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Evaluation of Symptoms 79
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their beginning, time characteristics (intermittent or constant occurrence), and influencing factors. How­ever, it is important to know about the influence of bolus consistency on swallowing. In neurologic patients with impaired swallowing reflex and unco­ordinated swallow, swallowing liquids is more diffi­cult than semisolids, because they cannot be controlled well. Patients with obstructive diseases (tumors, strictures, webs) often report sticking of solid food, whereas patients suffering from esophageal motility disorders complain about swallowing problems with both liquids and solid food. To estimate the patient’s oral intake, a description of his meals and weight during the last weeks may be valuable.
Furthermore, information regarding previous illnesses andtherapies (surgery, radiation—often years before), as well as medication, needs to be obtained. The side effects of many drugs can impair swallowing: psychopharmacological drugs possibly interfere with the swallow reflex or induce xerostomia, antipsychotic drugs can cause extrapyramidal symptoms, and spasmolytics may weaken muscles. It has to be pointed out that changes in voice, speech and language should be carefully noted. Above all, the examinershould also take into consideration the patient’s general condition, his nutritional status (body weight), and posture, and cognitive and emotional state. Due to the epidemio­logical changes, dementia will become a challenging problem in our society, and dysphagia may be an early or accompanying symptom. Depending on the patient’s needs, a holistic approach, including the mini mental status (Folstein et al. 1975) or the mini nutri­tional assessment MNA can help to judge the patient’s clinical condition.
Questionnaires may help describe the impaired quality of life due to dysphagia (e.g., The MD Anderson dysphagia inventory, Chen et al. 2001, the dysphagia handicap index, Silbergleit et al. 2012).
TM
(Nestlé Nutrition Services),
(with sensory testing), see also Sect. 6.2.4) has to be performed. It is a non-invasive dynamic procedure that delivers an immediate evaluation of pharyngeal swal­lowing function and directly visualizes the upper aerodigestive tract. However, it has the following lim­itations: no direct visualization of the bolus on it’s entire way from the oral cavity to the stomach, no visualization during the swallow, novisualization of the oral and esophageal phases and pharyngo-esophageal segment. Therefore, a videofluoroscopic swallowing study (Ekberg 1992;Logemann1993, 1998) has to be carried out routinely in many cases as a complementary dynamic diagnostic procedure.
7.3 Further Examinations
If necessary, further diagnostic methods are used (Table 4). Depending on the results of videoendo­scopic and videofluoroscopic swallowing studies, es­ophago-gastroscopy can eventually be performed as a first-line diagnostic method to exclude tumorous lesions or reveal disturbances in the esophageal phase (see also Sect. 6.3). Scintigraphy enables the quantifi­cation of bolus transit and aspiration. For an evaluation of neurogenic dysphagia, cranial magnetic resonance imaging (MRI) may detect intracranial lesions responsible for dysphagia. Impedance pH-metry rep­resents the gold standard for diagnostics of suspected gastroesophageal (-pharyngeal) reflux disease. To measure the pressure in the pharynx (especially before surgery of the pharyngo-esophageal segment) imped­ance manometry, which allows measurement of the intrabolus pressures and pharyngeal contraction, is recommended. Cervical auscultation of the pharyngeal swallow did not gain wide acceptance.
8 Conclusion
7.2 Basic Diagnostic Procedure
The ENT examination plays an important ‘‘key’’ role in the diagnostic work-up because it allows a direct mor­phological and functional analysis of the upper aero­digestive tract. Since clinical observation and palpation of the swallow alone do not meet the diagnostic demands, a videoendoscopic swallowing study, (FEES (ST), fiberoptic endoscopic evaluation of swallowing
Swallowing disorder symptoms necessitate an inter­disciplinary, holistic, and thorough diagnostic work­up that reveals etiologic factors and pathophysiolog­ical components. The patient should be asked precise questions relating to symptoms, and valuable infor­mation should be obtained, allowing suitable diag­nostic and therapeutic measures to be taken. Above all, the adequacy of oral nutrition and the presence or absence of aspiration is within the focus of diagnostic
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and therapeutic interests. Due to an absent cough reflex, aspiration may occur silently. Therefore, diagnostic methods must enable direct visualization of aspiration by videoendoscopy and videofluoros­copy. Aspiration cannot be diagnosed or excluded by patient history or clinical observation alone. How­ever, for appropriate management, the patient’s symptoms should be carefully evaluated.
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(Oropharyngeal Dysphagia). Ätiologie, Klinik, Diagnostik
und Therapie von Schluckstörungen, Thieme, Stuttgart Brown M, Glassenberg M (1973) Mortality factors in patients
with acute stroke. JAMA 224:1493–1495 Chen AY, Frankowski R, Bishop-Leone J, Hebert T, Leyk S,
Lewin J, Goepfert H (2001) The development and valida-
tion of a dysphagia-specific quality-of-life questionnaire for
patients with head and neck cancer: the MD Anderson
dysphagia inventory. Arch Otolaryngol Head Neck Surg
127:870–876 Denk D-M, Bigenzahn W (1999) Diagnostik oropharyngealer
Dysphagien (Diagnostics of oropharyngeal dysphagia). In:
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gien. Ätiologie, Klinik, Diagnostik und Therapie von
Schluckstörungen, Thieme, Stuttgart, pp 33–65 Denk D-M, Kaider A (1997) Videoendoscopic biofeedback: a
simple method to improve the efficacy of swallowing
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Dysphagien. Eine standortbestimmmung [Management of
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661–72 Ekberg O, Hamdy S, Woisard V, Wuttge-Hannig A, Ortega P
(2002) Social and psychological burden of dysphagia: its
impact on diagnosis and treatment. Dysphagia 17:139–146 Ekberg O (1992) Radiologic evaluation of swallowing. In:
Groher ME (ed) Dysphagia. Diagnosis and Management.
Butterworth-Heinemann, Stoneham, pp 163–195 Folstein MF, Folstein SE, McHugh PR (1975) Mini-mental
state (a practical method for grading the state of patients for
the clinician). J Psychiatr Res 12:189–198 Garliner D (1974) Myofunctional therapy in dental practice.
Bartel, New York Groher ME, Bukatman R (1986) The prevalence of swallowing
disorders in two teaching hospitals. Dysphagia 1:1–3
Hannig C, Wuttge-Hannig A, Hess U (1995) Analyse und
radiologisches staging des typs und schweregrades einer aspiration (analysis and radiological staging of type and grade of aspiration). Radiologe 358:741–746
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Lopatin D, Loesche WJ (1998) Predictors of aspiration pneumonia: how important is dysphagia? Dysphagia 13: 69–81
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and blood pressure as indirect objective physiologic markers to predict aspiration. Dysphagia 15(4):201–205
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N, Lee SH, Choo PW (2001) Accuracy of bedside clinical methods compared with fiberoptic endoscopic examination of swallowing (FEES) in determining the risk of aspiration in acute stroke patients. Dysphagia 16(1):1–6
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disorders. Pro-ed, Austin
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disorders. Pro-ed, Austin
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of swallowing, 2nd edn. Pro-ed, Austin
Logemann JA (1995) Dysphagia: evaluation and treatment.
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skeletal hyperostosis causing obstructive laryngeal edema. Eur Arch Otolaryngol 255:256–258
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Streiner DL, Diamant NE (2009) The toronto bedside swallowing screening test (TOR-BSST): development and validation of a dysphagia screening tool for patients with stroke. Stroke 40(2):555–561
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Neurology of Swallowing and Dysphagia
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Mario Prosiegel
Contents
1 Neuroanatomy and Neurophysiology .................... 83
1.1 Cerebral Hemispheres ............................................... 84
1.2 Brainstem................................................................... 85
1.3 Pseudobulbar and Bulbar Palsy ................................ 86
1.4 Upper Esophageal Sphincter..................................... 87
2 Examinations............................................................ 88
2.1 Clinical Examinations ............................................... 88
2.2 Instrumented Methods ............................................... 89
3 Diseases Associated with Neurogenic
Dysphagia.................................................................. 90
3.1 Diseases of the Central Nervous System ................. 90
3.2 Diseases of the Cranial Nerves................................. 98
3.3 Diseases of the Neuromuscular Junction.................. 98
3.4 Diseases of the Muscles ............................................ 99
3.5 Iatrogenic Causes....................................................... 101
3.6 Special Diagnostic Approaches................................. 102
3.7 Therapy ...................................................................... 102
References.......................................................................... 103
Abstract
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.
1 Neuroanatomy
and Neurophysiology
M. Prosiegel (&) Abteilung Neurologie, m&i-Fachklinik Bad Heilbrunn, Wörnerweg 30, 83670 Bad Heilbrunn, Germany e-mail: mario.prosiegel@fachklinik-bad-heilbrunn.de
O. Ekberg (ed.), Dysphagia, Medical Radiology. Diagnostic Imaging, DOI: 10.1007/174_2011_339, Ó Springer-Verlag Berlin Heidelberg 2012
This section deals with neuroanatomical and neuro­physiological basics of normal and abnormal swal­lowing: What role do the cerebral hemispheres and the brainstem play in deglutition? How can the pathogenesis of pseudobulbar as well as of bulbar palsy be explained?
Besides such topics, one focus lies also on the upper esophageal sphincter (UES), because opening deficits of the UES are very frequent in neurogenic
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Fig. 1 Swallowing cortex, corticobulbar fibers, and lower
brainstem. Top left: Right cerebral hemisphere with the frontoparietal operculum (closed circle) and the representa­tional areas of the esophagus (E), pharynx (P) and oral region (O). In this example, the right hemisphere is swallowing­dominant, with more corticobulbar fibers (long thick arrows) projecting to the ipsilateral and contralateral medulla. Top right: Left hemisphere after removal of the operculum; the insula with its anterior swallowing-relevant part (black area) can therefore bee seen. Bottom: Axial view of the lower
dysphagia (for other swallowing muscles see the chapter ‘‘Anatomy and Physiology’’ by O. Ekberg and G. Nylander, this volume).
1.1 Cerebral Hemispheres
In their pioneering work, Penfield and Boldrey (1937) from the Montreal Neurological Institute in Canada performed intraoperative electrical stimulations of the cerebral cortex in awake patients. Thereby, they found certain sensorimotor representational areas with the net result of the well-known sensorimotor homunculus (the ‘‘little man inside the brain’’). With regard to swallowing, they elicited deglutition by stimulation of the frontoparietal operculum, i.e., the lower portion of the precentral gyrus (primary motor
brainstem (medulla; lower part=anterior; upper part=posterior). On the right side (left side of the medulla) the nucleus of the solitary tract (NST), the nucleus ambiguus (NA), and the hypoglossal nucleus (HN) are shown. On the left side (right side of the medulla) the dorsomedial and ventrolateral central pattern generators for swallowing are shown (red area and blue area, respectively). The horizontally lined area corresponds to the site of a dorsolateral medullary infarction with consecutive Wallenberg’s syndrome. For details, see the text
area), of the premotor cortex, and of the postcentral gyrus (primary sensory area)––corresponding to Brodmann’s areas (BA) 4 (motor), 6 (premotor), and 3, 2, and 1 (sensory), respectively.
Magnetic resonance imaging (MRI) and functional imaging of the brain, including functional MRI, pos­itron emission tomography, and magnetoencephalog­raphy, confirmed these earlier findings and showed that also the anterior insula (BA 14–16) is involved in volitional swallowing (Barritt and Smithard 2009; Hamdy et al. 1999; Humbert and Robbins 2007; Riecker et al. 2009). Furthermore, by use of transcranial magnetic stimulation it was found that the esophageal, pharyngeal, and oral muscles are discretely represented within the motor cortex in a rostrocaudal direction, with esophageal muscles being situated more rostrally than the pharyngeal muscles,
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which in turn are more rostral than the oral muscles (Hamdy et al. 1996) (Fig. 1).
Functional brain imaging studies also showed that the swallowing cortex is represented bilaterally, but asymmetrically, i.e., it is (in most people) bigger on one side than on the other. The bigger swallowing cortex is called the dominant one. Swallowing domi- nance is independent of the language-dominant side or of handedness (Barrit and Smithard 2009; Hamdy et al. 1999).
The fibers which project from the motoneurons of the swallowing cortex to both sides of the brainstem are called corticobulbar fibers and constitute the corticobulbar (corticonuclear) tract. When the dom­inant swallowing cortex and/or its corticobulbar fibers are affected, a significant hemispheric dysphagia occurs (hemispheric dysphagia means swallowing problems caused by cortical and/or subcortical lesions of the left and/or the right cerebral hemisphere, i.e., supratentorial lesions) (Fig. 1). Additionally, right-sided cortical lesions are often associated with ‘‘neglect of swallowing,’’ ‘‘food stuffing,’’ and con­secutive problems in the pharyngeal phase (Robbins and Levin 1988), whereas left-sided lesions may cause swallowing apraxia with corresponding prob­lems in the oral phase (Daniels 2000). Independent of these behavioral/neuropsychological problems, left­sided and right-sided areas of the swallowing cortex seem to play different roles during the early and later phase of swallowing, respectively (Teismann et al.
2009).
Besides the abovementioned swallowing areas, other cortical and subcortical regions are involved in swallowing function, such as the supplementary motor area (SMA) corresponding to the medial part of BA 6, the basal ganglia, and many others. The SMA is responsible for the generation of the readiness potential (Bereitschaftspotential), which arises about 1 s before a volitional motor action. It was shown that also a swallowing potential (Schluckpotential) exists, which is generated in the SMA too, but spreads to both primary motor areas (whereas the readiness potential spreads to the motor area which is contra­lateral to the innervated extremity) (Huckabee et al.
2003).
According to Mosier and Bereznaya (2001) one can distinguish two swallowing networks: (1) an ‘‘insular loop’’ including the insula, the primary sen­sorimotor motor cortex, premotor cortex, posterior
parietal cortex, and the SMA/cingulate gyrus; (2) a ‘‘cerebellar loop’’ comprising the cerebellum, the SMA/cingulate gyrus, the inferior frontal gyrus, the secondary sensory cortex, the corpus callosum, and the basal ganglia as well as the thalamus. The influ­ence of the insula within the ‘‘insular loop’’ might be necessary to synchronize the kinematics of the swal­lowing movements, whereas the ‘‘cerebellar loop’’ might optimize and modulate movements using feedback information.
As shown by Power et al. (2007), the swallow response time is prolonged in dysphagic patients as compared with healthy volunteers owing to unilateral hemispheric stroke. Interestingly enough, in these stroke patients a sensory deficit of the faucial pillars was found bilaterally in 66% and the duration of laryngeal delay and the degree of the sensory deficit were associated with the severity of predeglutitive aspiration. Very similar results were found in a recent study by Oommen et al. (2010): the stage transition time duration was significantly longer in 52 post­stroke patients—19 aspirators and 33 nonaspirators— than in 12 healthy controls.
Since the cortical swallowing network comprises many sensorimotor areas, one can speculate that sensory input is very critical for an intact swallowing. This view was confirmed by a recent study in decer­ebrate pigs: the sensory threshold for the swallowing response was increased, since the facilitatory path­ways descending from cerebral structures to the brainstem had been lost (Thexton et al. 2007). Therefore, important roles of the cerebral cortex in deglutition seem to be initiation of swallowing, ensuring a normal coupling of the oral and pharyngeal phase (stage transition time) as well as normal sensory properties of the oropharyngeal region, direct modulation of swallowing, and modification of brainstem swallowing responses—in each case mainly dependent on sensory inputs (see also
Sect. 1.2).
1.2 Brainstem
Doty and Bosma (1956) conducted a pioneering study on the role of the brainstem in swallowing. By elec­trical stimulation of the superior laryngeal nerve with 30 Hz in different animals, including monkeys, they could elicit the complete sequential pattern of
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activation or inhibition of swallowing muscles of the pharyngeal phase. Therefore, they postulated the existence of a swallowing center in the medulla oblongata; this view was confirmed later (for a review, see Jean 2001). There are four swallowing centers—two on each side of the brainstem— for which the term ‘‘central pattern generators’’ (CPGs) for swallowing was coined. The dorsome­dial CPGs (dmCPGs) are situated close beside the nucleus of the solitary tract (NST) and the adjacent reticular formation; they contain so-called master neurons/programming interneurons which generate the temporal–spatial sequence of pharyngeal swal­lowing muscle activation or inhibition. This infor­mation is transmitted to ventrolateral CPGs situated near the nucleus ambiguus (NA); switching neurons/ command interneurons of the ventrolateral CPGs distribute the timed output to the cranial nerve nuclei V and VII in the pons as well as to the cranial nerve nuclei IX, X, and XII in the medulla oblongata (Fig. 1). The functioning of the brainstem central network can be influenced by peripheral inputs—e.g., from oropharyngeal mucosal receptors and muscle spindles of the tongue—as well as by central inputs from the cortex; both inputs converge at the NST and serve in particular to adapt the swallowing drive to properties of the bolus to be swallowed. The brainstem is also important for coordinating interactions between respiration and swallowing (Jean 2001; Miller 1993).
Owing to the role of the brainstem in swallowing, unilateral lesions of the medullary region—such as in Wallenberg’s syndrome caused by unilateral infarc­tions in the supply area of the posterior inferior cer­ebellar artery (Fig. 1)—affecting both ipsilateral CPGs and the NA and the NST, cause complex swallowing disturbances, including unilateral pha­ryngeal paresis (NA), impaired pharyngeal peristalsis (NA and dmCPG), sensory deficits in the oropha­ryngeal region (sensory trigeminal nucleus and NST), and secondary UES opening deficit due to impaired hyolaryngeal excursion and/or primary UES opening disturbance due to impaired sphincter relaxation (dmCPG) (Prosiegel et al. 2005a). Dysphagia may be the sole symptom in dorsolateral medullary infarctions but is—owing to ipsilateral vagal pare­sis—often associated with hoarseness due to unilate­ral vocal cord paresis. Other symptoms belonging to Wallenberg’s syndrome are rotatory nystagmus,
ipsilateral Horner’s syndrome, numbness of the face, and ataxia as well as contralateral hypalgesia and thermhypesthesia.
1.3 Pseudobulbar and Bulbar Palsy
Two frequently occurring syndromes associated with dysphagia are pseudobulbar palsy and bulbar palsy. Pseudobulbar palsy is caused by bilateral lesions of the cerebral cortex and/or its corresponding cortico­bulbar fibers, including those passing through the brainstem. In contrast, bulbar palsy (‘‘bulbus’’ is an outdated term formerly used for the lower brainstem) is due to bilateral lesions of pontine and medullary cranial nerve nuclei or their axons or is due to bilat­eral lesions of the cranial nerves themselves.
1.3.1 Pseudobulbar Palsy
The motoneurons of the swallowing cortex are called first motoneurons or upper motoneurons (UMNs). When the swallowing cortex itself and/or and its axons, i.e., the corticobulbar fibers, are lesioned bilaterally, there is diminished input to the brainstem. The consequence is severe dysphagia which affects predominantly the volitional oral phase. Owing to impaired cortical input, the mem­brane of the motoneurons in the brainstem lower their electrical threshold with consecutive hyperre­flexia (e.g., enhanced masseter reflex) and muscle stiffness in terms of spasticity. There are no muscle atrophies, since the second motoneurons/lower motoneurons (LMNs) in the brainstem are intact and, therefore, able to supply the corresponding muscles with the transmitter acetylcholine. This syndrome is called pseudobulbar palsy and occurs in UMN diseases (UMNDs); examples are amyo­trophic lateral sclerosis (ALS) due to bilateral degeneration of the UMNs or bilateral subcortical infarctions affecting the corticobulbar tracts. In most cases of pseudobulbar palsy, besides dysphagia also dysarthria and chewing problems occur; pathologi­cal crying or laughing is often associated with pseudobulbar palsy too. Bilateral lesions of the corticobulbar tract in the brainstem (e.g., in bilateral anterior mesencephalic infarctions) may also cause pseudobulbar palsy. Very typical for pseudobulbar palsy is automatic–voluntary dissociation: emotional or reflex responses are intact or enhanced, whereas
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volitional activities are disturbed e.g., enhanced palatal reflex, but no elevation of the soft palate during phonation of /A/ or videofluoroscopic study of swallowing (VFSS) is also spasm of the crico­pharyngeal muscle.
1.3.2 Bulbar Palsy
In contrast to pseudobulbar palsy, muscle atrophy occurs when the cranial nerve nuclei in the brainstem (or the motoneurons in the spinal cord)—i.e., the LMNs—are affected. Because of diminished input to the corresponding muscles, the muscular membrane develops a decreased electrical threshold with con­secutive pathological spontaneous activity. This can be assessed electromyographically or seen clinically in the form of fibrillations of the atrophic tongue (jerks of muscle fibers) or fasciculations of the face or body musculature (jerks of groups of muscle fibers). Other features are weakness of the orofaciopharyn­geal muscles and decreased muscle tone in terms of hypotonia with diminished reflexes as well as bulbar (slurred) speech. This syndrome, which is caused by affection of the LMNs, is called bulbar palsy and occurs in LMN diseases (LMNDs) such as ALS (ALS is an example of a combined UMND and LMND). Bulbar palsy may also be caused by lesions of the fibers of the cranial nerve nuclei or of the cranial nerves themselves.
1.4 Upper Esophageal Sphincter
The UES is defined as a high-pressure zone with a rostrocaudal extension of 2–6 cm, which maintains a closed pharyngoesophageal junction and opens pha­sically during various physiological states (Lang and Shaker 1997). It consists of striated muscles comprising the inferior pharyngeal constrictor, the cricopharyngeal muscle and the upper esophageal musculature. In contrast to the other swallowing muscles, the UES forms a network together with connective tissue (approximately 40%) and consists of more than 70% of slow twitch (tonic, type I) fibers. The number of these tonic fibers is especially high in the horizontal part of the cricopharyngeal muscle as compared with its oblique part as well as in the slow inner layer as compared with the fast outer layer of the UES. Acetylcholine is the transmitter which binds to the nicotinic motor end plates of the muscle fibers,
but many other transmitters (mainly found in blood vessels, glands, and the mucosa) occur in the UES, including neuropeptide Y, calcitonin-gene-related peptide, tyrosine hydroxylase, substance P, and vasoactive intestinal polypeptide. The slow inner layer is innervated by cranial nerve IX (glossopha­ryngeal nerve), whereas the fast outer layer is sup­plied by different branches of cranial nerve X (vagus nerve) in the following manner: (1) inferior pharyn­geal constrictor ––pharyngoesophageal nerve forming the pharyngeal plexus; external superior laryngeal nerve; (2) cricopharyngeal muscle––pharyngeal plexus; external superior laryngeal nerve; recurrent laryngeal nerve (RLN); (3) upper esophageal muscu­lature––recurrent laryngeal nerve (for a review, see Mu and Sanders 2007).
UES opening is a very complex event. Firstly, relaxation of the UES muscles occurs (as can be shown electromyographically). Secondly, about 100 ms later, there is a reduction of UES pressure (as can be shown by use of manometry). Thirdly, again about 100 ms later, UES opening occurs caused by two forces which have to overcomethe resistance (R)ofthe sphincter: (1) traction forces, exerted by the suprahyoid muscles during anterior–superior hyolaryngeal excursion, widen the cricopharyngeal muscle, since the crico­pharyngeal muscle originates from the arch of the cri­coid cartilage; (2) tongue base retraction with approximation ofthe baseof thetongue tothe posterior pharyngeal wall generates the force responsible for the primary pressure on the descending bolus (shortening of the pharynx helps the bolus meet the UES). These forces (F)/pressures (P) can be described mathemati­cally as follows: F
Traction
? P
Intrabolus
[ R
UES
(for a review, see Lang et al. 1991). Pharyngeal peristalsis is also important, but its predominant role is to clear pharyngeal bolus residuals.
Defective opening of the UES due to increased tonicity occurs frequently in patients with dysphagia due to medullary lesion (such as in Wallenberg’s syndrome or Parkinson’s disease (Williams et al.
2002) and is sometimes called cervical achalasia.
Defective tonicity of the UES between swallows (cervical chalasia) may occur in motonic dystrophy, myasthenia gravis, during ‘‘off’’ periods of Parkinson’s disease, and after radiotherapy of the neck; it is often asymptomatic, but may cause aerophagia, belching, and/or regurgitation of refluxed material (Ekberg and Olsson 1995).
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chewing or swallowing; residuals of food in the
2 Examinations
Diagnostics in (suspected) neurogenic dysphagia comprise history taking, clinical swallowing exami­nation, and instrumental methods. Bedside screening tests are only necessary in certain cases, which are described in Sect. 2.1.2.
Special diagnostic approaches such as laboratory
examination and MRI are dealt with in Sect. 3.6.
mouth after swallowing; nasal regurgitation of food or liquids; feeling of ‘‘food sticking’’ (where?); need for repetitive swallowing in order to remove all residuals; breathing problems; prior or current disease such as chronic pulmonary obstructive disease; prior surgery/ medical therapy such as anterior cervical surgery, carotid endarterectomy, or radiochemotherapy for head and neck cancer; current status such as depen­dence on a percutaneous endoscopic gastrostomy (PEG) tube, nasogastric tube or tracheal cannula; prior and current medication.
2.1 Clinical Examinations
2.1.2 Bedside Screening Examinations
2.1.1 History Taking: Signs and Symptoms in Neurogenic Dysphagia
In many textbooks or articles one can find the state­ment that dysphagia for liquids is typical for neuro­genic dysphagia. Although it often occurs, it is, however, not a pathognomonic symptom for dys­phagia of neurogenic origin. In reality, there is a broad range of different signs and symptoms occur­ring in patients with neurogenic dysphagia. During history taking it is helpful to use a checklist of questions and to ask the patient and his/her relatives to try to answer them as accurately as possible. Interestingly enough, in many cases the relatives may observe, e.g., disturbances of feeding behavior or postural changes which are not or not to the same extent realized by the patients themselves.
Some of the most important signs and symptoms are listed in the following: abrupt or gradual begin­ning of swallowing problems; difficulty with control of saliva; problems with liquids and/or thick consis­tencies; problems with warm, hot, or cold liquids and/ or food; involuntary weight loss; eating and/or drinking more slowly than in the time before symp­tom onset; eating and/or drinking smaller portions than in the time before symptom onset; unexplained fever and/or pneumonia; coughing and/or choking and/or voice change (e.g., wet, hoarse, nasal) after eating and/or drinking; drooling and/or sialorrhea; increase of secretions; dry mouth; articulation prob­lems (e.g., slurred speech); feeling of a ‘‘lump in the throat’’; fear of swallowing; pain during swallowing (where?); change of head or trunk posture during swallowing; chewing problems; problems to propel the bolus from the mouth backwards into the pharynx; problems to hold the bolus in the mouth during
Bedside screening examinations should predict the presence or absence of dysphagia or aspiration with sufficient sensitivity and specificity (more than 70%) (Doggett et al. 2002). A bedside screening examina­tion seems to be especially helpful in acute illnesses such as stroke; rapid therapeutic decisions have to be made in those situations with regard to oral admin­istration of food and water versus nil by mouth, because there is often not enough time to perform a clinical swallowing examination or instrumental methods within 72 h. Bedside screening examinations can, however, never replace an accurate clinical swallowing examination or an instrumental method such as flexible endoscopic evaluation of swallowing (FEES) or VFSS (see Sect. 2.2), since the latter ones are necessary for the assessment of the individual swallowing disturbance patterns and thus for applying the corresponding therapeutic interventions.
With regard to acute stroke, Hinchey et al. (2005) convincingly demonstrated the importance of an early screening procedure, which is reflected by the title of their article: ‘‘Formal dysphagia screening protocols prevent pneumonia.’’ Indeed, the most dangerous complication of dysphagia is aspiration pneumonia; but also malnutrition—defined as a body mass index of less than 18.5 kg/m elderly persons)—is an important variable, since its occurrence during the acute stroke phase correlates with a poor clinical outcome and with a prolonged length of stay in the hospital (Finestone et al. 1996; Gariballa et al. 1998).
The Standardized Swallowing Assessment was developed for use by nurses within the first 24 h after stroke; the sensitivity and specificity for detection of dysphagia were 97% and 90%, respectively; a kappa
2
(or less than 20 kg/m2in
Neurology of Swallowing and Dysphagia 89
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value of 0.88 indicated good agreement with sum­mative clinical judgment of swallow function (Perry
2001a, b). The Standardized Swallowing Assessment
consists of a preswallow screening checklist com­prising seven questions/symptoms concerning level of consciousness, ability to sit upright, volitional cough, control of saliva, licking of the lips, breathing, and voice; thereafter, a swallow screen consisting of a water test is performed with consecutive recommen­dations for interventions (e.g., in the case of problems such as coughing or choking after administration of a teaspoonful of water, the recommendation is ‘‘patient nil by mouth,’’ ‘‘refer to speech and language therapist’’).
A well-known instrument for assessing the risk of aspiration in the acute stroke phase was developed by Daniels et al. (1997), often referred to as the Daniels test. In their study on 59 patients (within 5 days of admission), the authors determined whether risk fac­tors detected in the clinical examination approximated the VFSS in identification of dysphagia severity. They found that occurrence of two or more of the following variables predicted aspiration with a sensitivity of
92.3% and a specificity of 66.7%, respectively: dysarthria, dysphonia, abnormal (decreased or absent) gag reflex, abnormal volitional cough, cough after swallow, and voice change after swallow. The last two items were assessed by use of a water test in the following manner: patient in sitting position; 5-ml liquid bolus administered from a cup or straw, progressed to 10- and 20-ml volumes; phonation of /Ah/ (change in vocal quality?); all volumes admin­istered twice, i.e., a total of 70 ml; termination of the test in the case of cough or voice change immediately after swallowing or within 1 min after ingestion of the liquid.
With regard to aspiration risk and feeding recom­mendations, the clinical utility of the 3-oz water swallow test was examined by Suiter and Leder (2008) in 3,000 patients. The diagnostic categories comprised 850 neurological disorders (most fre­quently stroke) and 232 neurosurgical disorders. The patients were required to drink 3 oz (90 ml) of water without interruption; the criteria for referral for fur­ther assessment of swallowing included inability to complete the task, coughing, choking, or a wet-hoarse vocal quality exhibited either during or within 1 min of test completion. The sensitivity and specificity for assessing the risk of aspiration were 96.5% and
48.7%, respectively. Owing to a high negative pre­dictive value of 98.3%, passing the 3-oz water swal­low test was a good predictor for the ability to tolerate oral diet without further dysphagia testing. Failing the test is, however, not a good predictor for unsuccessful swallowing of water, since the false-positive rate is very high. Because of the large amount of water, this test should not be used in patients with compromised medical status, e.g., in frail elderly persons, patients in the acute stroke phase, or persons with severe bulbar type of ALS.
2.1.3 Clinical Swallowing Examination
The clinical swallowing examination aims at detect­ing disturbances of specific swallowing components as a basis for adequate therapeutic interventions. It comprises—in descending order of cranial nerves— the following examinations: decreased strength of chewing muscles, asymmetry of the mandible, and sensory impairment of the facial and oral region— cranial nerve V; decreased strength and/or motility of facial muscles, fasciculations of the facial muscula­ture, and hypogeusia of the anterior two thirds of the tongue—cranial nerve VII; decreased or absent pal­atal and pharyngeal reflex, unilateral pharyngeal wall paresis (paralyzed side moving towards the healthy side, also called ‘‘Vernet’s mouvement de rideau’’), sensory impairment of the pharyngeal mucosa, impaired phonation (e.g., wet, hoarse, nasal), dis­turbed breathing, e.g., stridor, impaired volitional cough, and hypogeusia of the posterior third of the tongue—cranial nerves IX and X; fibrillations and/or atrophy of the tongue, decreased strength and/or motility, and asymmetry of the tongue during rest (to the healthy side) and protrusion (to the affected side)—cranial nerve XII.
Other findings may include dyskinesia or dystonia of the face, jaw, head, and neck; dysarthria; bucco­facial apraxia; neglect; attention or memory deficits; and impaired vigilance. Of special importance are pseudobulbar and bulbar signs (see Sect. 1.3).
2.2 Instrumented Methods
The two most important instrumental methods are FEES and VFSS. FEES is dealt with in the chapter ‘‘Endoscopy of the Pharynx and Esophagus’’ by D.-M. Denk and R. Schöfl (this volume). Therefore,