Flexible endoscopic evaluation of swallowing (FEES) for neurogenic dysphagia: training curriculum of the German Society of Neurology and the German stroke society.

BACKGROUND: 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. RESULTS: 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. CONCLUSION: 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.

[Consensus paper--adequate medication for neurologic and geriatric patients with dysphagia]. / Konsensuspapier--Bedarfsgerechte Medikation bei neurologischen und geriatrischen Dysphagie-Patienten.

BACKGROUND: The incidence for dysphagia amounts to 44-50% in nursing homes. Dysphagia is one of the main reasons for pneumonia in elderly. METHOD: Consensus paper. RESULTS AND CONCLUSIONS: For the advisory board consisting of 2 physicians, 2 pharmacists, a speech therapist, and a respresentative of nursing service it is common understanding that for the ideal maintenance and support of patients with dysphagia an interdisciplinary approach is crucial. Despite high clinical relevance of dysphagia the basic knowledge of this field is often underdeveloped. Specific and validated screening procedures for dysphagia have to be developed and implemented into the relevant guidelines. Specifically in this field an active and discipline spanning risk management should find its way into stationary geriatric care and nursing homes. Just as important is the provision of necessary patient information on the progress of the disease, on therapeutic actions and possible diet forms in a dysphagia pass. Additionally, the mentioned disciplines require an online risk screening (for dysphagia) of the pharmacist concerning the overall medication as well as information of galenic properties like facts regarding the possibility of crushing, portioning or tube feeding of the prescribed medication. In this way health risks due to administration errors concerning the medication can be significantly reduced for this patient group. Adequate oral liquids for adapted application of drugs are missing so far.

Prediction of outcome in neurogenic oropharyngeal dysphagia within 72 hours of acute stroke.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Neuromagnetic oscillations and hemodynamic correlates of P50 suppression in schizophrenia.

Behavioral and electrophysiological data indicate compromised stimulus suppression in schizophrenia. The physiological basis of this effect and its contributions to the etiology of the disease are poorly understood. We examined neural and metabolic measures of P50 suppression in 12 patients with schizophrenia and controls. First, whole-head magnetoencephalography (MEG) assessed amplitudes of left- and right-hemispheric evoked responses and induced oscillations. Secondly, functional magnetic resonance imaging (fMRI) measured the hemodynamic responses to pairs of beeps with a short interval (500ms) as compared with those with a long interval (1500ms). The suppression of alpha power (8-13Hz) time-locked to the stimuli was negatively correlated with the suppression of evoked components and the hemodynamic measures. Remarkably, the suppression of alpha power was reduced in the patients already prior to stimulus onset. Conceivably, alpha oscillations play a central role in stimulus adaptation of neuronal networks and reflect an active mechanism for sensory suppression. The reduced stimulus suppression in schizophrenia seems to be in part due to impaired generation of alpha oscillations in the auditory cortex, resulting in higher metabolic demand as detected by fMRI. Delayed recovery of alpha rhythm may reflect an impaired gating function and contribute to sensory and cognitive deficits in schizophrenia.

The contribution of mesiofrontal cortex to the preparation and execution of repetitive syllable productions: an fMRI study.

Clinical data indicate that the brain network of speech motor control can be subdivided into at least three functional-neuroanatomical subsystems: (i) planning of movement sequences (premotor ventrolateral-frontal cortex and/or anterior insula), (ii) preparedness for/initiation of upcoming verbal utterances (supplementary motor area, SMA), and (iii) on-line innervation of vocal tract muscles, i.e., motor execution (corticobulbar system, basal ganglia, cerebellum). Using an event-related design, this functional magnetic resonance imaging (fMRI) study sought to further delineate the contribution of SMA to pre-articulatory processes of speech production (preceding the innervation of vocal tract muscles) during an acoustically paced syllable repetition task forewarned by a tone signal. Hemodynamic activation across the whole brain and the time courses of the responses in five regions of interest (ROIs) were computed. First, motor preparation was associated with a widespread bilateral activation pattern, encompassing brainstem structures, SMA, insula, premotor ventrolateral-frontal areas, primary sensorimotor cortex (SMC), basal ganglia, and the superior cerebellum. Second, calculation of the time courses of BOLD ("blood oxygenation level-dependent") signal changes revealed the warning stimulus to elicit synchronous onset of hemodynamic activation in these areas. However, during 4-s intervals of syllable repetitions SMA and cerebellum showed opposite temporal activation patterns in terms of a shorter (SMA) and longer (cerebellum) latency of the entire BOLD response-as compared to SMC, indicating different pacing mechanisms during the initial and the ongoing phase of the task. Nevertheless, the contribution of SMA was not exclusively restricted to the preparation/initiation of verbal responses since the extension of mesiofrontal activation varied with task duration.

The role of the unaffected hemisphere in motor recovery after stroke.

The contribution of the ipsilateral (nonaffected) hemisphere to recovery of motor function after stroke is controversial. Under the assumption that functionally relevant areas within the ipsilateral motor system should be tightly coupled to the demand we used fMRI and acoustically paced movements of the right index finger at six different frequencies to define the role of these regions for recovery after stroke. Eight well-recovered patients with a chronic striatocapsular infarction of the left hemisphere were compared with eight age-matched participants. As expected the hemodynamic response increased linearly with the frequency of the finger movements at the level of the left supplementary motor cortex (SMA) and the left primary sensorimotor cortex (SMC) in both groups. In contrast, a linear increase of the hemodynamic response with higher tapping frequencies in the right premotor cortex (PMC) and the right SMC was only seen in the patient group. These results support the model of an enhanced bihemispheric recruitment of preexisting motor representations in patients after subcortical stroke. Since all patients had excellent motor recovery contralesional SMC activation appears to be efficient and resembles the widespread, bilateral activation observed in healthy participants performing complex movements, instead of reflecting maladaptive plasticity.

Whole brain-based analysis of regional white matter tract alterations in rare motor neuron diseases by diffusion tensor imaging.

Different motor neuron disorders (MNDs) are mainly defined by the clinical presentation based on the predominance of upper or lower motor neuron impairment and the course of the disease. Magnetic resonance imaging (MRI) mostly serves as a tool to exclude other pathologies, but novel approaches such as diffusion tensor imaging (DTI) have begun to add information on the underlying pathophysiological processes of these disorders in vivo. The present study was designed to investigate three different rare MNDs, i.e., primary lateral sclerosis (PLS, N = 25), hereditary spastic paraparesis (HSP, N = 24), and X-linked spinobulbar muscular atrophy (X-SBMA, N = 20), by use of whole-brain-based DTI analysis in comparison with matched controls. This analysis of white matter (WM) impairment revealed widespread and characteristic patterns of alterations within the motor system with a predominant deterioration of the corticospinal tract (CST) in HSP and PLS patients according to the clinical presentation and also in patients with X-SBMA to a lesser degree, but also WM changes in projections to the limbic system and within distinct areas of the corpus callosum (CC), the latter both for HSP and PLS. In summary, DTI was able to define a characteristic WM pathoanatomy in motor and extra-motor brain areas, such as the CC and the limbic projectional system, for different MNDs via whole brain-based FA assessment and quantitative fiber tracking. Future advanced MRI-based investigations might help to provide a fingerprint-identification of MNDs.

Contribution of the anterior insula to temporal auditory processing deficits in developmental dyslexia.

Developmental dyslexia has been assumed to arise from general auditory deficits, compromising rapid temporal integration both of linguistic and nonlinguistic acoustic stimuli. Because the effort of auditory temporal processing of speech and nonspeech test materials may depend on presentation rate, fMRI measurements were performed in dyslexics and controls during passive listening to series of syllable and click sounds, using a parametric approach. Controls showed a decrease of hemodynamic brain activation within the right and an increase within the left anterior insula as a function of the presentation rate both of click as well as syllable trains. By contrast, dyslexics exhibited this profile of hemodynamic responses under the nonspeech condition only. As concerns syllables, activation in dyslexics did not depend on presentation rate. Moreover, a subtraction analysis of hemodynamic main effects across conditions and groups revealed decreased activation both of the left and right anterior insula in dyslexics compared to controls during application both of click and syllables. These results indicate, in line with preceding studies, that the insula of both hemispheres is involved in auditory temporal processing of nonlinguistic auditory stimuli and demonstrate, furthermore, that these operations of intrasylvian cortex also extend to the linguistic domain. In addition, our data suggest that the anterior insula represents an important neural correlate of deficient temporal processing of speech and nonspeech sounds in dyslexia.

Dysphagia due to unilateral infarction in the vascular territory of the anterior insula.

We describe a patient who suddenly developed dysphagia for liquids as the sole manifestation of stroke. Magnetic resonance imaging (MRI) revealed a right-sided infarction of the superior part of the anterior insula and a small portion of the adjacent medial frontal operculum. These findings confirm the role of the anterior insula as a critical area in humans with regard to the origin of dysphagia.

Cerebral white matter alterations in idiopathic restless legs syndrome, as measured by diffusion tensor imaging.

In search for the pathoanatomical correlate of the restless legs syndrome (RLS), various neuroimaging and electrophysiological techniques have demonstrated partly conflicting results of cortical, subcortical, brainstem, and spinal alterations. In a novel approach, the delineation of potential cerebral white matter tract disruption was investigated by application of quantitative whole brain-based diffusion tensor imaging (DTI) to a well characterized group of 45 patients with idiopathic RLS. The data of patients and 30 healthy controls were statistically compared including computation of regional fractional anisotropy (FA) as a quantitative marker of white matter integrity by use of the tensor imaging and fiber tracking software. In the patient group, multiple subcortical areas of significantly reduced FA were observed bihemispherically in close proximity to the primary and associate motor and somatosensory cortices, in the right-hemispheric thalamus (posterior ventral lateral nucleus), in motor projectional fibers and adjacent to the left anterior cingulum. Together with the results of a recent study by use of an MRI-based gray matter analysis, which localized RLS-associated changes in the sensorimotor cortices, these findings gave support to an altered subcortical network, with the major component of altered cerebral sensorimotor pathways, within a hodological concept of the RLS pathoanatomy.

Dropped-head syndrome due to steroid responsive focal myositis: a case report and review of the literature.

Isolated severe weakness of the paraspinal musculature, either at the cervical level leading to a "dropped head syndrome" or at the thoracic level leading to a "bent-spine syndrome", is a rare disorder. Etiologically it may be present in a variety of neurological diseases including Parkinson's disease, multiple system atrophy, neuromuscular or motor neuron diseases, as well as non-inflammatory, inflammatory, dystrophic or metabolic myopathies. We present a previously healthy 74-year-old man with a 2-month history of progressive difficulty in lifting his chin off his chest. Magnetic resonance imaging and skeletal muscle biopsy revealed an isolated myositis of the neck extensor and trapezius muscles, which responded well to steroid therapy. This case and other rare reports obtained from a systematic review of the literature indicate that in a subgroup of patients "dropped head syndrome" or "bent spine syndrome" is caused by a myositis, which emphasizes the necessity to obtain a MRI examination, as well as a muscle biopsy to diagnose this potentially treatable disease.

Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis.

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.

Diffusion tensor imaging and tractwise fractional anisotropy statistics: quantitative analysis in white matter pathology.

BACKGROUND: Information on anatomical connectivity in the brain by measurements of the diffusion of water in white matter tracts lead to quantification of local tract directionality and integrity. METHODS: The combination of connectivity mapping (fibre tracking, FT) with quantitative diffusion fractional anisotropy (FA) mapping resulted in the approach of results based on group-averaged data, named tractwise FA statistics (TFAS). The task of this study was to apply these methods to group-averaged data from different subjects to quantify differences between normal subjects and subjects with defined alterations of the corpus callosum (CC). RESULTS: TFAS exhibited a significant FA reduction especially in the CC, in agreement with region of interest (ROI)-based analyses. CONCLUSION: In summary, the applicability of the TFAS approach to diffusion tensor imaging studies of normal and pathologically altered brains was demonstrated.

Atypical botulism sparing palsy of extraocular muscles.

Recent studies of patients with botulism found ophthalmoplegia as a characteristic clinical sign. Here we illustrate a very rare case of atypical foodborne botulism with multiple bilateral cranial nerve palsies sparing palsy of extraocular muscles. Therefore, the classical diagnostic pentad of botulism (dry mouth, nausea, vomiting, dysphagia, diplopia, fixed dilated pupils) may be of limited sensitivity in single cases.

Pharyngolaryngeal Sensory Deficits in Patients with Middle Cerebral Artery Infarction: Lateralization and Relation to Overall Dysphagia Severity.

BACKGROUND: Dysphagia is a frequent and dangerous complication of acute stroke. Apart from a well-timed oropharyngeal muscular contraction pattern, sensory feedback is of utmost importance for safe and efficient swallowing. In the present study, we therefore analyzed the relation between pharyngolaryngeal sensory deficits and post-stroke dysphagia (PSD) severity in a cohort of acute stroke patients with middle cerebral artery (MCA) infarction. METHODS: Eighty-four first-ever MCA stroke patients (41 left, 43 right) were included in this trial. In all patients, fiberoptic endoscopic evaluation of swallowing (FEES) was performed according to a standardized protocol within 96 h after stroke onset. PSD was classified according to the 6-point fiberoptic endoscopic dysphagia severity scale. Pharyngolaryngeal sensation was semi-quantitatively evaluated by a FEES-based touch technique. RESULTS: PSD severity was closely related to the pharyngolaryngeal sensory deficit. With regards to lateralization of the sensory deficit, there was a slight but significant preponderance of sensory loss contralateral to the side of stroke. Apart from that, right hemispheric stroke patients were found to present with a more severe PSD. CONCLUSIONS: This study provides evidence that an intact sensory feedback is of utmost importance to perform nonimpaired swallowing and highlights the key role of disturbed pharyngeal and laryngeal afferents in the pathophysiology of PSD.

Primary progressive aphasia accompanied by environmental sound agnosia: a neuropsychological, MRI and PET study.

As part of the frontotemporal dementias, primary progressive aphasia (PPA) is typically characterized by nonfluent speech with paraphasias, but there is growing evidence that also a fluent variant of PPA exists. We describe a patient suffering from PPA who adds to the broad clinical spectrum of this disorder. Moreover, we report for the first time that PPA may be associated with severe impairment in meaningful nonverbal sound recognition (environmental sound agnosia). These neuropsychological findings were found to be associated with distinct focal alterations in functional and structural neuroimaging.

Systematic review of early recurrent stenosis after carotid angioplasty and stenting.

BACKGROUND AND PURPOSE: Carotid angioplasty and stenting (CAS) has emerged as a potential alternative to endarterectomy (CEA) for the treatment of carotid artery disease. Aside from the periprocedural complication rates, the benefits of CAS will be affected by the incidence of recurrent carotid stenosis. METHODS: We conducted a systematic analysis of all peer-reviewed studies reporting on the rate of restenosis (> or =50%) after CAS based on duplex ultrasound or angiography that were published between January 1990 and July 2004. We identified 34 studies that reported on a total of 4185 patients with a follow-up of 3814 arteries over a median of 13 months (range, 6 to 31 months). The ultrasound criteria and the lower thresholds for defining a recurrent stenosis were very heterogeneous. RESULTS: The cumulative restenosis rates after 1 and 2 years were approximately 6% and 7.5% in those studies, which used a lower restenosis threshold > or =50% to 70% and approximately 4% in the first 2 years after CAS in those studies, which used a lower restenosis threshold >70% to 80%. CONCLUSIONS: In reviewing the current literature, the early restenosis rates after CAS compare well with those reported for CEA. However, this analysis of the peer-reviewed literature also indicates that the early restenosis rates after CAS might be higher than previously suggested in observational surveys. Therefore, an active follow-up of all stented arteries seems to be warranted. Moreover, the bulk of endovascular data are derived from small studies with short follow-up periods so that the long-term durability of CAS still needs to be established in large trials. Ideally, these studies should use a clear and uniform definition of restenosis and identical follow-up schedules.

Neural correlates of rate-dependent finger-tapping in Parkinson's disease.

Functional imaging demonstrated hemodynamic activation within specific brain areas that contribute to frequency-dependent movement control. Previous investigations demonstrated a linear relationship between movement and hemodynamic response rates within cortical regions, whereas the basal ganglia displayed an inverse neural activation pattern. We now investigated neural correlates of frequency-related finger movements in patients with Parkinson's disease (PD) to further elucidate the neurofunctional alterations in cortico-subcortical networks in that disorder. We studied ten PD patients (under dopaminergic medication) and ten healthy subjects using a finger-tapping task at three different frequencies (1-4 Hz), implemented in an event-related, sparse sampling fMRI design. FMRI data were analyzed by means of a parametric approach to relate movement rates and regional BOLD signal alteration. Compared to healthy controls, PD patients showed higher tapping response rates only during the lower 1 Hz condition. FMRI analysis revealed a rate-dependent neural activity within the supplemental motor area, primary sensorimotor cortex, thalamus and the cerebellum with higher neural activity at higher frequency conditions in both groups. Within the putamen/pallidum, an inverse neural activity and frequency response correlation could be observed in healthy subjects with higher BOLD signal responses in slow frequencies, whereas this relationship was not evident in PD patients. We could demonstrate similar behavioral responses and neural activation patterns at the level both of frontal and cerebellar areas in PD compared to healthy controls, whereas regions like the putamen/pallidum appear to be still dysfunctional under medication regarding frequency-related neural activation. These findings may, potentially, serve as a neural signature of basal ganglia dysfunctions in frequency-related task requirements.

Relation between regional functional MRI activation and vascular reactivity to carbon dioxide during normal aging.

Recent blood oxygenation level-dependent (BOLD) functional magnetic resonance imaging studies have shown a reduction of cerebral activation during aging, which may be associated with age-related changes of the cerebral vascular system. The authors used a global hypercapnic breath-holding challenge to define nonneuronal contributions to a significantly reduced activation in the primary sensorimotor cortex during finger tapping in a group of old (n = 6; mean age 65 years) compared with a group of young (n = 6; mean age 27 years) subjects. Within significantly activated voxels in both groups during finger tapping, the mean BOLD signal amplitudes were significantly smaller in the group of older subjects for both tasks. In those voxels showing significant activation only in young subjects during finger tapping, the response to hypercapnia was also greatly diminished in older subjects. The attenuated hypercapnic BOLD signal response in older subjects within this region suggests that age-dependent changes of the cerebral vasculature may alter the neuronal-vascular coupling. In older subjects, cerebral vessels may not react as effectively in response to a vasodilating stimulus, which will lead to differences in the number of voxels that pass a criterion threshold despite similar neuronal activation.