Swallowing abnormalities and their response to treatment in Parkinson's disease

We investigated swallowing abnormalities in patients with Parkinson's disease, the relationship between these abnormalities and general parkinsonian signs, as well as the response to therapy. Twenty patients and 13 controls were evaluated with clinical rating scales and modified barium swallows before and after oral levodopa (in combination with carbidopa). Fifteen patients, but only 1 control, had abnormal swallows (chi 2 = 11.722, df = 1, p less than 0.001). Abnormalities included disturbances of oral and pharyngeal phases of swallowing. Patients without dysphagia frequently had abnormal swallows, including silent aspiration. Seven patients had improved swallowing after levodopa, whereas 1 worsened. Improvement in general parkinsonian signs was not a reliable indicator of improved swallowing.     

Electrophysiological evaluation of oropharyngeal swallowing in myotonic dystrophy

OBJECTIVE: Oropharyngeal dysphagia is a common feature of patients with myotonic dystrophy and is not usually perceived due to their emotional deficits and lack of interest. The aim was to show the existence and frequency of subclinical electrophysiological abnormalities in oropharyngeal swallowing and to clarify the mechanisms of dysphagia in myotonic dystrophy. METHODS: Eighteen patients with myotonic dystrophy were examined for oropharyngeal phase of swallowing by clinical and electrophysiological methods. Ten patients had dysphagia whereas 11 patients had signs and symptoms reflecting CNS involvement. Four patients with myotonia congenita and 30 healthy volunteers served as controls. Laryngeal movements were detected by means of a piezoelectric sensor. EMG activities of the submental muscle (SM-EMG) and needle EMG of the cricopharyngeal muscle of the upper eosophageal sphincter (CP-EMG) were also recorded during swallowing. RESULTS: In about 70% of the patients with myotonic dystrophy, the existence of oropharyngeal dysphagia was indicated objectively by means of the technique of "dysphagia limit" and by clinical evaluation. Duration of the swallowing reflex as defined by the laryngeal relocation time (0-2 time interval) and submental muscle excitation as a part of the swallowing reflex (A-C interval) were significantly prolonged in patients with myotonic dystrophy, especially in dysphagic patients. Triggering time of the swallowing reflex (A-0 interval) also showed significant prolongation, especially in the patients having both dysphagia and CNS involvement. During swallowing, CP muscle activity was abnormal in 40% of the patients with myotonic dystrophy. CONCLUSION: Both myopathic weakness and myotonia encountered in oropharyngeal muscles play an important part in the oral and the pharyngeal phases of swallowing dysfunction in myotonic dystrophy. It was also suggested that CNS involvement might contribute to the delay of the triggering of the swallowing reflex and some abnormal EMG findings in the CP sphincter, resulting in oropharyngeal dysphagia in myotonic dystrophy.     

脑卒中患者吞咽障碍及康复效果影像学研究

目的探讨电视X线透视吞咽功能检查（videofluoroscopic swallowing study,VFSS）在脑卒中患者吞咽功能评估中的应用价值,观察脑卒中后吞咽障碍发生情况,康复前后患者吞咽功能变化.方法脑卒中患者70例及健康成人80名分别作为研究组与对照组均进行VFSS,比较两组误吸等VFSS异常征象的发生情况.对发现误吸患者进行吞咽功能康复训练4周,训练后复查VFSS,比较训练前后吞咽异常的发生情况变化.结果对照组渗透或误吸、口腔滞留、咽腔滞留的发生率为5.0%,13.4%,25.3%.主要为轻度渗透及口咽腔滞留.研究组渗透或误吸、口腔滞留、咽腔滞留发生率为45.0%,46.5%,48.9%,主要为重度渗透或误吸,中重度口咽腔滞留.其中隐匿性误吸为10次（占总误吸的24.4%）.误吸患者康复治疗后渗透或误吸发生率较康复治疗前减少（P＜0.05）.结论脑卒中后吞咽障碍在康复期仍较为常见,可表现为多种影像学异常.VFSS可确切诊断吞咽异常,进行针对性康复训练,使患者吞咽功能提高.     

Electrophysiological evaluation of pharyngeal phase of swallowing in patients with Parkinson's disease.

We studied the various physiological aspects of oropharyngeal swallowing in Parkinson's disease (PD). Fifty-eight patients with PD were investigated by clinical and electrophysiological methods that measured the oropharyngeal phase of swallowing. All patients except 1 had mild to moderate degree of disability score. Dysphagia was demonstrated in 53% of all patients in whom the test of dysphagia limit was abnormal. All PD patients with or without dysphagia displayed the following abnormalities: (1) the triggering of the swallowing reflex was prolonged probably due to inadequate bolus control in the mouth and tongue and/or a specific delay in the execution of the swallowing reflex; (2) the duration of the pharyngeal reflex time was extremely prolonged due to slowness of the sequential muscle movements, especially those of the suprahyoid-submental muscles; (3) cricopharyngeal muscle of the upper oesophageal sphincter was found to be electrophysiologically normal; and (4) the electrophysiological phenomena in PD patients could not be strongly correlated with the degree of the disability and clinical score of the PD. It was concluded that various motor disorders of PD have considerable influence on oropharyngeal swallowing: hypokinesia, reduced rate of spontaneous swallowing, and the slowness of segmented but coordinated sequential movements rather than any abnormalities in the central pattern generator of the bulbar center. Some compensatory mechanisms in the course of PD may explain the benign nature of swallowing disorder until the terminal stage of the disease. Similarly, the swallowing problems of PD are not only related with the dopamine deficiency; some other nondopaminergic mechanisms may also be involved.     

Dysphagia in multiple sclerosis

Objectives: (1) To determine the prevalence of swallowing problems in MS patients and its relation to the overall disability. (2) To define the most frequent symptoms suggestive of dysphagia. (3) To describe the abnormalities on manofluoroscopy (MFS). Methods: Three hundred and eight consecutive MS patients were asked whether they ever had swallowing problems. If so the questionnaire of the Johns Hopkins Swallowing Centre was applied to qualify the dysphagia. A MFS was performed in 30 patients with dysphagia covering the entire spectrum of MS. Overall disability was assessed using the Expanded Disability Status Scale (EDSS). Results: Seventy-three of our 309 patients had permanent dysphagia (24%). Another 5% had a history of transitory swallowing problems only. Permanent dysphagia started to be a problem in mildly impaired patients (EDSS 2–3). Prevalence increased together with rising disability to reach 65% in the most severely disabled subjects (EDSS 8–9). Two alarming symptoms of patients with swallowing problems, coughing or choking during the meal and a history of pneumonia were present in 59%, respectively, 12% of these patients. MFS showed deficiency of the oral phase in all patients, while only the patients with an EDSS higher than 7.5 showed abnormalities of the pharyngeal phase. Conclusions: Permanent dysphagia may already develop in mildly impaired MS patients but becomes a rather frequent finding in MS patients with moderate or severe disability. MFS is a sensitive and useful ancillary examination. Important qualitative changes of the pharyngeal phase on MFS are seen in patients with an EDSS higher than 7.5.     

Esophageal Motor Patterns During Episodes of Dysphagia for Solids

Although dysphagia for solids is a common complaint, the motor response to eating is not routinely studied during diagnostic manometry. Esophageal motility was examined in 47 patients with a history of dysphagia and 34 patients without dysphagia. In 22 of those with dysphagia, the symptoms were reproduced in the laboratory when the patients ate bread. These patients had a higher swallow frequency, reduced peristaltic swallow rate, and an increased rate of synchronous contractions compared to patients without dysphagia. In 22 of 53 separate episodes of dysphagia, a mixed pattern of esophageal motility was seen with peristaltic synchronous and nonconducted swallows (although peristaltic swallows were <50% of total swallows). In 26 of 53 episodes, dysphagia was associated with complete aperistalsis. Mean swallow frequency increased during episodes of dysphagia compared to asymptomatic periods. Swallow rate is an important determinant of esophageal motility during eating. Dysphagia is associated with either aperistalsis or markedly reduced peristaltic activity and may be produced, or exacerbated, by an increased swallow frequency. Our results indicate that assessment of motility patterns during eating is important in the examination of patients complaining of dysphagia.     

The importance of the reproducibility of oropharyngeal swallowing in amyotrophic lateral sclerosis. An electrophysiological study

Objective

To investigate electrophysiologically the reproducibility of oropharyngeal swallowing in patients with ALS.

Detecting dysphagia in inclusion body myositis

Dysphagia is an important yet inconsistently recognized symptom of inclusion body myositis (IBM). It can be disabling and potentially life-threatening. We studied the prevalence and symptom-sign correlation of dysphagia. Fifty-seven IBM patients were interviewed using a standard questionnaire for dysphagia and 43 of these underwent swallowing videofluoroscopy (VFS). Symptoms of dysphagia were present in 37 of 57 patients (65%). Nevertheless, only 17 of these patients (46%) had previously and spontaneously complained about swallowing to their physicians. Both symptoms of impaired propulsion (IP) (59%) and aspiration-related symptoms (52%) were frequently mentioned. Swallowing abnormalities on VFS were present in 34 of 43 patients (79%) with IP of the bolus in 77% of this group. The reported feeling of IP was confirmed by VFS in 92% of these patients. Dysphagia in IBM is common but underreported by the vast majority of patients if not specifically asked for. In practice, two questions reliably predict the presence of IP on VFS: ‘Does food get stuck in your throat’ and ‘Do you have to swallow repeatedly in order to get rid of food’. These questions are an appropriate means in selecting IBM patients for further investigation through VFS and eventual treatment.     

Persistent post-stroke dysphagia treated with cricopharyngeal myotomy

Post-stroke dysphagia is a common problem after stroke. About 8-13% patients have persistent dysphagia and are unable to return to pre-stroke diet even after 6 months of stroke. Use of percutaneous endoscopic gastrostomy (PEG) may be required in these patients, which may be psychologically unacceptable and impair the quality of life. In those with cricopharyngeal dysfunction leading on to refractory post-stroke dysphagia, cricopharyngeal myotomy and injection of botulinum toxin are the treatment options. We present a case of vertebrobasilar stroke who had persistent dysphagia due to cricopharyngeal dysfunction with good recovery of swallowing function following cricopharyngeal myotomy 1.5 years after the stroke.     

Sildenafil relieves symptoms and normalizes motility in patients with oesophageal spasm: a report of two cases

Oesophageal spasm presents with dysphagia and chest pain. Current treatments are limited by poor efficacy and side effects. Studies in health and oesophageal dysmotility show that sildenafil reduces peristaltic pressure and velocity; however the clinical efficacy and tolerability in symptomatic oesophageal spasm remains uncertain. We provided open-label sildenafil treatment to two patients with severe, treatment resistant symptoms associated with oesophageal spasm. The effects of sildenafil on oesophageal function and symptoms were documented by high resolution manometry (HRM). Patients were followed up to assess the efficacy of maintenance treatment with sildenafil b.i.d. HRM revealed focal and diffuse spasm in the smooth muscle oesophagus that were associated with symptoms in both cases, especially on swallowing solids. Lower oesophageal sphincter function was normal. A therapeutic trial of 25-50 mg sildenafil suppressed oesophageal contraction almost completely for water swallows; however effective, coordinated peristalsis returned with reduced frequency of spasm for solid swallows. Dysphagia and chest pain resolved during the therapeutic trial and efficacy was maintained on maintenance treatment with 25-50 mg sildenafil b.i.d. without troublesome side effects. This report shows that sildenafil can improve oesophageal function and relieve dysphagia and chest pain in patients with oesophageal spasm in whom other treatments have failed.     

Swallowing function in patients with spasmodic torticollis

We examined the oropharyngeal swallowing ability of 43 patients with spasmodic torticollis using a videofluoroscopic procedure. Twenty-two (51.2%) demonstrated objective evidence of swallowing abnormalities; 15 (34.9%) had subjective complaints. Delayed swallowing reflex and vallecular residue were more frequent (p less than 0.0046) than any other abnormality. The constellations of abnormalities were consistent with neurogenic, postural, and mixed neurogenic-postural types of dysphagia.     

Altered pharyngeal muscles in Parkinson disease

Dysphagia (impaired swallowing) is common in patients with Parkinson disease (PD) and is related to aspiration pneumonia, the primary cause of death in PD. Therapies that ameliorate the limb motor symptoms of PD are ineffective for dysphagia. This suggests that the pathophysiology of PD dysphagia may differ from that affecting limb muscles, but little is known about potential neuromuscular abnormalities in the swallowing muscles in PD. This study examined the fiber histochemistry of pharyngeal constrictor and cricopharyngeal sphincter muscles in postmortem specimens from 8 subjects with PD and 4 age-matched control subjects. Pharyngeal muscles in subjects with PD exhibited many atrophic fibers, fiber type grouping, and fast-to-slow myosin heavy chain transformation. These alterations indicate that the pharyngeal muscles experienced neural degeneration and regeneration over the course of PD. Notably, subjects with PD with dysphagia had a higher percentage of atrophic myofibers versus with those without dysphagia and controls. The fast-to-slow fiber-type transition is consistent with abnormalities in swallowing, slow movement of food, and increased tone in the cricopharyngeal sphincter in subjects with PD. The alterations in the pharyngeal muscles may play a pathogenic role in the development of dysphagia in subjects with PD.     

Reversible esophageal motor dysfunction in botulism

Two cases of botulism with autonomic and neuromuscular system involvement are presented. In both patients, dryness of the mouth and difficulties in swallowing were predominant symptoms. Esophageal manometry revealed a marked decrease in peristaltic amplitude, which was most pronounced in the upper third of the esophagus. These functional abnormalities returned to normal following recovery from the acute disease.     

Dysphagia in olivopontocerebellar atrophy

Clinical, radiological and manometric studies on thirteen patients with olivopontocerebellar atrophy were performed in order to investigate the characteristics of dysphagia. As a clinical study, a detailed history of dysphagia was taken to distinguish two types of dysphagia, that is to say swallowing disturbance in a narrow sense and passage disturbance. In the radiological study, each phase of swallowing was observed by X-rays with contrast medium (Dionosil). In the manometric study, intraluminal resting pressure in the esophagus and pressure of esophageal contraction after swallowing were measured. The results were as follows: Eight patients had the sensation of swallowing disturbance in a narrow sense and five patients has the feeling of passage disturbance. In X-ray studies four patients had pooling in piriformis sinus and six patients had slight dilatation of the lower esophagus. In manometric studies, six patients had low intraluminal resting pressure of the upper esophageal sphincter, but almost all patients had normal intraluminal resting pressure throughout the esophagus and in the lower esophageal sphincter. Two patients, who had suffered for five and seven years, had loss of both negative and positive wave in the upper esophageal sphincter after swallowing. Another three patients, who had suffered for two, six and seven years, respectively had loss of negative wave in the upper esophageal sphincter after swallowing. Regarding peristaltic wave, eight patients had low amplitude of the wave. Two patients, who had suffered for five and six years, had diphasic shape of the wave. One patient, who had suffered for nine years, had synchronous wave. Nine patients had loss of negative wave in the lower esophageal sphincter.(ABSTRACT TRUNCATED AT 250 WORDS)     

A timed test of swallowing capacity for neurological patients.

A timed test of swallowing capacity has been designed for use in patients with neurogenic dysphagia. Swallowing speed (ml/s) has been demonstrated to have high intra- and inter- rater and test- retest reliability, and to be essentially independent of flavour or temperature. "Guideline" normal values were established in individuals without a swallowing disorder: swallowing speed was less in females than males and declined in both groups with age. The validity of a swallowing speed less than 10 ml/s as an index of abnormal swallowing was tested by comparison with the complaint of abnormal swallowing in a group of 81 neurological patients. Swallowing speed had a sensitivity of 96% and specificity of 69%: some apparent false positive responses were found in patients with disordered swallowing, mainly due to multiple sclerosis. Using a standard questionnaire and examination a similar pattern of symptoms and signs were statistically associated with both the clinical complaint of abnormal swallowing and swallowing speed. It is concluded that swallowing speed is a reliable and valid index for assessing disordered swallowing in neurological patients and may be of value in monitoring response to therapy.     

Videofluorographic assessment of swallowing function in patients with Duchenne muscular dystrophy.

OBJECTIVE: To identify the characteristics of swallowing function in patients with Duchenne muscular dystrophy (DMD). METHODS: Swallowing function was evaluated using videofluorography (VF) in a cross-sectional observational study of 102 DMD patients (mean age 21.5 years) who had dysphagia or in whom dysphagia was suspected based on clinical signs. Reduced tongue movement, impaired bolus transport to the pharynx, decreased pharyngeal contraction, bolus delivery into the airway, and bolus residue at the epiglottic vallecula and at the piriform recess were qualitatively evaluated for test swallows of jelly and juice. During VF, the length of time of both the oral and pharyngeal phases of swallowing was measured in 59 patients. RESULTS: Patients started to show oral phase abnormalities in their mid-teens and pharyngeal phase abnormalities such as pharyngeal residue around age 20. Oral phase abnormalities was higher with juice than with jelly. Total oral/pharyngeal transit duration was longer with age, and total duration of hyoid maximum elevation was shorter with age. CONCLUSION: The weak positive correlation of total oral/pharyngeal transit duration and age was presumably due to gradual onset of functional abnormalities associated with deteriorated swallowing muscles starting in the teenage years. Reduced tongue movement and impaired bolus transport to the pharynx was more common in teenage DMD patients because they have limited tongue movements associated with structural abnormalities such as macroglossia and open bite. VF showed that the swallowing difficulties were more severe during the oral phase than in the pharyngeal phase in the teenage patients. The pharyngeal phase disorders such as pharyngeal residue and decreased pharyngeal contraction were seen more often in the patients in their 20s, presumably due to deterioration of swallowing muscles that becomes more apparent in the older age group.     

Dysphagia and nutritional status in multiple sclerosis

In this observational study of patients with multiple sclerosis (MS) admitted to a regional neurology centre we assessed the frequency of dysphagia (objectively defined), dysphagia related symptoms, bulbar signs and nutritional status. We studied 79 consecutive admissions with MS (24 at diagnostic admission and 55 more advanced cases admitted for treatment and/or rehabilitation): normative swallowing data were from 181 healthy controls. Swallowing symptoms and signs were semi-quantitatively measured and compared to healthy controls. Dysphagia was defined by a quantitative water test. Disability was determined by Kurtzke’s Expanded Disability Status Scale and Barthel’s index. Nutritional status was assessed by body mass index, estimated percentage body fat from skin fold thickness measurements at four sites, a global evaluation of nutrition, the presence of pressure sores and the pressure sore risk using the Waterlow score. Patients with MS were more likely to complain of abnormal swallowing, of coughing when eating, and of food ‘going down the wrong way’ than healthy controls (P < 0.005). These significantly associated symptoms had high specificity but relatively low sensitivity. 43% of patients had abnormal swallowing, almost half of whom did not complain of it: abnormal swallowing was associated with several factors including abnormal brainstem/cerebellar function, disability, vital capacity, and depression score. Those with abnormal swallowing had higher Waterlow scores (P < 0.001), but, overall, abnormal swallowing was not associated with a difference in nutritional indices or incidence of pressure sores. In summary, abnormal swallowing is common in MS although often not complained of. It is associated with disordered brainstem/cerebellar function, overall disability, depressed mood and low vital capacity. It was not associated with major nutritional failure or pressure sores in this study. Received: 16 June 1998 Received in revised form: 29 October 1998 Accepted: 19 January 1999     

Radiological evidence of subclinical dysphagia in motor neuron disease

Dysphagia in motor neuron disease (MND) may lead to dangerous complications such as cachexia and aspiration pneumonia. Functional evaluation of the oropharyngeal tract is crucial for identifying specific swallowing dysfunctions and planning appropriate rehabilitation. As part of a multidisciplinary study on the treatment of dysphagia in patients with neuromuscular diseases, 23 MND patients with different degrees of dysphagia underwent videoflouroscopy, videopharyngolaryngoscopy and pharyngo-oesophageal manometry. The results of the three instrumental investigations were analysed in order (1) to define the pattern of swallowing in MND patients complaining of dysphagia; (2) to evaluate whether subclinical abnormalities may be detected; and (3) to assess the role of videofluoroscopy, videopharyngolaryngoscopy and manometry in the evaluation of MND patients with deglutition problems. Correlations between the instrumental findings and clinical features (age of the patients, duration and severity of the disease, presence and degree of dysphagia) were also assessed. The results of our study showed that: (1) The oral phase of deglutition was compromised most often, followed by the pharyngeal phase. (2) In all patients without clinical evidence of dysphagia, subclinical videofluoroscopic alterations were present in a pattern similar to that found in the dysphagic group. (3) Videofluoroscopy was the most sensitive technique in identifying oropharyngeal alterations of swallowing. Impairment of the oral phase, abnormal pharyngo-oesophageal motility and incomplete relaxation of the upper oesophageal sphincter were the changes most sensitive in detecting dysphagia. Videofluoroscopy was also capable of detecting preclinical abnormalities in non-dysphagic patients who later developed dysphagia. Practical guidelines for the use of instrumental investigations in the assessment and management of dysphagia in MND patients are proposed. Received: 24 March 1997 Received in revised form: 11 November 1997 Accepted: 18 November 1997     

Oropharyngeal swallowing in craniocervical dystonia

OBJECTIVE: To clarify the pathophysiology of dysphagia by electrophysiological methods. METHOD: Electrophysiological methods related to oropharyngeal swallowing were used to investigate 25 patients with cervical dystonia and 25 age matched normal volunteers. RESULTS: Dysphagia was suspected in 36% of patients with cervical dystonia on the basis of clinical assessment. The incidence of dysphagia increased to 72% on electrophysiological evaluation of pharyngeal swallowing. Submental muscle electromyographic (EMG) and laryngeal relocation times were significantly prolonged and the triggering time to swallowing reflex was significantly delayed. Some abnormalities seen in cricopharyngeal sphincter muscle EMG indicated that the striated sphincter muscle is hyperreflexive in some patients. CONCLUSION: Neurogenic dysphagia was more prominent and longer lasting than mechanical dysphagia, which was transient and varied from patient to patient. Although these electrophysiological methods were not suitable for detecting anatomical changes during swallowing, as in videofluoroscopic studies, observations supported the neurogenic cause of dysphagia in patients with any kind of cervical dystonia.     

Endoscopic characteristics and levodopa responsiveness of swallowing function in progressive supranuclear palsy

Dysphagia is a frequent and early symptom in progressive supranuclear palsy (PSP) predisposing patients to aspiration pneumonia. Fiberoptic endoscopic evaluation of swallowing (FEES®) has emerged as a valuable apparative tool for objective evaluation of neurogenic dysphagia. This is the first study using FEES® to investigate the nature of swallowing impairment in PSP. Eighteen consecutive PSP patients (mean age 69.7 ± 9.0 years) were included. The salient findings of FEES® in PSP patients were compared with those of 15 patients with Parkinson's disease (PD). In 7 PSP patients, a standardized FEES® protocol was performed to explore levodopa (L ‐dopa) responsiveness of dysphagia. Most frequent abnormalities detected by FEES® were bolus leakage, delayed swallowing reflex, and residues in valleculae and piriformes. Aspiration events with at least one food consistency occurred in nearly 30% of PSP patients. Significant pharyngeal saliva pooling was observed in 4 PSP patients. We found no difference of salient endoscopic findings between PSP and PD patients. Endoscopic dysphagia severity in PSP correlated positively with disease duration, clinical disability, and cognitive impairment. No correlation was found with dysarthria severity. In early PSP patients, swallowing dysfunction was solely characterized by liquid leakage with the risk of predeglutitive aspiration during the oral phase of swallowing. Two PSP patients showed relevant improvement of swallowing function after L ‐dopa challenge. Chin tuck—maneuver, hard swallow, and modification of food consistency were identified as the most effective therapeutic interventions. In conclusion, FEES® assessment can deliver important findings for the diagnosis and refined therapy of dysphagia in PSP patients. © 2010 Movement Disorder Society