and patient feedback. An investigation that was carried out in the context of internal and external quality assurance stroke projects revealed a significant correlation between the NOD step-wise concept and low rates of pneumonia and in-house mortality. The quality of life measures show a delta value that can contribute to "post-stroke" depression. The NOD step-wise concept (NSC) should, on the one hand, be capable of being routinely used in clinical care and, on the other, being able to fulfil the requirements of being scientifically based for investigating different stages of swallowing disorders. The value of our NSC relates to the effective management of clinical resources and the provision of adequate diagnostic and therapeutic options for different grades of dysphagia. We anticipate that our concept will provide substantial support to physicians, as well as swallowing therapists, in clinical settings and rehabilitation facilities, thereby promoting better guidance and understanding of neurogenic dysphagia as a concept in acute and rehabilitation care, especially stroke-unit settings.

Over 30% of persons with multiple sclerosis (pwMS) suffer from swallowing symptoms, a higher rate than previously assumed. Neurogenic dysphagia (ND) may cause many different kinds of oropharyngeal sensorimotor dysfunctions in pwMS, and is associated with both the amount of disability and brainstem signs. About 15% of pwMS with mild disability may also suffer from ND. Diagnostic tools comprise history taking, bedside screening examination (50 ml water test combined with assessment of pharyngeal sensation or with pulse oximetry) and sometimes a videofluoroscopic swallowing study (VFSS) and fibreoptic endoscopic evaluation of swallowing (FEES). VFSS and FEES are complementary methods and both have advantages and disadvantages. Interventions for ND in pwMS are mainly based on functional swallowing therapy, including methods of restitution, compensation and adaptation. The aim of intervention is to prevent aspiration and aspiration pneumonia. Outcome assessment should focus on clinically relevant parameters, such as activity limitation, participation restriction and health-related quality of life.

Our understanding of brainstem swallowing centers is mainly based on experimental animals. In order to solve this problem also in humans, a clinical-anatomical study on dysphagic patients with different lesion patterns was performed.We studied 43 consecutively admitted dysphagic patients with unilateral paresis of the vagal nerve (PVN), Avellis' syndrome (AS), Wallenberg's syndrome (WS), posterior fossa tumour (PFT) or cerebellar hemorrhage (CH) with regard to clinical and anatomical aspects.There was a continuum with regard to functional outcome from neurogenic dysphagia (ND): Patients with PFT or CH had a significantly worse outcome than patients with WS; the outcome of WS patients was significantly worse than that of patients with PVN or AS. In AS only the Nucleus ambiguus (NA) and its surrounding reticular formation (RF) were affected. In all patients with WS, the infarctions of the dorsolateral medulla were situated in the rostral third of the medulla and affected the NA and the Nucleus tractus solitarii (NTS) with their surrounding RF. In patients with PFT and CH, the NTS and its surrounding RF were affected on both sides. The overlap area of WS and PFT lesions is situated in the NTS and the surrounding RF, especially in its Nucleus parvocellularis.Our results point to the fact, that in humans the dorsomedial central pattern generators (CPGs) for swallowing are situated in the rostral part of the dorsal medulla oblongata near the NTS/surrounding RF (especially Nucleus parvocellularis) and that the dorsomedial CPGs are superior to the ventrolateral CPGs (near the NA/surrounding RF) with regard to their swallowing-relevance. Furthermore, we hypothesize that due to the individual asymmetry of the swallowing-dominant forebrain hemisphere - the outcome from ND in WS depends on the side of the medullary infarction.

The frequency of cognitive deficits in multiple sclerosis (MS) patients is rather high and the estimates vary between 43% and 72% depending on the patient samples studied as well as on the methods of cognitive assessment. Despite the great impact of cognitive dysfunction on several aspects of the quality of life, the importance of accurate assessment and rehabilitation of neuropsychological deficits in MS patients has long been ignored. In this article, we first describe tests for the assessment of impairments, disabilities and handicaps. We emphasize that after screening with brief assessment instruments, detailed testing of the basic target deficits is mandatory for the planning of special cognitive training programs. Second, the correlation of certain cognitive deficit patterns with important magnetic resonance imaging (MRI) variables such as total lesion area, size of the corpus callosum and specific lesion location is outlined in detail. Third, some recommendations are made with regard to general rehabilitation principles such as restitution, compensation and adaptation as well as for special rehabilitation techniques including cognitive retraining of basic deficits and/or training of activities of daily living. Finally, we emphasize that there is a need for the development of tailor-made neuropsychological rehabilitation techniques for MS patients, which take into account the course and stage of the disease as well as the specific psychosocial problems of the individual patient.

No studies exist dealing with the outcome of dysphagic patients with posterior fossa (IV. ventricle) tumours (PFT) or cerebellar hemorrhage (CH), and the outcome of patients with Wallenberg's syndrome (WS) after functional swallowing therapy (FST) has so far not been studied in detail. Patients and methods. 208 patients with neurogenic dysphagia (ND) who were consecutively admitted for functional swallowing therapy (FST) over a 3 year period to our hospital were examined clinically, by use of a videofluoroscopic swallowing study (VFSS) and/or fibreoptic evaluation of swallowing (FEES). The most frequent etiology was stroke (48%), followed by CNS tumours (13%). In the present study we defined three groups. Group 1 comprised 8 patients with PFT or CH. Group 2 consisted of 27 patients with WS, which was the leading cause among patients with non-hemispheric stroke. Since in WS a vagal nerve paresis due to affection of the Nucleus ambiguus occurs, 8 patients with Avellis' syndrome or unilateral paresis of the vagal nerve served as controls and were defined as group 3. Findings. In the three groups, functional feeding status showed significant improvement after FST comprising methods of restitution, compensation and adaptation, each of which were applied in more than 80% of patients. Outcome was, however, significantly worse in group 1 as compared to group 2 and in group 2 as compared to group 3. Dysfunction of the upper esophageal sphincter and reflex triggering were significantly more severely disturbed in groups 1 and 2 as compared to group 3. Group 1 showed significantly more severe disturbances of the oral phase as compared to groups 2 and 3. After FST, more than 50% (5/8) of group 1 and 30% (8/27) of WS patients (group 2) were dependent on tube feeding, whereas all patients of group 3 were full-oral feeders. Interpretation. This is the first study dealing with the outcome of dysphagic patients with PFT or CH. Based on our results it can be assumed that in these patients pressure is exerted on both dorsomedial central pattern generators (DMCPGs) for swallowing in a posterior-anterior direction. Due to the importance of the DMCPGs for swallowing, bilateral (and often MRI-invisible) lesions seem to be very harmful. For a better understanding of the pathomechanism responsible for ND in patients with PFT or CH, modern imaging methods such as proton magnetic resonance spectroscopy should be used for studying metabolic changes in the dorsal medulla in the future. Since the outcome of patients with WS with regard to dependence of tube feeding was not associated with the site or size of the lesion, it may due to the individual asymmetry of the swallowing-dominant forebrain hemisphere - depend on the side of the medullary infarction.

The release of leukotriene C4 (LTC4), an important 5-lipoxygenase product of the arachidonic acid metabolism from polymorphonuclear leucocytes (PMNLs) of guinea pigs with experimental allergic encephalomyelitis (EAE), the animal model of MS, has been found to be significantly increased compared with healthy animals. Subsequently, the dual cyclo-oxygenase and 5-lipoxygenase inhibitor BW755C was applied to 15 guinea pigs with EAE. Two control groups (15 each) were treated with the cyclo-oxygenase inhibitor indomethacin or physiological saline, respectively. In the BW755C treated group, no animal developed symptoms of the disease in contrast to, respectively, 5 and 3 animals in the 2 other groups. Histological examination of the CNS revealed a highly significantly lower inflammation score in the BW755C treated animals, and the release of LTC4 from PMNLs was highly significantly decreased in this group compared with each of the others. The findings suggest that the vascular permeability enhancing LTC4 plays a pathogenetic role in EAE and indicate that inhibition of this sulfidopeptide leukotriene suppresses the disease. Therefore, the application of leukotriene inhibitors could contribute to the future treatment of MS.

Measurements of blood platelet aggregation were carried out in 30 patients suffering from multiple sclerosis (MS) and in 15 healthy individuals. Compared with the control group, the MS patients showed an increase in both spontaneous and induced (ADP and serotonin) platelet aggregation. The possible pathogenetic significance of these results is discussed.

It has recently been suggested that the sulfidopeptide leukotriene C4 (LTC4), a 5-lipoxygenase product of the arachidonic acid metabolism and one of the most potent mediators of vascular permeability, might be involved in the pathogenesis of experimental autoimmune encephalomyelitis (EAE) and multiple sclerosis (MS). Subsequently, 20 guinea pigs with EAE were treated with sulfasalazine, a substance with a proved leukotriene inhibiting effect, which has previously been described as exerting beneficial effects in patients with inflammatory bowel disease and rheumatoid arthritis. The sulfasalazine-treated guinea pigs showed a significantly better clinical outcome, as well as a significantly lower histological inflammation score compared with 19 controls.

Fibre-endoscopic evaluation of swallowing (FEES) to detect dysphagia is gaining more and more importance as a diagnostic tool. Therefore, we have investigated the impact of FEES in neurological patients in a clinical setting.Cross-sectional hospital-based registry.Primary acute care in a neurological department of a German university hospital.241patients with various neurological diseases who underwent FEES procedure.Dysphagia and related comorbidities.267 FEES were performed in 241 patients with various neurological diagnoses. Dysphagia was diagnosed in 68.9% of the patients. In only 33.1% of the patients, appropriate oral diet was chosen prior to FEES. A relevant dysphagia occurred more often in patients with structural brain lesions (83.1% vs 65.3%, P=0.001), patients with dysphagia had a longer hospitalisation (median 18 (IQR 12-30) vs 15 days (IQR 9.75-22.75), P=0.005) and had a higher mortality (8.4% vs 1.3%, P=0.041). When the oral diet was changed, we observed a lower pneumonia rate (36% vs 50%, P=0.051) and a lower mortality (3.7% vs 11.3%, P=0.043) in comparison to no change of oral diet. A restriction of oral diet was identified more often in older patients (median 75 years (IQR 66.3-82 years) vs median 72 years (IQR 60-79 years), P=0.01) and in patients with structural brain lesions (86.8% vs 73.1%, P=0.05).On clinical investigation, dysphagia was misjudged for the majority of the patients. FEES might help to compensate this drawback, revising the diet regime in nearly 70% of the patients.

Diagnosing dysphagia in acute stroke patients is crucial, as this comorbidity determines morbidity and mortality; we therefore investigated the impact of flexible nasolaryngeal endoscopy (FEES) in acute stroke patients.The FEES investigation as performed in acute stroke patients treated at a large university hospital, allocated as a standard procedure for all patients suspected of dysphagia. We correlated our findings with baseline data, disability status, pneumonia, duration of hospitalisation, necessity for mechanical ventilation and treatment on the intensive care unit. The study was designed as a cross-sectional hospital-based registry.We investigated 152 patients. The median age was 73; 94 were male. Ischemic stroke was diagnosed in 125 patients (82.2%); 27 (17.8%) suffered intracerebral haemorrhage. Oropharyngeal dysphagia was diagnosed in 72.4% of the patients, and was associated with higher stroke severity on admission (median NIHSS 11 [IQR 6-17] vs. 7 [4-12], p = .013; median mRS 5 [IQR 4-5] vs. 4 [IQR 3-5], p = .012). Short-term mortality was higher among patients diagnosed with dysphagia (7.2% vs. 0%, p = .107). FEES examinations revealed that only 30.9% of the patients had an oral diet appropriate for their swallowing abilities. A change of oral diet was associated with a better outcome at discharge (mRS; p = .006), less need of mechanical ventilation (p = .028), shorter period of hospitalisation (p = .044), and lower rates of pneumonia (p = .007) and mortality (p = .011).Due to the inability of clinical assessments to detect silent aspiration, FEES might be better suited to identify stroke patients at risk and may contribute to a better functional outcome and lower rates of pneumonia and mortality. Our findings also point to a low awareness of dysphagia, even in a specialised stroke centre. FEES in acute stroke patients helps to adjust the oral diet for the vast majority of stroke patients (69.1%) based on their swallowing abilities, potentially avoiding severe complications.

This study was conducted to compare the Extended Barthel Index (EBI) to the Functional Independence Measure (FIM) and the Expanded Disability Status Scale (EDSS) as a measure used to assess changes in a patient's need for help with activities of daily living. One hundred patients with multiple sclerosis were evaluated by the same person upon arrival in a rehabilitation clinic and four weeks later. EBI and FIM both proved to be far more sensitive in detecting changes than the EDSS (29% of patients changed their EBI score, 32% their FIM score, 5% their EDSS score). EBI and FIM showed a very high correlation (Spearman correlation coefficient r s = 0.9705). Both scales correlated somewhat less with the EDSS ( r s = -0.7624, resp. -0.7611). Considering the high correlation and equal sensitivity of EBI and FIM, the study recommends the use of the EBI, since it has a simpler rating system and the elimination of some redundant FIM items increases user-friendliness and compliance.

In neun unterschiedlichen deutschen Kliniken, die Patienten der neurologischen Frührehabilitation in der Phase B behandeln, wurden bei insgesamt 1280 Patienten innerhalb eines halben Jahres demografische und medizinische Daten erfasst, um Vorbehandlung, Diagnosen, Verlauf und Komplikationen abzubilden. Dabei ergab sich ein Überwiegen männlicher (62 %) Patienten und ein Durchschnittsalter von 65 Jahren. Die Patienten wurden vorwiegend aus neurologischen und neurochirurgischen Abteilungen in die Rehabilitation der Phase B verlegt, im Median nach 27 Tagen (bei Stroke-Units nach 13 Tagen) stationärer Behandlung im Akutkrankenhaus. Bei der Aufnahme in die Phase B waren knapp 17 % der Patienten noch künstlich beatmet, gemäß der Glasgow Coma Scale bestanden im Mittel leichte bis mittelschwere Komastadien. Während der im Median 57 Tage langen Behandlung in der Reha-Phase B ergaben sich - abhängig von der Diagnose entweder nur sehr geringe (hypoxische Hirnschädigung) oder aber rasche und umfangreiche (Schädel-Hirn-Trauma) Steigerungen der Funktionen und Fähigkeiten. Die Entlassung aus der Phase B erfolgte zu 24,3 % nach Hause, zu 33,1 % in die weiterführende Rehabilitation Phase C und zu 23,1 % in ein Pflegeheim. 6,4 % der Patienten verstarben während der Behandlung in der Phase B. Die aufgetretenen Komplikationen, vor allem Harnwegsinfekte und Pneumonien, konnten in den meisten Fällen in den Phase-B-Einrichtungen beherrscht werden, zur Verlegung in Akutkrankenhäuser führten vorwiegend Komplikationen wie Hydrozephalus und Tracheomalazien/-stenosen, bei denen oft ein invasives Vorgehen erforderlich ist. Der Krankheitszustand bei Entlassung aus der Phase B (Outcome) nach der Glasgow Outcome Scale wurde bei einem hohen Anteil als „teilweise Unabhängigkeit” oder „Unabhängigkeit in den Aktivitäten des täglichen Lebens” eingeschätzt, sodass insgesamt betrachtet die Rehabilitationsphase B als sehr effektiv anzusehen ist.

Release of leukotriene B4 (LTB4) and leukotriene C4 (LTC4) from neutrophils and platelet-neutrophil suspensions in response to ionophore A23187 was measured in 12 multiple sclerosis (MS) patients and 8 healthy volunteers. LTC4 release from neutrophils, as well as from platelet-neutrophil suspensions, was significantly decreased in MS patients compared with the controls. There was no significant difference in the release of LTB4 between MS patients and controls. The findings suggest that permanent stimulation of platelets and neutrophils e.g., by encephalitogenic peptide leads to continuous LTC4 release with subsequent depletion of intracellular substrates serving as precursors for the formation of 5-lipoxygenase products. Since the target of microvascular actions of LTC4 are postcapillary venules, the release of this sulfidopeptide leukotriene might play a pathogenetic role in the formation of MS lesions.

Einleitung: Anliegen der Arbeit war es, ein diagnostisches Stufenkonzept zur Untersuchung der neurogenen oropharyngealen Dysphagie (NOD) zu erstellen, das sowohl von der ausgebildeten Pflegekraft als auch von Logopäden oder einem Arzt eingesetzt werden kann, um Patienten mit NOD frühzeitig zu identifizieren und eine entsprechende Therapie zu ermöglichen.

Aims: There is behavioral evidence of increased spontaneous recruitment of visual attention to ancestral evolved categories, such as animals, compared with expertise-derived categories, such as a computer. In order to investigate the association between visual perception and spontaneous visual attention, a study was performed to determine if brain activation whilst viewing moving animals was increased compared with optokinetic computer stimuli. Methods: Functional MRI was performed in 12 healthy volunteers using a standard block-design paradigm, consisting of three consecutive experiments. Subjects viewed the following images: Experiment one — optokinetic computer stimuli alternating with static computer stimuli; Experiment two — moving animals alternating with non-moving animals; Experiment three — moving animals alternating with optokinetic computer stimuli. Results: Moving animals evoked motion-dependent activation bilaterally in the middle and superior temporal gyri, right inferior temporal gyrus, left occipital gyrus, right supramarginal gyrus, and left straight gyrus. Integrated object-and-motion-dependent activation was found bilateral in inferior and middle temporal gyri, right superior temporal gyrus, right superior parietal lobule, left dorsal putamen, and right amygdala. Conclusions: These results suggest that there is increased cerebral activity in the visuo-attentional network whilst viewing moving animals compared with optokinetic computer stimuli.

Platelet aggregation (PA) stimulated by encephalitogenic peptide (EP) and PA induced by ADP were measured in 83 multiple sclerosis (MS) patients and 70 control subjects with other neurological diseases (OND). EP-stimulated PA was significantly increased in MS patients as compared with the controls. There was no significant difference in ADP-induced PA between patients with MS and OND. The results are discussed in terms of EP-stimulated platelets playing a role in the pathogenesis of MS by affecting the venular permeability of the brain.

Dysphagia is common in critically ill neurological patients and is associated with a high mortality and morbidity. Data on the usefulness of flexible endoscopic examination of swallowing (FEES) in neurological intensive care unit (ICU) patients are lacking, raising the need for evaluation.FEES was performed in neurological intensive care patients suspected of dysphagia. We correlated findings with baseline data, disability status, pneumonia and duration of hospitalisation, as well as a need for mechanical ventilation or tracheotomy.This analysis consisted of 125 patients with suspected dysphagia. Most of the patients (81; 64,8%) suffered from acute stroke. Dysphagia was diagnosed using FEES in 90 patients (72%). FEES results led to dietary modifications in 80 patients (64%). The outcome at discharge was worse in dysphagic stroke patients diagnosed by FEES as compared to non-dysphagic stroke patients (p = 0.009). Patients without oral diet had higher need for intubation (p = 0.007), tracheotomy (p = 0.032) and higher mortality (p < 0.001) in comparison to patients with at least small amounts of oral intake.As the clinical assessment of the patients often classified the dysphagia incorrectly, the broad use of FEES in ICU patients might help to adequately adjust patients' oral diet. This knowledge might contribute to lower mortality and morbidity.

The purpose of this prospective cohort study was: (1) to document and investigate long-term post-treatment outcome focusing on swallowing disability; and (2) to reveal variables predicting successful functional follow-up results in 63 patients with neurogenic dysphagia. All patients were admitted to an inpatient neurologic rehabilitation unit.Information was gathered through chart review and questionnaires. Functional outcome was categorized according to the degree of feeding status: (1) total tube feeding; (2) oral and tube feeding combined; (3) oral feeding with compensation; and (4) total oral feeding. 'Improvement' was determined as a positive shift in the type of feeding, 'deterioration' as a negative shift and 'no change' was defined as remaining at the same nutritional level. The safety of feeding was assessed by tracking the occurrence of pneumonia.Seventy percent of the patients achieved an improved immediate outcome after therapy. During long-term follow-up examinations, 43% of all patients showed further improvement, 57% did not show any change in their feeding ability and no deterioration was reported for any patient. Comparisons of the relative frequencies of the feeding modalities before and after therapy revealed a significant reduction in tube feeders and a significant increase in oral feeders with compensation during inpatient-treatment. The outpatient-interval showed a significant shift in total oral feeders without compensations but no significant improvement within the tube feeders and within the partial oral feeders. The improvement in nutritional status was not associated with an increased risk of pneumonia. Additional comparisons of the relative frequencies of the compensatory strategies indicated a significant reduction in all treatment techniques at final follow-up. Using logistic regression, predictors of successful post-discharge outcome involved a decreasing pre-treatment interval and unexpectedly low Barthel-ADL mobility scores.As a result we advocate regular follow-up controls using videofluoroscopic and/or videoendoscopic examination. This facilitates comparisons of swallowing efficiency with and without compensation in order to reduce compensatory strategies as early as possible. The analysis of predictive variables suggests early use of therapy to enhance the chances of successful long-term outcome.

Neurogene Dysphagien resultieren nicht nur aus muskulären Schwächen oder sensiblen Defiziten des oro-pharyngo-laryngealen Bereiches, sondern auch aus einer verzögerten Schluckreflextriggerung, einer gestörten Hyoid-Larynx-Elevation oder einer unzureichenden Öffnung des oberen Ösophagussphinkters (OÖS). Zur Diagnostik einer Dysphagie bzw. (des Schweregrades) einer Aspiration sind daher insbesondere zeitlich und räumlich hochauflösende Röntgenuntersuchungen, Radiomanometrie des Pharynx und OÖS sowie endoskopische Verfahren notwendig. In therapeutischer Hinsicht spielen invasive Verfahren zahlenmäßig eine untergeordnete Rolle gegenüber der Rehabilitation, welche in Verfahren der Restitution (z.B. thermale Stimulation) und der Kompensation (z.B. Schlucktechniken wie das Mendelsohn-Manöver) unterteilt werden kann. Bei ca. zwei Drittel der Patienten, die bei Therapiebeginn nur über eine Sonde ernährt werden können, gelingt es durch funktionelle Schlucktherapie, eine vollorale Ernährung zu erreichen (bei einer Therapiedauer von über drei Monaten). Für das Endergebnis ist die Zeit zwischen Schädigung und Beginn der Schlucktherapie von untergeordneter Bedeutung. Folgende bei Klinikaufnahme ermittelte Variablen erwiesen sich als Prädiktoren eines eher ungünstigen funktionellen Outcome: gestörte Speichelkontrolle, niedriger Barthel-Index, funktionell stark beeinträchtigtes Schlucken. Höheres Alter und die Anzahl der während des Klinikaufenthaltes aufgetretenen Pneumonien waren ebenfalls - allerdings schwächere -Prädiktoren eines ungünstigen Outcome. Unter neuropsychologischen Defiziten sind Aufmerksamkeitsstörungen besonders hinderlich, führen zu einer Zunahme der Therapiedauer und können in schweren Fällen Schlucktherapie unmöglich machen. Ob EMG-Biofeedback bez. Outcome oder Therapiedauer wirksam ist, wird zur Zeit untersucht. Mit modernen Methoden der dreidimensionalen Bewegungsanalyse kann die laryngeale Kinematik und damit das motorische Lernen während/nach Schlucktherapie erfaßt und damit der Therapieverlauf evaluiert werden.

Abstract This chapter discusses dysphagia or the difficulty of swallowing common in ALS patients, including its assessment and management strategies. Assessment of dysphagia includes clinical examination, videoflouroscopy, and endoscopic evaluation of swallowing. Treatment of dysphagia in patients includes several measures such as positioning, dietary modifications, compensatory techniques, and safety strategies. Patients with severe dysphagia are administered with augmented feeding techniques such as percutaneous endoscopic gastrostomy, nasogastric, or jejunostomy. Dysphagia-related symptoms such as sialorrhoea, mucus secretions, and gastroesophageal reflux can be treated through medicines. The efficacy of palliative care is assessed through nutritional status of the patient, the use of specific dysphagia-related scales, and quality of life scales. Management of dysphagia requires a multidisciplinary team approach and ongoing assessment of the medical community, language therapists, dieticians, the patients, and their families.

Neurogenic dysphagia is difficulty swallowing due to neurological diseases and compromises especially the oral and/or pharyngeal stage. The first section of this chapter deals with the neuroanatomy and neurophysiology of swallowing as a basis for a better understanding of neurogenic dysphagia. Then, diagnostic approaches are described comprising history taking, screening examinations, clinical swallowing examination, and instrumental methods. The third section focuses on those neurological diseases which are frequently associated with dysphagia and ends with the description of the problem that only a few pharmacological and invasive therapeutic interventions against neurogenic dysphagia exist. This expressly underlines the need for swallowing therapy and the development of new therapeutic approaches such as electrical pharyngeal or repetitive transcranial magnetic stimulations.

In a population comprising 197 patients, serum and CSF proteins were assayed using the radial immunodiffusion technique devised by Mancini. Multiple discriminants analysis was applied to investigate whether the measured CSF/serum protein relations and their ratios could be regarded as an indicator of specific neurological diseases. One significant finding was that the slope angle alpha of the regression line between the serum/CSF relation and molecular weight may represent an important indicative parameter. A small angle is suggestive of enhanced permeability of the BBB, a large angle of a correspondingly lowered permeability. Further, the analyses demonstrated that the combined use of several predictors can markedly improve differential diagnosis. The study also demonstrates the potential of a statistical analytic technique that is still rarely applied in medicine.

Abstract Dysphagia is common in neurological disease. However, our understanding of swallowing and its central nervous control is limited. Sensory information plays a vital role in the initiation of the swallowing reflex and is often reduced in stroke patients. We hypothesized that the sensitivity threshold of the anterior faucial pillar could be facilitated by either electrical stimulation (ES) or taste and smell information. The sensitivity threshold was measured by ES in the anterior faucial pillar region. The measurement was repeated 5 min after baseline. Thirty minutes after baseline, the participants underwent a test for taste and smell. Immediately after the test, the ES was repeated. Thirty healthy volunteers with a mean age of 27 ± 5.1 participated in the trial. Mean sensitivity threshold at baseline was 1.9 ± 0.59 mA. The values 5 min after baseline (1.74 ± 0.56 mA, p = 0.027) and 30 min after baseline (1.67 ± 0.58 mA, p = 0.011) were significantly lower compared to the baseline, but there was no difference between the latter ( p = 0.321). After 5 min, a potentially facilitating effect was found on oral sensitivity by ES of the faucial pillar area. Thirty minutes later, this effect was still present. Trial registration Clinicaltrials.gov, NCT03240965. Registered 7th August 2017— https://clinicaltrials.gov/ct2/show/NCT03240965 .

Immunoglobulins are often used as an optimizing therapy in cases of infectious diseases of the central nervous system. To clarify the question of whether or not an intravenously administered compound of the IgG class is able to penetrate the cerebrospinal fluid barrier despite its high molecular weight, 12 anti-HBs negative patients received 20 ml each of a beta-Propiolacton treated IgG compound with a high anti-HBs titre (1 : 115 000) used as a marker. Four patients having an inconspicuous fluid condition were consulted for control. Five patients were suffering from slight disturbances and three other patients had severe disorders of the blood-cerebrospinal fluid barrier function resulting from inflammatory diseases of the central nervous systems. Cerebrospinal fluid was produced by way of lumbar puncture resp. drainage for the determination of anti-HBs. Simultaneously, the concentration of antibodies in serum was determined. In all patients having barrier disturbances, anti-HBs was evident in the cerebrospinal fluid, the transfer of intravenously administered immunoglobulins to cerebrospinal fluid increasing in correlation with the degree of the barrier disorder. The therapeutical importance of immunoglobulin therapy in treating infections of the central nervous system is pointed out.

Introduction: The aim of our work was to develop a step-wise concept for investigating neurogenic oropharyngeal dysphagia (NOD) that could be used by both trained nursing staff, as well as swallowing therapists and physicians, to identify patients with NOD at an early stage and so enable an appropriate therapy to be started.

Neurogenic dysphagia is difficulty swallowing due to neurological diseases and compromises especially the oral and/or pharyngeal stage. The first section of this chapter deals with neuroanatomy and neurophysiology of swallowing as a basis for a better understanding of neurogenic dysphagia. Then diagnostic approaches are described comprising history taking, screening examinations, comprehensive clinical swallowing examination, and instrumented methods. The third section focuses on those neurological diseases which are frequently associated with dysphagia and ends with the description of the problem that only few pharmacological and invasive therapeutic interventions against neurogenic dysphagia exist. This expressly underlines the need for swallowing therapy and the development of new therapeutic approaches such as electrical pharyngeal or noninvasive magnetic and electrical brain stimulation.

Based on five of our own cases and the literature on hand the etiological, clinical, diagnostic and intensive therapeutic aspects of the syndrome are represented. Special emphasis is placed on the possible role of the oral contraceptives in triggering this syndrome.

Unilateral hemispheric stroke may cause dysphagia. Based on studies using functional imaging methods, the anterior insula plays an eminent role in the initiation process of swallowing. However, no unilateral lesion solely confined to the territory of the anterior insuloopercular arteries has been described as a cause of dysphagia.