Swallowing disturbances in the corticobasal syndrome.

OBJECTIVES: To determine the characteristics of swallowing and speech disturbances in patients with corticobasal syndrome (CBS) compared to healthy controls, and whether a subjective swallowing questionnaire, the NIH-Speech Pathology swallowing questionnaire (NIH-SQ), can predict swallowing impairment. METHODS: Twenty-four consecutive CBS patients underwent a swallowing assessment comprised of the NIH-SQ, ultrasound swallow study (US) and modified barium swallow (MBS) study. Healthy controls (n = 28) completed the NIH-SQ and the US. RESULTS: Ninety-six percent of the patients with CBS reported at least one complaint in the NIH-SQ, 59% had abnormal dry swallow duration and 10% abnormal wet swallow duration. Twenty-three patients with CBS had some abnormality on the MBS. The MBS category "piecemeal deglutition" (excessive lingual gestures causing multiple swallows required to clear a single bolus) was characteristic of CBS patients. No aspiration was detected. No NIH-SQ cutoff score or combination of subjective complaints predicted an abnormal MBS. Fifty-two percent of the patients had speech apraxia. CONCLUSIONS: Swallowing and speech disturbances are common in patients with CBS and differ from those previously reported in patients with PSP syndrome. Piecemeal deglutition and speech apraxia are characteristic features of our CBS patients. Although the NIH-SQ cannot predict the results of the more objective MBS in this population, it characterizes the patients' major subjective swallowing complaints.

Sex differences in prenatal oral-motor function and development.

The aim of this study was to investigate sex-related differences in the prenatal development of early oral, lingual, pharyngeal, and laryngeal motor activities. Sonographic images of oral-upper airway regions were observed in 85 healthy fetuses (43 males, 42 females; mean gestational age 24wks 3d [SD 0.69]; range 15-38wks). Biometric data on morphologic development and associated motor patterns were compared across second and third trimesters. Results showed that while males and females demonstrated statistically similar patterns of general physical growth (p>0.05), significant differences in development of specific lingual and pharyngeal structures were present (p<0.05). Significant differences were found for laryngeal and pharyngeal motor activity, and oral-lingual movements (p<0.05). Complex oral-motor and upper airway skills emerged earlier in females, suggesting a sex-specific trajectory of motor development. It was concluded that differential patterns of prenatal motor development may be important in defining sex-specific indices of oral skill maturation.

Patterns of care for dysphagic patients with degenerative neurological diseases.

Several of the most common neurodegenerative conditions associated with dysphagia are Parkinsons's disease, progressive supranuclear palsy, postpolio syndrome, and amyotrophic lateral sclerosis. The best clinical practices for treating dysphagic patients with these common conditions are discussed in relation to medication, surgery, diagnosis, and clinical management. Best practice patterns are explained as they relate to the following concepts of care, including comprehension and cognition; interaction with caregivers; dependence-independence during therapy; matching treatment plans to changes in physiology; and establishing realistic expectations including consideration of personal, cultural, and family needs for continuance or termination of care.

Motor strategy in rapid sequential swallowing: new insights.

This study examined the physiological properties and movement strategies of normal, rapid sequential swallowing during simultaneous videofluoroscopy (VFS) and submental surface electromyography (EMG). Ten subjects performed discrete (5 and 15 cc) and sequential (150 cc in tilted and upright head postures) swallowing tasks. Analyses included VFS event timing, movement/bolus passage characteristics, EMG amplitude waveforms, and peak and offset EMG amplitudes. Results revealed that sequential swallows were significantly shorter than discrete swallows in several VFS event durations, but significantly longer in pharyngeal transit and stage transition times. The hyolaryngeal system exhibited a cyclical "rise and partial fall" movement pattern during sequential swallows on VFS, corresponding to a repetitive "activation and partial deactivation" characteristic on EMG. Greater peak EMG amplitude for sequential than discrete swallows was found in 6/10 subjects. Pharyngeal bolus merging, preparatory laryngeal gestures, and penetration without aspiration were also observed in some subjects on VFS. Intersubject differences were significant in timing measures and EMG amplitude. Our findings of individual variability and subject-specific strategies for task accommodation support the notion of built-in plasticity in the deglutitive motor complex.

Effects of physostigmine on swallowing and oral motor functions in patients with progressive supranuclear palsy: A pilot study.

The purpose of this pilot study was to investigate whether cholinergic stimulation reduces swallowing and oral motor disturbances in patients with progressive supranuclear palsy (PSP). A controlled, double-blind crossover trial of physostigmine, a centrally active cholinesterase inhibitor, and placebo was conducted. Patients were randomized to a 10-day crossover placebo-controlled double-blind trial of physostigmine at their previously determined best dose administered orally every 2 hr, six times per day. Patients were evaluated with ultrasound imaging of the oropharynx and an oral motor examination at baseline and during the third or fourth days of each study phase (placebo and drug). Under the double-blind placebo-controlled conditions, patients showed no statistically significant improvement in oral motor functions or swallow durations. Because patients with PSP have increased sensitivity to cholinergic blockade compared with control subjects, studies with newer, more potent cholinergic stimulating agents need further exploration. Suggestions for future research include the evaluation of newer direct cholinergic agonists in the treatment of the less-impaired PSP patients who may have a greater number of cholinergic neurons preserved and the evaluation of combined therapies.

Current evaluation and treatment of patients with swallowing disorders.

To determine the varied causes of oropharyngeal dysphagia and their respective pathophysiology, a working understanding of the normal anatomy and function of the highly integrated mechanism of swallowing is outlined. This information is presented as the basis for a reasoned and detailed approach to the history, physical examination, and endoscopic evaluation of normal and altered oropharyngeal swallowing. The management of swallowing disorders depends on the nature and magnitude of the responsible clinical condition. Conservative and surgical approaches are discussed. These modalities and their indications are described in detail.

A pilot study: use of fludarabine for refractory dermatomyositis and polymyositis, and examination of endpoint measures.

OBJECTIVE: To study the effects of the adenine analog, fludarabine, on patients with refractory dermatomyositis and polymyositis, and to assess variables used in following myositis patients during medical intervention. METHODS: Patients whose myositis was not controlled by prednisone and at least one other immunosuppressive medication were entered into a pilot study during which they received 6 monthly cycles of intravenous fludarabine. Patients were assessed at baseline, every other month, and at month 7 for primary outcome measures of strength and function. Other measurements including peripheral blood cell subsets, muscle enzymes, and various assessments of disease activity were followed monthly during the fludarabine infusion period and for up to 6 months post therapy. RESULTS: Of 16 patients who entered the study, 4 patients were classified as improved, and 7 patients were classified as unchanged. Five patients who withdrew before month 7 were classified as treatment failures. Fludarabine caused a significant and prolonged lymphopenia without an increase in infectious complications over that seen with other immunosuppressive agents used for myositis. A sudden death of one patient at the end of the study was not thought to be drug related. Variables followed during the study emphasized the distinction between patient functional improvement and disease remission. CONCLUSION: A subset of patients with refractory myositis may benefit from fludarabine therapy and controlled trials are indicated. Refinement and validation of variables useful for following myositis patients await larger studies.

Characterizing swallowing abnormalities in progressive supranuclear palsy.

The dysphagia that occurs as an early sign of progressive supranuclear palsy (PSP), and which may predispose patients to aspiration pneumonia, has never been fully characterized. We evaluated 27 patients (mean +/- SEM: age, 64.9 +/- 1 years; symptom duration, 52 +/- 5 months) who met the clinical National Institute of Neurological Disorders and Stroke and Society for PSP (NINDS-SPSP) criteria for possible or probable PSP, with a swallowing questionnaire, an oral motor and speech examination, and either a modified barium swallow or ultrasound studies. Twenty-eight age- and sex-matched healthy controls (age, 65.6 +/- 1.5 years) were also evaluated with the questionnaire, oral examination, and the ultrasound study. We used ANOVA statistics to evaluate differences between groups; nonparametric correlations to assess associations between swallowing and motor and cognitive abnormalities; and logistic regression analysis to determine if the items of the questionnaire or oral examination predicted ultrasound or modified barium swallow abnormalities. While PSP patients had at least one complaint on the swallowing questionnaire (mean, 6.6), healthy controls had fewer and less relevant complaints (0.3). Patients with moderate-to-severe cognitive disabilities had significantly more complaints of dysphagia than those with mild or no impairment. PSP patients' oral motor skills and speech were mildly impaired but significantly different from those of controls. In the ultrasound studies, PSP patients had significantly fewer continuous swallows and required a longer duration to complete their swallows than did healthy controls. They also had mild-to-moderate abnormalities in the modified barium swallow study. The swallowing questionnaire, oral motor examination, and speech production examination accurately predicted the abnormalities detected with the swallowing studies. While 75% of patients had abnormal speech, all but one had abnormal swallowing studies. Thus, although dysphagia is associated with dysarthria, the two conditions are not always paired in the same patient. Our results suggest that the swallowing questionnaire and oral motor examination are an easy and cost-effective method to predict the swallowing disturbances in PSP.

Treatment of inclusion-body myositis with IVIg: a double-blind, placebo-controlled study.

We randomized 19 patients with inclusion-body myositis (IBM) to a double-blind, placebo-controlled, crossover study using monthly infusions of 2 g/kg intravenous immunoglobulin (IVIg) or placebo for 3 months. Patients crossed over to the alternate treatment after a washout period. We evaluated responses at baseline and at the end of each treatment period using expanded (0-10) MRC scales, the Maximum Voluntary Isometric Contraction (MVIC) method, symptom and disability scores, and quantitative swallowing studies. We calculated the differences in scores between IVIg and placebo from baseline to end of treatment. Of the 19 patients, 9 (mean age, 61.2 years; mean disease duration, 5.6 years) were randomized to IVIg and 10 (mean age, 66.1 years; mean disease duration, 7.4 years) to placebo. During IVIg the patients gained a mean of 4.2 (-16 to +39.8) MRC points, and during placebo lost 2.7 (-10 to +8) points (p < 0.1). These gains were not significant. Similar results were obtained with the MRC and MVIC scores when the patients crossed to the alternate treatment. Six patients had a functionally important improvement by more than 10 MRC points that declined when crossed over to placebo. Limb-by-limb analysis demonstrated that during IVIg the muscle strength in 39% of the lower extremity limbs significantly increased compared with placebo (p < 0.05), while a simultaneous decrease in 28% of other limbs was detected. The clinical importance of these minor gains is unclear. The duration of swallowing functions measured in seconds with ultrasound improved statistically in the IVIg-randomized patients (p < 0.05) compared with placebo. Although the study did not establish efficacy of IVIg, possibly because of the small sample size, the drug induced functionally important improvement in 6 (28%) of the 19 patients. Whether the modest gains noted in certain muscle groups justify the high cost of trying IVIg in IBM patients at a given stage of the disease remains unclear.

Evaluation and treatment of speech and swallowing disorders associated with myopathies.

Dysphagia, or disordered swallowing, can be demonstrated at any time over the course of many myopathies. Ability to swallow may be impaired because of weakness, inflammation, or dysfunction of the oropharyngeal, laryngeal, and esophageal musculature. Dysphagia may occur during the progression of disease regardless of whether the patient is properly treated. The presentation of signs of dysphagia can vary among patients because of differing patterns of weakness or incoordination of the facial muscles, lips, tongue, palate, pharyngeal constrictors, or smooth and striated muscles of the esophagus. Although the literature has focused on problems in the esophagus, scant attention has been paid to the oropharynx, which is often equally affected. Studies suggest that surgical myotomy and botulinum toxin injection may provide benefits for some patients with esophageal dysfunction. Although the condition is pervasive, there is little information on the incidence of dysphagia in muscular disorders. Because a major complication of dysphagia is aspiration, any sign of swallowing impairment demands medical attention and treatment.

Dysphagia and post-polio syndrome: past, present, and future.

Swallowing difficulties, oral motor weakness, and oral pharyngeal paralysis have been reported in the historical literature on polio and post-polio. Attention to this finding was minimal in the original epidemics as many persons with bulbar polio who may have been dysphagic did not survive. Swallowing was often supplemented by artificial feeding for those who were placed in respirators. Although swallowing difficulty was often present in the acute stages of the initial attack, it usually subsided or disappeared after several weeks. In cases in which bulbar polio was diagnosed, swallowing impairment may have lingered, but most survivors learned to produce compensatory maneuvers to aid them to swallow and did not complain of difficulty. In the recent decades of the 20th century, many persons who had believed themselves fully recovered from the insult of polio began to experience new symptoms; among them were new or heightened complaints of swallowing difficulty. Studies of these patients with PPS have revealed that dysphagia is not only present but may be progressive, and suggest that there is a slowly progressive deterioration of the bulbar neurons of PPS patients underlying the finding of new or exacerbated swallowing signs. Patients with PPS should be referred to speech-language pathologists for evaluation of voice, phonatory-respiratory, and swallowing function and initiation of appropriate remediation strategies to avoid the potential negative consequences of dysphagia.

Evaluation of normal and abnormal hyoid bone movement during swallowing by use of ultrasound duplex-Doppler imaging.

We developed a new method to analyze normal and abnormal movements of the hyoid muscular region as an indicator of hyoid bone motion during swallowing using ultrasound duplex-Doppler imaging. Hyoid bone motion can be monitored by studying the Doppler shift spectra and B-mode images produced by ultrasound duplex imaging of the hyoid region muscular attachments. We can accurately determine swallowing duration and trajectory of hyoid bone movement. This procedure can assist in discriminating between normal and abnormal movements of the hyoid bone and the surrounding muscles during swallowing. This method appears to be a highly consistent measure. We suggest that Doppler spectrum analysis can be used for defining hyoid position and displaying accurate movement, which may be useful in the diagnosis of swallowing disorders.

Practical guidelines for the assessment and treatment of selective mutism.

OBJECTIVE: To provide practical guidelines for the assessment and treatment of children with selective mutism, in light of the recent hypothesis that selective mutism might be best conceptualized as a childhood anxiety disorder. METHOD: An extensive literature review was completed on the phenomenology, evaluation, and treatment of children with selective mutism. Additional recommendations were based on clinical experience from the authors' selective mutism clinic. RESULTS: No systematic studies of the phenomenology of children with selective mutism were found. Reports described diverse and primarily noncontrolled treatment approaches with minimal follow-up information. Assessment and treatment options for selective mutism are presented, based on new hypotheses that focus on the anxiety component of this disorder. Ongoing research suggests a role for behavior modification and pharmacotherapy similar to the approaches used for adults with social phobia. CONCLUSION: Selectively mute children deserve a comprehensive evaluation to identify primary and comorbid problems that might require treatment. A school-based multidisciplinary individualized treatment plan is recommended, involving the combined effort of teachers, clinicians, and parents with home- and clinic-based interventions (individual and family psychotherapy, pharmacotherapy) as required.

An image processing scheme to quantitatively extract and validate hyoid bone motion based on real-time ultrasound recordings of swallowing.

An image processing technique with associated hardware was developed to quantitatively extract hyoid bone motion from realtime submandibular ultrasound images recorded during the swallowing act. Videofluorographic imaging, the "gold standard" of swallowing studies, was recorded simultaneously and synchronized to the ultrasound. Hyoid position obtained from the ultrasound was validated based on the videofluorography using personal computer-based image processing methods.

Remediation challenges in treating dysphagia post head/neck cancer. A problem-oriented approach.

A problem-oriented approach to dysphagia treatment was developed for a post head/neck surgery patient with a hematologic condition. Treatment was graded so that a hierarchical approach was used for all problems. Passive and resistive exercises preceded active exercise, and sensory stimulation preceded motion tasks. After 3 mo of treatment using an oral sensory motor stimulation paradigm and graded series of lip and tongue strengthening and motion exercises, swallowing had improved so that total nutritional intake was by mouth and weight was restored to normal. He was able to resume an active social life and to engage in vocational activities and hobbies.