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www.nature.com/clinicalpractice/neuro Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis Peter Kühnlein, Hans-Jürgen Gdynia, Anne-Dorte Sperfeld, Beate Lindner-Pfleghar, Albert Christian Ludolph, Mario Prosiegel and Axel Riecker*

SUMMARY Continuing Medical Education online Medscape, LLC is pleased to provide online continuing Amyotrophic lateral sclerosis (ALS) is the most common neurodegenerative medical education (CME) for this journal article, disease of the motor system. Bulbar symptoms such as and allowing clinicians the opportunity to earn CME credit. are frequent features of ALS and can result in reductions in life Medscape, LLC is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to expectancy and quality of life. These dysfunctions are assessed by clinical provide CME for physicians. Medscape, LLC designates examination and by use of instrumented methods such as fiberendoscopic this educational activity for a maximum of 0.75 AMA PRA evaluation of swallowing and videofluoroscopy. Laryngospasm, another Category 1 CreditsTM. Physicians should only claim credit well-known complication of ALS, commonly comes to light during commensurate with the extent of their participation in the intubation and extubation procedures in patients undergoing surgery. activity. All other clinicians completing this activity will be issued a certificate of participation. To receive credit, Laryngeal and pharyngeal complications are treated by use of an array of please go to http://www.medscape.com/cme/ncp measures, including body positioning, compensatory techniques, voice and and complete the post-test. breathing exercises, communication devices, dietary modifications, various safety strategies, and neuropsychological assistance. Meticulous monitoring Learning objectives Upon completion of this activity, participants should be of clinical symptoms and close cooperation within a multidisciplinary able to: team (physicians, speech and language therapists, occupational therapists, 1 Describe the clinical features of amyotrophic lateral dietitians, caregivers, the patients and their relatives) are vital. sclerosis (ALS). 2 Identify the most common bulbar symptoms in keywords amyotrophic lateral sclerosis, dysarthria, dysphagia, patients with ALS. laryngospasm, therapeutic options 3 Review the effects of upper and lower degeneration in ALS on voice and speech. Review criteria 4 List communication aids for managing speech and MEDLINE was used to search for papers dating back to 1995. Papers and reviews swallowing impairments in patients with ALS. relating to amyotrophic lateral sclerosis were searched with the following terms: 5 Describe options for managing salivary flow and “ALS”, “MND”, “amyotrophic lateral sclerosis”, “motor neuron(e) disease” and drooling in patients with ALS. “motorneuron disease”. Papers were further selected for relevance using the following terms: “dysphagia”, “deglutition”, “swallowing”, “management”, “sialorrhea”, Competing interests “aspiration”, “symptom control” and “palliative care”. Additional papers and book The authors, the Journal Editor H Wood and the CME chapters from the authors’ own files were included when judged appropriate. questions author D Lie declared no competing interests.

cme INTRODUCTION Amyotrophic lateral sclerosis (ALS), the most frequent of the motor neuron diseases, is P Kühnlein and H-J Gdynia are Residents in the Department of , A-D Sperfeld is an Associate Professor in the Department of characterized by progressive degeneration of Neurology and Head of the Department for Neuromuscular Disorders, upper and lower motor neurons in the motor B Lindner-Pfleghar is a Speech Therapist in the Department of Neurology, cortex, and . The clinical AC Ludolph is Director of the Department of Neurology, and A Riecker is hallmarks of ALS are progressive limb weakness, an Associate Professor in the Department of Neurology and Head of the respiratory insufficiency, , hyperreflexia, Department for Swallowing Disorders, all at the University of Ulm, Ulm, and bulbar symptoms such as dysarthria and Germany. M Prosiegel is Director of the Department of Neurology at the dysphagia. Neuropsychological disturbances can Rehabilitation Clinic Bad Heilbrunn, Bad Heilbrunn, Germany. also occur, although these are normally subtle and

Correspondence cannot be detected without comprehensive neuro­ 1–4 *University of Ulm, Department of Neurology, Oberer Eselsberg 45, D-89081 Ulm, Germany psychological testing. Bulbar symptoms at ALS [email protected] onset can be observed in up to 30% of patients, and almost all patients demonstrate bulbar involve­ Received 4 February 2008 Accepted 12 May 2008 Published online 17 June 2008 5,6 www.nature.com/clinicalpractice ment at later stages of the disease. Dysphagia doi:10.1038/ncpneuro0853 affects food intake with the complications of

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choking, malnutrition and pulmonary aspira­ Box 1 Clinical assessment of bulbar symptoms in amyotrophic tion. Malnutrition has been shown to be an lateral sclerosis. independent risk factor for death in ALS.7,8 In patients with amyotrophic lateral sclerosis (ALS), bulbar symptoms are assessed In view of the limited treatment options for clinically by examination of the involved anatomical structures, and of the patient’s ALS, palliative care and symptom control are of ability to speak and manage food and drinks. Patients should be assessed major interest. Progressive bulbar symptoms are continually to establish therapeutic strategies to maintain communication and a often disturbing, and they result in reductions sufficient and secure oral intake, and to define aspiration risks. in quality of life and life expectancy.9,10 In this To assess swallowing, the examiner should palpate a dry swallow with two Review, we highlight the importance of early fingers on the thyroid cartilage to assess the degree of laryngeal elevation and detection, as well as appropriate treatment and difficulties with swallow initiation. A test swallow with water can demonstrate management, of bulbar symptoms and compli­ incomplete pharyngeal stripping with residual water on the vocal cords; the cations in patients with ALS, with our emphasis presence of ’moist phonation‘ in the speech immediately after completion of the swallow is indicative of this phenomenon. A marked fall in oxygen saturation on a multidisciplinary approach. (as measured by pulse oximetry) or increased respiration rate after oral intake suggests aspiration.30,33 PATHOPHYSIOLOGY OF BULBAR Facial and lateral jaw movements are usually normal in the early stages of ALS. SYMPTOMS The ability to maintain a lip seal is important for the oral retention of food and The involvement in ALS fluids and permits a positive pressure within the oral cavity to initiate swallowing causes supranuclear symptoms, which are also and facilitate laryngeal elevation. Lip strength can be tested by asking the patient known as pseudobulbar palsy. The clinical to suck on the examiner’s gloved finger. The ability to take food from a utensil characteristics of pseudobulbar palsy are spas­ and to use a straw should also be assessed. The tongue should be examined ticity of the bulbar muscles (jaw, face, soft palate, for fibrillations at rest, and for rapid alternating movements and range of motion, including protrusion and lateral movement. Tongue strength can be tested by pharynx, larynx and tongue), emotional lability having the patient press the tongue against a finger through the cheek. From the (pathological laughing and crying), and a brisk strength, rate, coordination and range of tongue movement, the ability to collect jaw jerk. Degeneration of the lower motor and control a bolus within the mouth and to push the bolus effectively into the neurons with involvement of the cranial nerve pharynx can be inferred. The presence of oral residues after a swallow is a sign of nuclei in the and pons that disturbed tongue function. innervate the bulbar muscles results in a bulbar Intact velar function is essential to create a sufficient intraoral pressure and palsy with flaccid pareses, muscular atrophy, helps to establish a negative pressure in the pharyngeal phase of swallowing. and fasciculations and/or tongue fibrillations. The palate can be examined by stimulating the gag with a tongue blade In addition, loss of motor neurons in the spinal or by asking the patient to make a prolonged “a” sound. The pharyngeal phase cannot be directly observed, but information can be gleaned through observation cord causes muscular weakness, resulting in of swallowing. Coughing before, during or after swallowing might indicate progressive respiratory dysfunction, effortful aspiration. It is important to stress, however, that weakness of the laryngeal communication and decreased voluntary as well closure and respiratory musculature can make patients cough insufficiently or as reflexive coughing, all of which can aggravate stop coughing. Pseudobulbar palsy is typically characterized by dissociation the symptoms of dysphagia.11,12 between voluntary and reflexive actions; that is, lack of elevation of the palate during phonation but a brisk palatal reflex when the palatal arch is touched. CLINICAL PRESENTATION The laryngeal structures usually appear normal in patients with ALS, although Dysarthria and dysphagia are the most common incomplete vocal fold closure, bowing and supraglottic hyperfunction can be 14 bulbar symptoms in ALS. Studies have reported observed. Examination can show both spasticity and weakness of the vocal folds, although weakness is usually predominant in the later stages of the dysarthria in 93%, dysphagia in 86% and tongue disease. Adduction of the vocal folds during vocalization is sometimes weak, fasciculations in 64% of patients with ALS who resulting in a loss of vocal volume. Abduction is usually incomplete, and the 13,14 have bulbar symptoms. The clinical assess­ glottic airway can be limited to as little as 1 mm. In cases of pseudobulbar ment of bulbar symptoms requires a multi­ involvement, hyperadduction of the vocal cords is reported, whereas in disciplinary approach, comprising history taking, bulbar involvement a pattern of hypoadduction predominates.30,64 evaluation of weight and BMI, respiratory func­ tion assessment and clinical examination of swallowing and speech (Box 1). perceptible changes include a harsh, hoarse or Dysarthria strained voice, a breathy speech pattern with The underlying pathology of dysarthria is a short phrases, inappropriate pauses in speech, flaccid or spastic paresis of the musculature imprecise consonants, hypernasality, and a of the face, tongue, lips, palate, pharynx and decreased range of pitch and loudness.15–19 larynx. In the early stages of ALS, patients often Through a multitude of subtle movements, report mild changes in voice and speech. These the tongue acts as the principal articulator of

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speech. Slurring can be one of the first symp­ disease course, all muscles of the tongue, lips, toms of impaired tongue function in ALS, and as palate, jaw, pharynx, larynx and upper trunk can the disease progresses patients increasingly fail to be affected,25–27 resulting in inefficient bolus produce accurate speech, ultimately developing transport. As the motility, strength and some­ anarthria. The tongue can be weak and atrophic times the coordination of the orofacial and lingual with fasciculations, or spastic with slowed muscles decrease, difficulties in oral preparation, movements in all directions, depending on the mastication and oral transport of food emerge, underlying pathology.20 which result in oral, pharyngeal and/or laryngeal Flaccid or spastic paresis of the oropharyngeal residues of food or secretions. Patients with ALS muscles results in weak speech production and generally experience difficulties in managing problems with articulation, causing impaired dry, tough-textured or crumbly food and thin intelligibility of speech. Spastic orofacial muscles liquids, although the specific nature of the diffi­ are typically retracted, causing problems with lip culties depends on the patient’s individual clinical closure, whereas flaccid paresis is often associated pathology. Typical symptoms are an increased with drooping lips and drooling of saliva. eating time, unintentional weight loss, and fever Weakness of the soft palate and pharyngeal of unknown origin or recurrent respiratory infec­ musculature causes hypernasality and decreased tions. With increasing loss of tone and strength intelligibility of speech owing to insufficient in the muscles that control lip closure and diffi­ nasopharyngeal closure and reduced oral airflow. culty in swallowing their saliva, patients tend to These impairments result in breathy speech, and drool.28–30 Moreover, the inability to keep the lips in an inability to generate long phrases owing to closed results in increased breathing through the inadequate breath control.21 mouth, leading to thickening of oral secretions. The laryngeal musculature controls movement In addition, weakness of the hypopharyngeal of the vocal cords and larynx. The vocal cords muscles causes poor pharyngeal stripping during are open during respiration, and for vocaliza­ swallowing, as well as pooling of saliva in the tion they move to the midline and vibrate as mouth and oropharynx. the airstream passes through. Changes in pitch Typical features of laryngeal involvement are achieved by extension or contraction of are the failure of the larynx to move superiorly the vocal cords from movements of the larynx. or anteriorly during the swallowing reflex, Flaccid paresis of laryngeal muscles owing to and incomplete closure of the larynx during predominant degenera­ elevation.30 In the early stages of ALS, proce­ tion produces a soft, weak, low-pitched and dures such as tactile or thermal stimulation monotonous voice. Severe upper motor neuron can be used to compensate for late triggering impairment, by contrast, makes the voice sound of the swallowing reflex, as described later in harsh and strained.22,23 Dysarthria with a spas­ this article. With increasing weakness of laryn­ modic component (focal laryngeal ) has geal adduction, however, these approaches also been described.17 become ineffective, thereby increasing the The pattern of speech impairment in ALS risk of pulmonary aspiration (that is, passage usually mixes symptoms of flaccid and spastic of material into the larynx below the level of pareses of the bulbar muscles.16 Furthermore, the vocal cords). Sensory deficits of unknown a decrease in respiratory function compromises origin in the pharynx have been shown to be speech and voice, producing, for example, frequent, and these deficits further increase the effortful, breathy and strained phrasing, or risk of aspiration.31,32 Penetration—which, affecting prosodic features such as stress, rhythm in contrast to aspiration, is defined as passage and tone, thereby limiting expressive communica­ of material into the larynx above the glottic tion.20 Early detection of impaired speech and level—or aspiration without any subsequent voice is crucial for timely provision of speech cough are termed silent.23,30,33 Reduced tongue and language therapy in patients with ALS. force, delayed triggering of the swallowing reflex and weak elevation of the hyoid and Dysphagia larynx result in reduced opening of the upper Impairment of swallowing in ALS can result esophageal sphincter, with retention of saliva, from weakness or spasticity of the muscles that food and liquids in the valleculae, the piriform are innervated by trigeminal, facial, hypoglossal, sinuses and the postcricoid region. In addition, glossopharyngeal or vagal nerves.24 During the opening of the pharyngoesophageal segment

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can be disturbed, especially in patients with Compensatory speech techniques pseudobulbar palsy, owing to a hyperreflexic If disease progression is slow, speech therapy can and hypertonic upper esophageal sphincter.34 help to correct ineffective compensation strate­ A forceful cough reflex is crucial to enable gies in patients with ALS. In the early stages of clearance of aspirated food or saliva, but in the disease, for example, patients tend to use patients with ALS coughing is often impaired too much force for their voice and articulation by a progressive weakness of the respiratory and as they become aware of difficulties in being laryngeal muscles. In some cases, therefore, it understood. This forceful speech, as well as an will be necessary to discuss whether a patient increasing loss of breath, often leads to a further could benefit from a tracheostomy.23 deterioration of intelligibility. Patients can learn and perform breathing and relaxation exer­ Laryngospasm cises to optimize usage of the available respira­ Laryngospasm is defined clinically as a tory resources. In speech and language therapy, paroxysmal episode with the sensation that patients are instructed to use their voice and air cannot be moved in and out, accompanied articulation patterns, as well as their breathing by inspiratory stridor. This condition is caused by rate during speech, in the most economical rapid and forceful contraction of the laryngeal way, so as to optimize usage of the available sphincter, which sometimes results in complete respiratory resources (Lindner-Pfleghar B et al., upper airway occlusion.35 Laryngospasm usually unpublished data). In addition, facilitation tech­ comes to light during intubation or extubation niques, such as vibration, or application of ice to procedures in patients undergoing surgery. the involved musculature, can help to decrease In one study by Sperfeld et al., laryngospasm muscle tone and improve speech intelligibility was observed in up to 50% of patients with for a short time period.20,38 X-linked spinobulbar muscular atrophy (Kennedy In patients with ALS who have rapidly progres­ disease), whereas only 2% of the control group, sing disease, voice and articulation therapies do who were in the early stages of ALS, reported not seem to produce notable therapeutic effects; this symptom.36 Laryngospasm was reported in in fact, resistance and isometric exercises, oral about 19% of another group of patients who motility, voice strengthening training and loud­ were in the later stages of ALS.37 Smoking and ness practices can cause a decrease in voice quality gastroesophageal reflux seem to be important and intelligibility.19 risk factors for laryngospasm. On the basis of gastroscopy findings, Sperfeld et al. diagnosed Communication aids gastroesophageal reflux in 3 out of 15 (20%) As dysarthria progresses, the attending speech patients who had Kennedy disease and laryngo­ and language therapist or physician must initiate spasm.36 It is currently assumed that laryngospasm timely provision of augmentative and alterna­ is caused by a combination of gastroesophageal tive communication devices. Beukelman and reflux, aspiration of gastric contents, and functional Ball revealed a rapid decline in sentence intel­ impairment of neurons involved in swallowing ligibility when the speaking rate dropped below and regurgitation. 100 words per minute in patients with acquired neurogenic disorders.39 THERAPY FOR SPEECH IMPAIRMENT The most simple and economical communica­ Various general strategies, as well as more- tion aids are pencil and paper, an alphabet board, specialized speech and language therapies, can be and word or picture boards. The patient must, employed to facilitate speech in patients with ALS. however, have sufficient function of the upper Positioning and physical comfort can decrease extremities in order to use these aids. If such abnormal muscle tone and prevent or reduce tools are no longer an option, other augmen­ reflexive responses or spasticity. Furthermore, tative communication devices are necessary. an optimized body position reduces the effort For example, laser pointers fixed on glasses or required to maintain body position and respira­ a headband can be used in combination with tion. Physiotherapists or occupational therapists an alphabet board. Electronic communication can be very helpful in this regard. Background devices with a keyboard or a scanner to detect noise, such as that from a television or radio, and head or eye movements and with a voice output group settings with many people talking at the enable patients to use telephones and computers same time, should be avoided.20 in a very effective way.20,40 Patients can be sent

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for recording of their voice patterns before they and shortens the passage along which food is develop severe dysarthria so that the communica­ transported, thereby reducing the risk of aspira­ tive device can be programed with their own tion.41 To avoid drooling of food or liquids, voice instead of a robotic sound. Timely educa­ patients should be encouraged to seal their lips tion about future prospects helps the patient to with their hand. accept the situation, and early adaptation to a In cases in which predeglutitive or intra­ communication device makes it easier for the deglutitive aspiration caused by weak laryn­ patient to learn how to use it. geal closure or a delayed swallowing reflex is diagnosed (Box 2), the supraglottic swallowing THERAPY FOR SWALLOWING IMPAIRMENT maneuver helps to close the vocal cords during To facilitate swallowing, patients with ALS swallowing (glottal closure). In this maneuver, should be brought into a normal physiological patients hold their breath while swallowing and position while eating and drinking—usually exhale at full force immediately afterwards.42 upright, and supported by adjuvants (e.g. cush­ This method enables food or secretions to be ions and specially designed wheelchairs, tables, expelled from the laryngeal vestibule in order to spoons and cups) if necessary. Although marked avoid aspiration. The technique is appropriate improvement in swallowing is an unrealistic goal for patients with minor oral, laryngeal and/or in this progressive disease, compensatory and respiratory dysfunction. restitutional swallowing therapeutic methods can help to ensure oral alimentation for as long Restitutional methods as possible and also help prevent food aspira­ Sensorimotor exercises aimed at partial resti­ tion.23 An occupational therapist can help by tution of impaired functions might provide providing instruction on the optimum use of some limited benefits to patients with ALS remaining body functions, and by introducing who have swallowing impairments, although special aids, such as utensils that are easier to intensive exercises might merely exhaust the grip for patients with impaired hand function. weakened muscles. The patient or their carers should be advised to If the triggering of the swallowing reflex is practice meticulous hygiene of the oral cavity, delayed or patients show impaired oral bolus as persisting oral residues could cause bacterial control, tactile or thermal stimulation of the infections of the airways. anterior faucial pillars and the tongue before meal times might produce a temporary improvement. Compensatory methods Passive pressure on the hyoid bone (either by the Swallowing in patients with ALS can be improved patient or by an assisting nurse or relative) during by changes of posture, or by the use of special eating can support the elevation of the tongue and swallowing techniques. The various methods thereby trigger the swallowing reflex. should be used in modified form, taking into account the special disease characteristics. It is Dietary modifications important to train not only the patient, but also During meal times, patients with ALS should the carer, as the carer can help the patient to use avoid distractions such as conversation, tele­ methods of compensation during swallowing, as vision or radio. Patients who experience appre­ well as prepare appropriate kinds of meal (e.g. ciable levels of fatigue are advised to eat their fluid, thickened or solid). food as several small meals a day. If an episode Patients with tongue weakness or reduced of choking occurs, the Heimlich maneuver can tongue mobility can tilt their heads backwards be applied by the carer. to support the transport of the food bolus In mild or moderate dysphagia, dietary by gravity. Problems with mastication can modification has been shown to be an effective necessitate a change of diet to a fluid or moist approach. Dieticians can give advice on how to consistency. If the problem is associated with enrich meals by use of foods high in calories, a delayed triggering of the pharyngeal swallow proteins and vitamins, and by the addition of or leaking of food into the pharynx, chin tuck high-energy supplements.43,44 In cases of consti­ (tilting the chin down before swallowing) is a pation caused by abdominal weakness and failure helpful compensatory change of posture. This of glottic closure, dietary fiber can be added position widens the vallecular space, helps to to the diet. The triggering of the swallowing divert food away from the laryngeal vestibule, reflex can be enhanced by emphasizing taste or

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temperature; for example, drinks can be made Box 2 Instrumental assessment of bulbar easier to swallow by cooling them to below room symptoms in amyotrophic lateral sclerosis. temperature. Special eating or drinking aids can Videofluoroscopic study of swallowing (VFSS) is an also be employed. In patients whose swallow is important technique in the evaluation of delayed, thickened fluids are usually better toler­ swallowing disorders in patients with amyotrophic ated than thin fluids as they tend to move more lateral sclerosis, as it provides an image of slowly through the oral cavity. For these patients, the entire duration of the swallow and enables the it is recommended that thickeners in powder complete oropharyngeal tract, including the upper form are added to drinks to minimize the risk esophageal sphincter and the esophagus, to be of penetration and aspiration.45 Soft textures viewed.23 On the basis of VFSS observations, or puréed food can compensate for a poor oral a treatment plan for swallowing therapy can be preparation phase and ease oral and pharyngeal established and a decision made as to whether to provide dietary modification, or whether to instigate transport. Liquid supplements can be helpful, alternative feeding methods or compensatory but beyond a certain stage in the disease choking positioning of the head.65,66 can become frequent, especially with thin Some clinicians advocate the use of a modified liquids. Clinical and instrumental investigations barium swallow to test for aspiration pneumonia in (Boxes 1 and 2) are required to establish whether patients with ALS, although great care should be an individual patient will benefit from fluids or taken owing to the frequent finding of respiratory thickened drinks. dysfunction and the high risk of aspiration in 67 In cases of slowed eating, encouraging patients with dysphagia. Every precaution should patients to take longer over meals is not neces­ be taken to avoid barium aspiration, which is likely to cause stress to the patient. The isosmolar sarily beneficial; for instance, slow eating can contrast agent Iotrolan, which has no significant induce earlier satiety, or some patients may be adverse effects even in the case of aspiration, is embarrassed that they are taking longer than recommended as an alternative to barium.68 The others. In cases of very slowed eating, therefore, penetration–aspiration scale (PAS) is used to judge a ‘little and often’ approach to meals should the degree of aspiration.69 be encouraged.45 Like VFSS, transnasal fiberoptic endoscopic evaluation of swallowing (FEES) enables direct Augmented feeding techniques observation of predeglutitive and postdeglutitive As dysphagia increases and oral food intake actions within the pharynx and larynx. No observation is possible during the intradeglutitive becomes impossible because of exceedingly phase as muscle contraction in the larynx and prolonged meal times or frequent choking, pharynx brings the mucous membrane into contact enteral nutrition should be considered. Careful with the endoscope.70 FEES is generally well discussion with the patient and the patient’s tolerated by patients, and real food can be eaten family is required to ensure timely provision during the examination. Complications such as of enteral nutrition, for reasons that we will epistaxis or vasovagal syncopes are rare, and discuss below. FEES is more portable than VFSS and can even be Fine-bore nasogastric tubes can be used for performed in bedridden patients. short-term feeding, although they can easily In conclusion, VFSS and FEES are complementary methods and both have advantages and become displaced and are very visible and disadvantages.71 The techniques are useful for uncomfortable for the patient. Furthermore, educating patients and carers, as they clearly reveal these tubes carry a high risk of ulceration and the process of swallowing and demonstrate the aspiration pneumonia, as well as of oropharyn­ risks involved in swallowing and the benefits of geal secretions.46 For medium-term and long- modifications to the feeding process.72 term purposes, enteral nutrition should be administered via percutaneous endoscopic gastro­ stomy (PEG) or percutaneous endoscopic jejuno­ stomy (PEJ).47 In patients who have a marked It is important that the option of enteral respiratory impairment (vital capacity <50%), a nutrition is offered early in the disease, as the radiologically inserted gastrostomy (RIG) can be risk associated with the insertion of a PEG or considered. The advantage of this last approach PEJ tube is relatively low at this stage. Enteral is that it does not generally require sedation feeding can initially be used as a supplement to during insertion,48 and it might, therefore, be ongoing oral intake. To minimize the risks of the safer than PEG under these circumstances.49 procedure, it has been suggested that the issue of

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enteral nutrition should be raised with the patient Surgical options soon after the onset of dysphagia, when the Palliative surgery to treat dysphagia in patients patient’s forced vital capacity is still over 50% of with ALS should be considered only in selected the predicted value.50,51 Although major compli­ cases, and even then with caution, as surgical cations such as peritonitis, necrotizing fasciitis, intervention and general anesthesia might hemorrhage and death have been described, contribute to neuronal death and a more PEG or PEJ tube insertion is usually safe, and only progressive disease course.60,61 The efficacy of mild complications such as local skin infections, cricopharyngeal myotomy in patients with ALS reflux, gastric ulcer, or mechanical problems who have disturbed opening of the upper esopha­ with the tube are usually observed.52 A study of geal sphincter remains controversial (the same 35 patients with ALS demonstrated an increasing holds true for botulinum toxin injection into BMI after insertion of a PEG tube, as well as a the sphincter). In patients who are unable to lower mortality rate in comparison with patients swallow their own secretions safely and therefore with ALS who did not receive a PEG.53 Other experience severe aspiration of saliva (perhaps studies have indicated improvements in quality of with subsequent pneumonia), a tracheostomy with life, as well as maintenance of or a gain in weight, insertion of a blocked tracheal cannula might be after introduction of enteral nutrition in patients necessary.62 It is important, however, to discuss with ALS.54 this procedure and its consequences (i.e. life- prolonging effect with ongoing worsening of Pharmacological interventions motor function) with patients and their relatives In patients with ALS who have drooling prob­ in detail before going ahead. lems, it is possible to reduce salivary flow with transdermal patches that dispense scopo­ THERAPY FOR LARYNGOSPASM lamine (hyoscine hydrobromide) over a period Rapid upright positioning of the trunk or fixa­ of 72 hours,55 or with atropine or drugs with tion of the arms to stabilize the body were found anticholinergic effects, such as amitriptyline to be sufficient maneuvers to shorten episodes or doxepin.44 Botulinum toxin injection into of laryngospasm in patients with ALS.36 Long- the parotid glands has been shown to be an term therapy for laryngospasm should consist efficient and cost-effective alternative to these primarily of lifestyle modifications, including approaches.56 Local radiation of the salivary appropriate spacing of meals and avoidance glands with 7–7.5 Gy is an experimental therapy of late-night meals. In addition, medications for drooling that is rarely used at present.57 that increase gastric acid secretion should be Many patients worry about choking to death and avoided. In general, avoidance of alterations find it reassuring to have a suction machine to of the upper aerodigestive tract, and gastro­ hand. Another major problem for patients who esophageal reflux treatment with prokinetic and drool is the lip excoriation that occurs with the antisecretory drugs, can reduce the frequency continuous rubbing of the mouth. Patients can of attacks.36,63 be instructed to dab away the saliva instead of rubbing the mouth, and to use petroleum jelly CONCLUSIONS or other lip protectors. Optimal care of patients with ALS requires Thick mucous secretions are frequently attention to laryngeal and pharyngeal symp­ reported by patients with ALS and can be a toms. With the assistance of a speech and sign of dehydration. Dehydration can occur language therapist, a physiotherapist, an occu­ because patients are unable to swallow fluids or pational therapist and a dietitian, the patient can are drooling large amounts of saliva that would overcome mild to moderate degrees of limited usually be swallowed and reabsorbed. Hydration activity. The adoption of special techniques is, therefore, an important first step in the treat­ can aid swallowing and communication. In ment of these patients. Liquefaction of thick advanced dysphagia, augmented feeding strat­ mucous secretions by use of N-acetylcysteine egies should be considered, and progressive might also be helpful. Manual support by a dysarthria requires the use of special communica­ physiotherapist and mechanical cough assisting tion devices. In summary, comprehensive devices (insufflators) are vital for patients who clinical care of patients with ALS requires a multi­ have thick mucous secretions combined with a disciplinary team to increase life expectancy and weak cough pressure.58,59 enhance quality of life.

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11 Lowe JS and Leigh N (2002) Disorders of movement KEY POINTS and system degenerations. In Greenfield´s ■ Bulbar symptoms such as dysarthria and Neuropathology, vol 2, edn 7, 325–430 (Eds Graham DI dysphagia are frequent features of amyotrophic and Lantos PL) London: Arnold lateral sclerosis (ALS) and can reduce life 12 Strand EA et al. (1996) Management of oral-pharyngeal expectancy and quality of life dysphagia symptoms in amyotrophic lateral sclerosis. Dysphagia 11: 129–139 ■ Dysarthria results from flaccid or spastic 13 Carpenter RJ III et al. (1978) The otolaryngologic presentation of amyotrophic lateral sclerosis. paresis of the musculature of the face, tongue, Otolaryngology 86: 479–484 lips, palate, pharynx and larynx 14 Chen A and Garrett CG (2005) Otolaryngologic presentations of amyotrophic lateral sclerosis. 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