Oculopharyngeal Muscular Dystrophy
Conditions
Keywords
screening, OPMD, dysphagia, dysarthria
Brief summary
Oculopharyngeal muscular dystrophy (OPMD) is a rare myopathic disease that results in progressive degeneration of the oral and pharyngeal muscular, resulting in severe dysphagia and dysarthria. OPMD is considered a rare disease; therefore, limited research is available on the natural progression of the disease or the utility of biomarkers to identify swallowing impairment. The aim of this study is: 1. To identify accurate, reliable and non-invasive clinical markers of swallowing impairment 2. To determine the discriminate ability of these markers to identify impairments in swallow safety and swallowing efficiency.
Detailed description
Participants will be recruited from the Neurology clinic at the University of Florida. The single evaluation will occur in the PIs research laboratory at the University of Florida, Gainesville, Florida.
Interventions
PROCEDUREVideofluoroscopic swallowing study
Videofluoroscopic swallowing study will be performed to measure the oropharyngeal swallowing.
The Functional Oral Intake Scale (FOIS) will be used for participants to report their food intake habits.
The Eating Assessment Tool-10 will be used for participant reporting of swallowing system severity.
Sponsors
University of Florida
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
No
Inclusion criteria
* diagnosis of oculopharyngeal muscular dystrophy * no allergies to barium or capsaicin, * no tracheotomy or mechanical ventilation * no significant concurrent respiratory disease (e.g., COPD).
Exclusion criteria
* Pregnant Women
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Dynamic Imaging Grade of Swallowing Toxicity | Baseline | Global Metric of swallowing Safety and Efficiency |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Dietary intake as confirmed by Functional Oral Intake Scale (FOIS) | Baseline | FOIS is a validated 7-point scale indexing an individual's ability to intake food on a day-to-day basis. TUBE DEPENDENT (levels 1-3) No oral intake Tube dependent with minimal/inconsistent oral intake Tube supplements with consistent oral intake TOTAL ORAL INTAKE (levels 4-7) Total oral intake of a single consistency Total oral intake of multiple consistencies requiring special preparation Total oral intake with no special preparation, but must avoid specific foods or liquid items Total oral intake with no restrictions |
| Participant perception of swallowing related symptoms as confirmed by The Eating Assessment Tool 10 (EAT-10) | Baseline | EAT-10 is a 10-item validated self-administered dysphagia severity symptom survey. Score is 0 - 40 with 0 being the best possible score and 40 the worst possible score. |
Countries
United States
Outcome results
None listed