Brief assessment of motor function: content validity and reliability of the oral motor scales.

OBJECTIVE: The Brief Assessment of Motor Function consists of five 0- to 10-point hierarchical scales designed for rapid assessment of gross, fine, and oral motor skills. We describe the development and evaluation of the two Brief Assessment of Motor Function Oral Motor Scales: Oral Motor Articulation and Oral Motor Deglutition. DESIGN: This validation study employed an expert panel of 28 speech-language pathologists, who rated the Brief Assessment of Motor Function Oral Motor Scales items on a scale from 1 to 4 (disagree to agree) to establish content validity. For reliability, oral motor performances of 18 participants (6 mos-20 yrs) were videotaped to represent a wide range of articulation and deglutition capabilities. Four speech-language pathologists, and 1 undergraduate and 10 graduate speech-language pathology students rated the participants' taped samples using the Brief Assessment of Motor Function Oral Motor Scales. RESULTS: All items on the content validity questionnaire had average agreement scores that exceeded criteria, except two, which were not clearly worded; these were clarified. Interrater and intrarater reliability values were 0.997 and 0.986 for the Oral Motor Articulation Scale and 0.977 and 0.997 for the Oral Motor Deglutition Scale. CONCLUSIONS: Expert feedback and reliability procedures suggest that the Brief Assessment of Motor Function Oral Motor Articulation and Deglutition Scales represent the content that they are designed to assess and are reliable for rapid assessment of oral motor skills.

Oropharyngeal dysphagia assessment and treatment efficacy: setting the record straight (response to Campbell-Taylor).

In September 2008, an article was published in the Journal of the American Medical Directors Association criticizing current dysphagia assessment and management practices performed by speech-language pathologists in Long-Term Care (LTC) settings. In the same issue, an editorial invited dialogue on the points raised by Campbell-Taylor. We are responding to this call for dialogue. We find Campbell-Taylor's interpretation of the literature to be incomplete and one-sided, leading to misleading and pessimistic conclusions. We offer a complementary perspective to balance this discussion on the 4 specific questions raised: (1) Is the use of videofluoroscopy warranted for evaluating dysphagia in the LTC population? (2) How effective are thickened liquids and other interventions for preventing aspiration and do they contribute to reduction of morbidity? (3) Can aspiration be prevented and is its prevention important? and (4) Is there sufficient evidence to justify dysphagia intervention by speech language pathologists?

Intravenous immune globulin in hereditary inclusion body myopathy: a pilot study.

BACKGROUND: Hereditary Inclusion Body Myopathy (HIBM) is an autosomal recessive, adult onset, non-inflammatory neuromuscular disorder with no effective treatment. The causative gene, GNE, codes for UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase, which catalyzes the first two reactions in the synthesis of sialic acid. Reduced sialylation of muscle glycoproteins, such as alpha-dystroglycan and neural cell adhesion molecule (NCAM), has been reported in HIBM. METHODS: We treated 4 HIBM patients with intravenous immune globulin (IVIG), in order to provide sialic acid, because IgG contains 8 micromol of sialic acid/g. IVIG was infused as a loading dose of 1 g/kg on two consecutive days followed by 3 doses of 400 mg/kg at weekly intervals. RESULTS: For all four patients, mean quadriceps strength improved from 19.0 kg at baseline to 23.2 kg (+22%) directly after IVIG loading to 25.6 kg (+35%) at the end of the study. Mean shoulder strength improved from 4.1 kg at baseline to 5.9 kg (+44%) directly after IVIG loading to 6.0 kg (+46%) at the end of the study. The composite improvement for 8 other muscle groups was 5% after the initial loading and 19% by the end of the study. Esophageal motility and lingual strength improved in the patients with abnormal barium swallows. Objective measures of functional improvement gave variable results, but the patients experienced improvements in daily activities that they considered clinically significant. Immunohistochemical staining and immunoblotting of muscle biopsies for alpha-dystroglycan and NCAM did not provide consistent evidence for increased sialylation after IVIG treatment. Side effects were limited to transient headaches and vomiting. CONCLUSION: The mild benefits in muscle strength experienced by HIBM patients after IVIG treatment may be related to the provision of sialic acid supplied by IVIG. Other sources of sialic acid are being explored as treatment options for HIBM.

Effects of surface electrical stimulation both at rest and during swallowing in chronic pharyngeal Dysphagia.

We tested two hypotheses using surface electrical stimulation in chronic pharyngeal dysphagia: that stimulation (1) lowered the hyoid bone and/or larynx when applied at rest, and (2) increased aspiration, penetration, or pharyngeal pooling during swallowing. Bipolar surface electrodes were placed on the skin overlying the submandibular and laryngeal regions. Maximum tolerated levels of stimulation were applied while patients held their mouth closed at rest. Videofluoroscopic recordings were used to measure hyoid movements in the superior-inferior and anterior-posterior dimensions and the subglottic air column position while stimulation was on or off. Patients swallowed 5 ml liquid when stimulation was off, at low sensory stimulation levels, and at maximum tolerated levels (motor). Speech pathologists, blinded to condition, tallied the frequency of aspiration, penetration, pooling, and esophageal entry from videofluorographic recordings of swallows. Only significant (p = 0.0175) hyoid depression occurred during stimulation at rest. Aspiration and pooling were significantly reduced only with low sensory threshold levels of stimulation (p = 0.025) and not during maximum levels of surface electrical stimulation. Those patients who had reduced aspiration and penetration during swallowing with stimulation had greater hyoid depression during stimulation at rest (p = 0.006). Stimulation may have acted to resist patients' hyoid elevation during swallowing.

Manofluorographic evaluation of swallowing in amyotrophic lateral sclerosis and its relationship with clinical evaluation of swallowing.

The aim of this cross-sectional study was, first, to identify swallowing dysfunctions in an ALS population of 40 consecutive patients through combined videofluoroscopy and manometry. Secondly, these objective swallowing data were correlated with the functional feeding status as reported by the patient or family member. Videofluoroscopic evaluation showed dysfunctions in the oral phase of swallowing, pharyngeal initiation and pharyngeal transport. In addition, manometric data revealed low tongue driving forces and pharyngeal contraction amplitudes but normal relaxation of the upper oesophageal sphincter (UES). Aspiration was noted in a not negligible number of 9/40 patients. These objective data were then correlated with the clinical swallowing and feeding status, assessed by means of the ALS Swallowing Severity Scale. Patients receiving scores of 6 or lower on the ALSSSS, report dietary consistency changes but are considered 'safe oral feeders'. Nevertheless, our data revealed that these patients showed significant aspiration during videofluoroscopy. Although not every patient with ALS should be referred routinely for radiographic evaluation of swallowing, our findings suggest referral for a radiological examination as soon as the ALSSSS drops to a score of 6 or lower, to evaluate the presence of (silent) aspiration.

The effect of surface electrical stimulation on hyolaryngeal movement in normal individuals at rest and during swallowing.

Surface electrical stimulation is currently used in therapy for swallowing problems, although little is known about its physiological effects on neck muscles or swallowing. Previously, when one surface electrode placement was used in dysphagic patients at rest, it lowered the hyolaryngeal complex. Here we examined the effects of nine other placements in normal volunteers to determine 1) whether movements induced by surface stimulation using other placements differ, and 2) whether lowering the hyolaryngeal complex by surface electrical stimulation interfered with swallowing in healthy adults. Ten bipolar surface electrode placements overlying the submental and laryngeal regions were tested. Maximum tolerated stimulation levels were applied at rest while participants held their mouths closed. Videofluoroscopic recordings were used to measure hyoid bone and subglottic air column (laryngeal) movements from resting position and while swallowing 5 ml of liquid barium, with and without stimulation. Videofluoroscopic recordings of swallows were rated blind to condition using the National Institutes of Health-Swallowing Safety Scale. Significant (P < 0.0001) laryngeal and hyoid descent occurred with stimulation at rest. During swallowing, significant (P

Swallowing dysfunction in 101 patients with nephropathic cystinosis: benefit of long-term cysteamine therapy.

Nephropathic cystinosis is a rare, autosomal recessive lysosomal storage disorder caused by mutations in the CTNS gene that codes for a cystine transporter in the lysosomal membrane. Affected patients store 50-100 times the normal amounts of cystine in their cells, and suffer renal tubular and glomerular disease, growth retardation, photophobia, and other systemic complications, including a myopathy and swallowing dysfunction. Using videofluoroscopy and ultrasound examinations, we assessed the swallowing function of 101 patients with nephropathic cystinosis on their most recent admission to the National Institutes of Health Clinical Center between 1987 and 2004. These patients ranged in age from 6 to 45 years; more than half had significant complaints of swallowing difficulty. On examination of barium swallow, the oral, pharyngeal, and esophageal phases of swallowing were abnormal in 24%, 51%, and 73% of patients, respectively. The frequency of dysfunction increased with age for each phase of swallowing. Both the Swallowing Severity Score (a measure of dysfunction on barium swallow) and the Oral Muscle Composite Score (a reflection of vocal strength, oral-facial movement, and tongue and lip function) increased (that is, worsened) with the number of years that a patient was not receiving treatment with cysteamine, the cystine-depleting agent of choice in cystinosis. The severity scores decreased with the number of years on cysteamine therapy. The Swallowing Severity Score varied directly with the severity of muscle disease, but was not correlated with the presence or absence of the 57-kb CTNS deletion that commonly occurs in nephropathic cystinosis patients. We conclude that swallowing dysfunction in cystinosis presents a risk of fatal aspiration, correlates with the presence of muscle atrophy, and, based on cross-sectional data, increases in frequency with age and number of years without cysteamine treatment. Cystine-depleting therapy with cysteamine should be considered the treatment of choice for both pre- and posttransplant cystinosis patients.

Ultrasound imaging distinguishes between normal and weak muscle.

OBJECTIVE: To determine whether real-time ultrasound imaging can provide quantitative data that distinguish pathologic from healthy muscle and that correlate with strength measures. DESIGN: Nonrandomized matched-pair, repeated-measures design. SETTING: Ultrasound imaging laboratory, rehabilitation medicine department, government research hospital. PARTICIPANTS: Nine patients with stable active or inactive myositis, stratified into 3 groups based on their 10-point manual muscle test (MMT) scores, and 9 age- and gender-matched controls. INTERVENTIONS: Maximal isometric contraction of the rectus femoris muscle in 2 knee-flexion positions (60 degrees, 90 degrees ) during simultaneous ultrasound imaging and muscle force dynamometry. MAIN OUTCOME MEASURES: Changes of the rectus femoris muscle in horizontal (X) and vertical (Y) diameters between relaxed and contracted states, and muscle force measurements. RESULTS: The X diameters decreased and the Y diameters increased during isometric contraction in all participants. For each group, average changes in cross-sectional diameters were consistently higher in controls than in patients. Patients with MMT less than 8 differed significantly from controls in both X and Y dimensions. A moderately strong correlation was found between muscle force and the Y diameter during contraction at 60 degrees (r =.78) and 90 degrees (r =.67) knee-flexion angles. CONCLUSIONS: Ultrasonography provided a quantitative measure of change between relaxed and contracted state of muscle, which correlated with muscle force. Ultrasound identified significant differences in cross-sectional diameters between the myopathic and normal muscles sampled and may be useful for measuring muscle response to drug and exercise therapy.

Emergence of oropharyngeal, laryngeal and swallowing activity in the developing fetal upper aerodigestive tract: an ultrasound evaluation.

The developing fetal upper aerodigestive system provides the structural support for respiratory and ingestive functions necessary to sustain life at birth. This study investigated prenatal development of upper aerodigestive anatomy and the association of emerging functions as predictors of postnatal feeding skills. Biometric measures of oral, lingual, pharyngeal and laryngeal structures were obtained in fetuses 15-38 weeks gestational age using a four-plane sonographic technique. Accompanying ingestive behaviors were tallied across development. The data from 62 healthy controls were compared to seven cases at risk for postnatal feeding and swallowing dysfunction (Type II Arnold Chiari Malformation, trisomy 18, polyhydramnios, intrauterine growth restriction, Brachmann-de Lange Syndrome). Significant (p<0.001) linear regressions occurred in pharyngeal and lingual growth across gestation while ingestive behavior such as suckling emerged in a sequence of basic to complex movement patterns. Jaw and lip movements progressed from simple mouth opening to repetitive open-close movements important for postnatal suckling. Lingual movements increased in complexity from simple forward thrusting and cupping to anterior-posterior motions necessary for successful suckling at term. Laryngeal movements varied from shallow flutter-like movements along the lumen to more complex and complete adduction-abduction patterns. Fetal swallowing primarily occurred in the presence of concomitant oral-facial stimulatory activity. Significant variations (p<0.01) in the form and function of the ingestive system occurred in comparisons of gestational age-matched controls to at-risk cases. We postulate that prenatal developmental indices of emerging aerodigestive skills may guide postnatal decisions for feeding readiness and, ultimately, advance the care of the premature, medically fragile neonate.

Effects of systematic bolus viscosity and volume changes on hyoid movement kinematics.

Using ultrasonography with head and transducer stabilization, this study examined the effects of maximally controlled, systematic changes in bolus viscosity (thin juice-like, 7 cP; nectar-like, 243-260 cP; honey-like, 724-759 cP; spoon-thick, 2760-2819 cP) and volume (5, 10, 20, 30 cc) on hyoid kinematics in 31 healthy subjects (16 male, 15 female) in three age groups (20-39, 40-59, 60-79 years). Frame-by-frame hyoid displacements were tracked from digitized images of 612 swallows. Measures of movement durations, maximal amplitudes, total distances, and peak velocities were subjected to repeated measures multivariate analyses of variance with viscosity, volume, age, and gender as factors. Results showed that (1) spoon-thick swallows had the greatest preswallow gesture and total movement durations; (2) larger-volume swallows had significantly greater maximal amplitudes, forward peak velocity, and total vertical distance; (3) older subjects had longer start-to-max duration (though shorter preswallow gesture and total movement durations), greater maximal vertical amplitude, longer total vertical distance, and greater backward peak velocity than younger subjects; (4) males had greater values for all kinematic parameters except preswallow gesture, hyoid-at-max, and max-to-end durations. The results illustrate the importance of examining the interrelations among kinematic variables to better understand task accommodation and motor control strategies. The evidence also supports the concept of suprahyoid-infrahyoid functional adaptation and compensation in the healthy elderly.

Power Doppler imaging of the fetal upper aerodigestive tract using a 4-point standardized evaluation: preliminary report.

OBJECTIVE: Our investigation of fetal swallowing has identified potential limitations in the use of color Doppler imaging for detection of amniotic fluid flow and discrimination of respiratory from ingestive activity. The objective of this study was to evaluate an alternative imaging modality, power Doppler sonography, as a technique to enhance detection of amniotic fluid flow in the upper aerodigestive tract. METHODS: We applied a standardized 4-axis sonographic examination of upper aerodigestive structures and used power Doppler imaging to document amniotic fluid flow. Normal aerodigestive activities from 62 healthy control subjects were compared with 4 abnormal cases. RESULTS: Our longitudinal experience with 66 subjects showed that a directed evaluation of the fetal upper aerodigestive tract with power Doppler imaging provided a systematic approach for studying the physiologic development of this region in both healthy and at-risk fetuses. CONCLUSIONS: A standardized 4-axis examination with power Doppler imaging is a useful adjunct in addressing ingestive and respiratory functions in the developing fetus.

Kinematic strategies for hyoid movement in rapid sequential swallowing.

Past videofluoroscopic and EMG evidence has shown that rapid sequential swallowing differs from discrete swallows, but our knowledge of the control strategies remains incomplete. This study examined in detail the interrelationships among kinematic variables to discern the strategies for deglutitive hyoid motion during discrete (5 cc, 10 cc, 20 cc, 30 cc) and rapid sequential (120 cc) swallowing tasks. Submental ultrasound was conducted with head and transducer stabilization on 30 healthy subjects (15 males, 15 females) in three age groups (20-39, 40-59, 60-79 yrs). Frame-by-frame changes in hyoid position were tracked from digitized images of 236 discrete and 318 rapid sequential swallows. Repeated-measures analyses of variance were conducted on a number of kinematic variables with corrections for multiple tests and comparisons. The main effect of task was significant for all variables except forward peak velocity. Per post hoc contrasts, rapid sequential swallows had significantly reduced maximal amplitude (maximal displacement), total distance, backward peak velocity, at-max and total durations, and time to backward peak velocity in comparison with discrete swallows of any volume. Amplitude "down-scaling" was the prominent kinematic strategy used to accomplish rapid sequential swallows in a shorter time while keeping forward peak velocity essentially unchanged. In contrast, amplitude "up-scaling" was the strategy for accommodating larger-volume discrete swallows. Our results confirm built-in flexibility in the functional range of deglutitive hyoid motion.