Screening in Oculopharyngeal Muscular Dystrophy

University of Florida8 enrolled

Overview

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.

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.

Study Type

OBSERVATIONAL

Enrollment

Conditions

Oculopharyngeal Muscular Dystrophy

Interventions

Videofluoroscopic swallowing studyPROCEDURE

Videofluoroscopic swallowing study will be performed to measure the oropharyngeal swallowing.

Functional Oral Intake ScaleOTHER

The Functional Oral Intake Scale (FOIS) will be used for participants to report their food intake habits.

Eating Assessment Tool-10OTHER

The Eating Assessment Tool-10 will be used for participant reporting of swallowing system severity.

Eligibility

Sex: ALLMin age: 18 YearsMax age: 80 Years

Medical Language ↔ Plain English

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

Locations (1)

University of Florida

Gainesville, Florida, United States

Outcomes

Primary Outcomes

Dynamic Imaging Grade of Swallowing Toxicity

Global Metric of swallowing Safety and Efficiency

Time frame: Baseline

Secondary Outcomes

Dietary intake as confirmed by Functional Oral Intake Scale (FOIS)

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

Time frame: Baseline

Participant perception of swallowing related symptoms as confirmed by The Eating Assessment Tool 10 (EAT-10)

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.

Time frame: Baseline

Data from ClinicalTrials.gov

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