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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.

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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

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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.

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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.

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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.

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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.

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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.

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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.

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