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Adapting Human Videofluoroscopic Swallow Study Methods to Detect and Characterize Dysphagia in Murine Disease Models
Published on: March 1, 2015
Clinical Scales Predict Significant Videofluoroscopic Dysphagia in Machado Joseph Disease Patients
Aline D Russo1, Estela R Reckziegel2, Ana C Krum-Santos2
1Post-graduate Program of Medical Sciences Universidade Federal do Rio Grande do Sul Porto Alegre Rio Grande do Sul Brazil.
Background:
Although aspiration is one of the main causes of death in SCA, such as SCA3/Machado Joseph disease (SCA3/MJD), clinical studies on dysphagia are lacking for these diseases. The aims of this study were to characterize dysphagia in SCA3/MJD through videofluoroscopy (VF) of swallowing, correlate VF with disease severity criteria and weight loss, and determine the clinical criteria cutoffs for performing VF in the clinical routine, in order to detect aspiration.
Methods:
A cross-sectional study on 34 SCA3/MJD patients was performed. Clinical and molecular data, as well as body mass index (BMI), were obtained. Neurological scales, such as the Scale for the Assessment and Rating of Ataxia (SARA), and the Swallowing Quality of Life (SWAL-QOL) questionnaire were applied. The VF scores, Dysphagia Outcome and Severity Scale (DOSS) and penetration/aspiration scale (PAS), were obtained: Moderate-to-severe scores were grouped as "significant dysphagia."
Results:
Overall, 31 of 34 individuals showed abnormal scores at VF. SARA, BMI, and the domain "eating duration" of SWAL-QOL correlated with VF: Their relation to significant dysphagia (DOSS <4 points or PAS >3) was evaluated through receiver operating characteristic curves. A sensitivity of 100% was equivalent to a cutoff of 15 points on SARA score, 23.72 kg/m2 on BMI, and 60% on eating duration-SWAL-QOL (P < 0.05).
Conclusion:
Significant dysphagia was not related to age at onset, disease duration, or CAG repeat expansion, but with SARA scores, lower BMI, and the domain eating duration of SWAL-QOL. As a guideline for preventing aspiration, we suggest that SARA scores greater than 15 or eating duration-SWAL-QOL lower than 60% should urge VF studies in SCA3/MJD.
Insights
Dysphagia and aspiration are serious risks in Spinocerebellar Ataxia type 3 (SCA3/MJD). This study identified key clinical indicators, including SARA scores and eating duration, to guide videofluoroscopy (VF) for early detection and aspiration prevention in SCA3/MJD patients.
Area of Science:
- Neurology
- Gastroenterology
- Genetics
Background:
- Aspiration poses a significant mortality risk in Spinocerebellar Ataxia type 3 (SCA3/MJD).
- Clinical characterization of dysphagia in SCA3/MJD remains limited.
- This study aimed to characterize dysphagia and identify predictors for aspiration risk in SCA3/MJD patients.
Purpose of the Study:
- To characterize dysphagia in SCA3/MJD using videofluoroscopy (VF).
- To correlate VF findings with disease severity and weight loss.
- To establish clinical criteria for timely VF performance to detect aspiration.
Main Methods:
- A cross-sectional study involving 34 SCA3/MJD patients.
- Assessment included clinical, molecular data, BMI, SARA, and SWAL-QOL.
- VF was evaluated using DOSS and PAS scores; significant dysphagia was defined as DOSS <4 or PAS >3.
Main Results:
- 31 out of 34 patients exhibited abnormal VF scores.
- SARA scores, BMI, and SWAL-QOL "eating duration" significantly correlated with dysphagia severity.
- Cutoffs for 100% sensitivity in predicting significant dysphagia were SARA ≥ 15, BMI ≤ 23.72 kg/m², and "eating duration" ≤ 60%.
Conclusions:
- Significant dysphagia in SCA3/MJD is associated with higher SARA scores, lower BMI, and reduced "eating duration" on SWAL-QOL.
- Disease onset age, duration, and CAG repeat size were not correlated with dysphagia severity.
- SARA scores >15 or "eating duration" <60% warrant VF to prevent aspiration in SCA3/MJD.
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