We are using both ultrasound and videofluorography to examine effects of disease and status of oral motor function on the following conditions: stroke, postpolio syndrome, cystinosis, sydenham's chorea, and Sjogren's disease. Patients who were seen at the NIH for baseline studies from two to fifteen years after their initial examination are being followed to determine the course of change in these functions. Videofluorographic and ultrasound swallowing studies are administered along with complete oral sensory motor function examinations. Data on corticobasal degeneration and apraxia of swallowing have been analyzed. We completed a study to determine the kinematic strategies used during randomized discrete and sequential swallows on 30 subjects age 20 to 79 years. Significant differences were revealed for these two tasks relative to age, gender and movement of the hyoid bone in support of a theory of motor performance that suggests that the Deglutitive motor system is more flexible than previously known. A subset of patients with Cystinosis who were seen between 1987 and 2004 have been re-evaluated to examine the effects of cysteamine treatment and kidney transplantation on swallowing and oral motor function. To date there have been 101 subjects studied, ages ranging from 6 to 45. All subjects were administered a standard battery of assements including a swallowing self-assessment questionnaire, a cranial nerve based oral sensory-motor examination, orpharyngeal ultrasound swallow and modified barium swallow study. A significant positive correlation was found between swallowing severity and years without cystamine therapy i.e. swallowing worsened in patients who were medicated for shorter times and declines in swallowing either stabilized or improved when medicated for longer periods. Analysis of the data from these subjects indicates that older subjects with cystinosis and those with multiple medical conditions such as diabetes, hypothyroidism and elevated blood pressure appear to be at greater risk of oropharyngeal and esophageal dysphagia. This study indicates that early initiation of oral cysteamine is critical for these individuals. New signs of swallowing difficulty are also appearing in post polio patients. These continuing studies are pointing to a progression of swallowing difficulties that place patients with various neuromotor conditions at risk for aspiration.

Agency
National Institute of Health (NIH)
Institute
Clinical Center (CLC)
Type
Intramural Research (Z01)
Project #
1Z01CL060042-17
Application #
7006018
Study Section
(RM)
Project Start
Project End
Budget Start
Budget End
Support Year
17
Fiscal Year
2004
Total Cost
Indirect Cost
Name
Clinical Center
Department
Type
DUNS #
City
State
Country
United States
Zip Code
Sonies, B C (2000) Patterns of care for dysphagic patients with degenerative neurological diseases. Semin Speech Lang 21:333-44; quiz 334-5
Frattali, C M; Sonies, B C (2000) Speech and swallowing disturbances in corticobasal degeneration. Adv Neurol 82:153-60
Chi-Fishman, G; Sonies, B C (2000) Motor strategy in rapid sequential swallowing: new insights. J Speech Lang Hear Res 43:1481-92