Abstract
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.
Keywords: Machado Joseph disease, spinocerebellar ataxias, dysphagia, videofluoroscopy of swallowing, guidelines
Machado Joseph disease/SCA type 3 (SCA3/MJD) is a polyQ disorder caused by a dominant CAG repeat expansion (CAGexp) at the ATXN3 gene.1 SCA3/MJD is the most frequent SCA worldwide; in our region, SCA3/MJD has a minimal prevalence of 3 in 100,000 inhabitants.2 Very disabling, SCA3/MJD usually starts at around 32 to 40 years. Gait ataxia is usually the first neurological deficit; a progressive incoordination also affects eye and limb movements, speech, and swallowing. Lower motor neuron, pyramidal, extrapyramidal, and peripheral nerve dysfunctions may occur.3 Currently untreatable, the median survival time after onset is 21 years.4
Dysphagia affects approximately 63% of SCA3/MJD patients.5, 6 Common symptoms include coughing or choking, insufficient control of solids or liquids in the mouth, drooling, food lodging in the pharynx, aspiration pneumonia, and asphyxia. Given that postmortem studies have reported that aspiration pneumonia was the most common cause of death in SCAs,6, 7 dysphagia stands out as a significant complication of these disorders. Detailed clinical studies on dysphagia in SCAs are lacking. We have previously evaluated dysphagia according to patient information and obtained some intriguing results.5, 8, 9 By using the patients' reply to one item in the clinical scale, Neurologic Examination Score of Spinocerebellar Ataxias (NESSCA),9 the degree of dysphagia has been related to disease duration (DD), disease severity (DS), and length of the CAGexp,5 but not to weight loss, as measured by the body mass index (BMI), in SCA3/MJD.8
The most valid assessment of dysphagia is the videofluoroscopic (VF) study of swallowing.10 Specific materials containing barium are swallowed and viewed by examiners as they pass from the lips to the upper esophageal sphincter. Among the quantitative evaluations of VF are the penetration/aspiration scale (PAS)11 and the Dysphagia Outcome and Severity Scale (DOSS)12: Both measure the severity of airway invasion. Very few VF studies have been performed in SCAs, and all have used very small sample sizes.6, 13
Early detection of dysphagia is essential for prescribing swallowing rehabilitation, foods with easy‐to‐swallow textures, and percutaneous endoscopic gastrostomy (PEG), where needed. This management might prevent aspiration pneumonia caused by dysphagia and might change the clinical course of SCA patients. Therefore, we aimed to describe dysphagia severity in SCA3/MJD by VF, correlate VF findings with DD, CAGexp, and BMI, and determine the best clinical criteria and their cutoffs to indicate VF in the clinical routine, in order to prevent complications of dysphagia in SCA3/MJD patients.
Methods
Symptomatic individuals over 18 years of age with molecular diagnosis of MJD/SCA3 from the Hospital de Clinicas de Porto Alegre (Rio Grande do Sul, Brazil) were invited to participate in the study between 2013 and 2014. Patients were selected consecutively, with the proviso that there would be a broad representation of DD—from 2 to more than 21 years after onset. Exclusion criteria included previous diagnosis of another disease that impairs swallowing, gastrointestinal obstruction, and pregnancy. This study was approved by our institution's ethics in research committee (register no.: 12‐0476).
After consent, clinical data, such as gender, age, age at onset (AO), and DD, were obtained. Nutritional status was evaluated by BMI [weight/(height)2]. NESSCA and the Scale for Assessment and Rating of Ataxia (SARA)14 were applied by trained researchers (A.C.K‐S., E.R.R., and M.C.A.). The Swallowing Quality of Life questionnaire (SWAL‐QOL)15 was applied by one investigator (A.D.R.), as previously described. Briefly, SWAL‐QOL is a 44‐item dysphagia‐specific outcomes tool that assesses quality of life in swallowing, related to 11 domains: burden; food selection; eating duration; symptoms; mental health; social functioning; fear; eating desire; communication; sleep; and fatigue. SWAL‐QOL scores range from 0 to 100 points. A mean score for all domains was constructed, and the higher the value, the better is the quality of life related to swallowing.15
Within 30 days of the clinical evaluation, subjects were submitted to VF, performed by a speech therapist blind to the previous evaluations (B.S.). Exams were run in the Axion Iconos R100 machine by Siemens. Patients were seated and video images were recorded on DVDs in lateral and anteroposterior views. Feeding was offered in the following sequence: pasty; thickened liquid; liquid; and solid (50%/50% between food and barium contrast). The subjects were requested to swallow in their usual way. The worst score obtained in these swallowing attempts was used, according to the scales described below.
VF results were measured by PAS and DOSS scores. PAS has the following possible scores: (1) The material does not enter airway; (2) it enters the airway, remains above the vocal folds, and is ejected from the airway; (3) the material enters the airway, remains above the vocal folds, and is not ejected from the airway; (4) it enters the airway, contacts the vocal folds, and is ejected from the airway; (5) it contacts the vocal folds and is not ejected from the airway; (6) it passes below the vocal folds and is ejected into the larynx or out of the airway; (7) it passes below the vocal folds and is not ejected from the trachea despite effort; and (8) it passes below the vocal folds, and no effort is made to eject.11 The 7‐point bidimensional DOSS has detailed items that evaluate observations similar to those described in PAS, plus information about feeding strategies—from coughing to enteral nutrition—used by the individual. DOSS has the following possible scores: (1) severe dysphagia: swallowing impossible; (2) moderately severe dysphagia: maximum assistance and total use of strategies with partial PO, only after training; (3) moderate dysphagia: needs supervision, or strategies, two or more diet consistencies restricted; (4) mild‐to‐moderate dysphagia: needs intermittent supervision, one to two consistencies restricted; (5) mild dysphagia: needs distant supervision, may need one diet consistency restricted; (6) within functional limits/modified independence; and (7) normal in all situations.12
Patient characteristics are given as median (range), when applicable. Categorical variables were represented by absolute frequencies and were compared through chi‐square test. Several continuous variables did not show a normal distribution on Shapiro‐Wilk's test and therefore were tested by Mann‐Whitney's U and Spearman's correlation tests. These analyses were performed in PASW 18.0.0.
The cut‐off values of SARA score, BMI, and eating duration‐SWAL‐QOL for detecting significant dysphagia (DOSS <4 points or PAS >3) were calculated through the receiver operating characteristic (ROC) curve; the optimal cut‐off values were calculated by Youden's index: max [Sensitivity + (Specificity − 100)]. Areas under the curves (AUCs) were compared by means of the roc.test function of the pROC R 3.1.0 package.16 In addition, a joint criterion (SARA, BMI, and eating duration) was formed by logistic regression analysis, with a resulting ROC curve produced with the predicted probabilities. Statistical significance was defined as P < 0.05.
Results
Thirty‐four SCA3/MJD individuals were included in the present study. Clinical and molecular characteristics are described in Table 1. Although there were more women than men in the present sample (21 of 31), this skewness only interfered with DD, which were longer among men.
Table 1.
General characteristics of the present sample
| Variable | Overall | Males | Females | P Value |
|---|---|---|---|---|
| No. of individuals | 34 | 13 | 21 | 0.0001a |
| Ageb, yr | 53 (18–73) | 56 (20–73) | 51 (18–71) | NSc |
| Age at onsetb, yr |
38 (2) (11–54) |
45 (2.8) (18–54) |
37 (11–52) | NSc |
| Disease durationb, yr | 10 (2–30) | 12 (2–20) | 10 (4–30) | 0.02c |
| Normal CAGnb repeats | 23 (14–29) | 23 (14–29) | 23 (14–28) | NAc |
| Expanded CAGnb repeats | 74 (70–81) | 71 (70–81) | 76 (72–83) | NSc |
| BMIb, kg/m² | 23 (15.4–31.6) | 24.2 (19.4–31.6) | 21.5 (15.4–28.8) | NSc |
| NESSCA (0–40)b | 18 (10–33) | 18 (10–33) | 18 (14–29) | NSc |
| SARA (0–40)b | 15.75 (1.5–39) | 15 (1–31) | 16 (7–39) | NSc |
| Total SWAL‐QOL (0–100)b | 65.5 (25.7–94) | 80.6 (25.7–88.2) | 60 (46–94) | NSc |
| DOSS (1–7)b | 5 (1–7) | 5 (4–7) | 5 (1–7) | NSc |
| PAS (1–8)b | 1 (1–8) | 1 (1–8) | 1 (1–8) | NSc |
Chi‐square.
Median (range).
Mann‐Whitney's U test.
Three individuals were totally normal according to DOSS and scored 7; the other 31 (91%) presented some disturbance in deglutition. Fourteen of thirty‐four subjects were normal according to PAS and scored 1; in the other 20 (58%), the ingested material entered the airway, with different responses. Figure 1 and Video 1 illustrate some findings at VF. DOSS and PAS scores correlated significantly (ρ = −0.8; P = 0.0001, Spearman). Because many patients presented mild scores in both VF severity scores, in order to look for associations, we dichotomized the sample according to the presence of nonejected aspiration (PAS = 3) and mild‐moderate dysphagia (DOSS = 4); the distribution of scores obtained is shown in Figure S1. There were 29 mild (≥5) and 5 moderate‐severe (≤4 points) DOSS patients and 28 mild (≤2) and 6 moderate‐severe (≥3 points) PAS patients. Low scores indicated individuals with low risk for aspiration. High scores identified subjects with significant dysphagia (or high risk for aspiration) and will be called “significant scores” from now on.
Figure 1.
X‐ray images captured from VF studies of patients with SCA3/MJD. (A) Aspiration of solid content. (B) Microaspiration of liquid content. (C) Microaspiration of thickened liquid or nectar.
VF Scores Versus Parameters of Disease Severity
AO, DD, and CAGexp were not associated with PAS and DOSS, either in correlation or in comparison between dichotomized groups (Spearman and Mann‐Whitney, nonsignificant [NS]).
DOSS correlated with SARA (ρ = −0.363; P = 0.035) and NESSCA (ρ = −0.351; P = 0.042), and PAS with SARA (R = 0.353; P = 0.040). Subjects with significant DOSS scores showed SARA and NESSCA scores significantly higher than the mild DOSS group (P = 0.003 and 0.02, Mann‐Whitney). Subjects with significant PAS scores presented SARA scores significantly higher than the mild PAS group (P = 0.007, Mann‐Whitney). The distribution suggested that a specific SARA score might divide both groups (Fig. 2A).
Figure 2.
Significant associations obtained between potentially predictive variables under study and the VF scores DOSS and PAS. (A) SARA scores obtained according to PAS scores mild (1–2 points) and severe (>3 points). (B) BMI obtained according to DOSS scores mild (≥5 points) and severe (<4 points), and to (C) PAS scores mild (1–2 points) and severe (>3 points). (D) Scores obtained on domain eating duration of SWAL‐QOL, according to DOSS scores mild (≥5 points) and severe (<4 points), and to (E) PAS scores mild (1–2 points) and severe (>3 points).
VF Scores Versus Weight Loss
Both DOSS (ρ = 0.454; P = 0.007) and PAS (ρ = −0.453; P = 0.007) correlated with BMI. When the results were stratified per subgroup, the association was maintained. The distribution suggested that BMI of 23 would detect all individuals with significant dysphagia according to PAS (Fig. 2B).
SWAL‐QOL Scores and the Dysphagia Item of NESSCA Versus Severity Parameters of MJD/SCA3 and Versus VF
SWAL‐QOL was not associated either with NESSCA or SARA, or with severity variables CAGexp, AO, and DD (Spearman, NS). NESSCA item “dysphagia” also failed to be associated with these variables (Kruskal‐Wallis, NS).
More important, SWAL‐QOL scores as a whole and NESSCA item “dysphagia” were not associated with VF scores (Spearman and Kruskal‐Wallis, NS).
Only the domain eating duration of SWAL‐QOL questionnaire was associated with DOSS (ρ = 0.446; P = 0.008) and PAS (ρ = −0.497; P = 0.003) scores. The distribution suggested that low scores at eating duration were associated with significant dysphagia (Fig. 2C).
Predictors of Dysphagia in the Present Sample
SARA, BMI, and eating duration‐SWAL‐QOL were tested in order to identify their value as predictors of significant dysphagia, taking both DOSS and PAS scores as the gold‐standard tests. As decided earlier, DOSS <4 points and PAS >3 points were considered the parameters for significant dysphagia.
Cut‐off points were set by ROC curves. As can be seen in Figure 3, SARA, BMI, and eating duration‐SWAL‐QOL presented good distribution of sensitivity versus 1‐specificity.
Figure 3.
ROC curves. (A) ROC curves for the three variables, SARA, BMI, and eating duration of SWAL‐QOL, when the dysphagia gold standard was DOSS (<4 points). Values with the highest index for DOSS <4 points were 18.25 points on SARA score (AUC = 0.91; 95% CI: 0.80–1.00; sensitivity = 0.76; specificity = 1.0; NPV = 1; PPV = 0.42); 17 kg/m2 on BMI (AUC = 0.93; 95% CI: 0.79–1.00; sensitivity = 1.0; specificity = 0.80; NPV = 0.97; PPV = 1.0) and 31.25 on eating duration‐SWAL‐QOL (AUC = 0.88; 95% CI: 0.77–0.99; sensitivity = 0.79; specificity = 1.0; NPV = 1.0; PPV = 0.45). Depicted are 100% sensitivities for each. (B) ROC curves for the three variables, SARA, BMI, and eating duration of SWAL‐QOL, when the dysphagia gold standard was PAS (>3 points). Values with the highest index for PAS >3 were 29 points on SARA score (AUC = 0.85; 95% CI: 0.70–1.00; sensitivity = 0.92; specificity = 0.67; NPV = 0.92; PPV = 0.67); 17 kg/m2 on BMI (AUC = 0.86; 95% CI: 0.68–1.00; sensitivity = 1.0; specificity = 0.67; NPV = 0.93; PPV = 1.0) and 31.25 on eating duration‐SWAL‐QOL (AUC = 0.84; 95% CI: 0.70–0.97; sensitivity = 0.79; specificity = 0.83; NPV = 0.96; PPv = 0.45). Depicted are 100% sensitivities for each. (C) ROC curve for the three variables together, when the gold standard was DOSS. AUC was of 0.98 (95% CI: 0.9329–1.00). (D) ROC curve for the three variables together, when the gold standard was PAS. AUC was 0.93 (95% CI: 0.82–1.00). CI, confidence interval; NPV negative predictive value; PPV, positive predictive value.
Values with the highest index for DOSS <4 points are depicted in Figure 3A. Comparisons between areas under the ROC curves did not disclose significant differences. A sensitivity of 1 corresponded to a cutoff of 18 points on SARA, 22.88 kg/m2 on BMI, and 31.25 on eating duration‐SWAL‐QOL.
Values with the highest index for PAS >3 are shown in Figure 3B. Comparisons between areas under the ROC curves did not disclose significant differences. A sensitivity of 1 corresponded to a cutoff of 15 points on SARA, 23.72 kg/m2 on BMI, and 56.25% (rounded to 60%) on eating duration‐SWAL‐QOL.
When the three variables, SARA, BMI, and eating duration‐SWAL‐QOL, were combined to construct a unique ROC curve, AUCs were slightly improved for both DOSS <4 points and PAS >3 (Fig. 3C,D).
Discussion
In this study, dysphagia was present in a substantial number of SCA3/MJD individuals: 58% presented aspiration, as measured by PAS, and 91% showed some degree of dysphagia, as measured by DOSS. In the majority of cases, dysphagia was mild. Dysphagia was not associated with DD or with CAGexp, but with clinical scores SARA and NESSCA. Subjects with worse scores of dysphagia presented lower BMI and complained of an increased eating time. In order to suggest guidelines for preventing aspiration in SCA3/MJD, we estimated cut‐off values with a 100% sensitivity to identify all individuals with significant dysphagia.
Dysphagia in SCAs has been measured mostly by clinical scales or patient descriptions.9, 17, 18 In NESSCA, for instance, the patient is asked if he or she chokes: if no, he or she scores 0. If yes, he or she is asked if it happens every day: if no, he or she scores 1; if yes, he or she scores 2. This score has been previously related to DD, DS, and to the CAGexp.5 The same score was not related to weight loss, as measured by BMI,8 and we started to think that subjective evaluations might be prone to denial by patients, for instance, in order to postpone or avoid measures such as PEG. Among the alternatives to evaluate dysphagia, there were the more detailed clinical scales based on Likert methodology, such as SWAL‐QOL, and the quantitative evaluations of VF, such as DOSS and PAS.
The SWAL‐QOL questionnaire was developed to be used in clinical research.15, 19 Focused on the impact of swallowing problems in quality of life, SWAL‐QOL was considered the most reliable tool for studying subjective swallowing difficulties.20 However, total SWAL‐QOL results did not correlate with severity variables CAGexp, AO, DD, NESSCA, or SARA in our study. Moreover, SWAL‐QOL did not correlate with the gold‐standard VF scales, DOSS and PAS. The lack of association between total SWAL‐QOL and PAS has been previously reported on, in spite of the very mild correlations with some subdomains.21 Therefore, SWAL‐QOL results should not be use used to infer dysphagia in SCA3/MJD.
VF has only been reported on in two retrospective studies on SCAs: It was obtained in 2 SCA3/MJD patients and 1 SCA7 patient, apparently of European origin6, 22; and in 7 SCA3/MJD and 13 SCA6 patients of Japanese origin.13
In order to confirm whether dysphagia was related to disease progression, we tried to recruit individuals with a broad range of DD. Although prevalent, the dysphagia measured by VF was more frequently mild in our case series and failed to be associated with DD or with CAGexp. Similar findings were obtained by others.13 This means that some SCA3/MJD patients are free from significant dysphagia even after several years of disease, and that DD and CAGexp cannot be used to decide when to perform VF studies.
In contrast, VF studies correlated with SARA and NESSCA, which represent disease severity in a very objective way. Distribution of SARA scores according to the severity of PAS suggested, and the ROC curve confirmed, that a SARA cutoff could be used to decide when to perform VF in clinical settings (Figs. 2A and 3).
Subjects with significant scores of dysphagia presented lower BMI than those with mild scores. Distribution of BMI and its ROC curve pointed to a cut‐off point between patients with and without significant dysphagia—the 23 kg/m2 (Figs. 2B and 3). This is a strange cutoff, given that it lies between the limits of health. According to the World Health Organization, healthy BMI values range between 18.5 and 24.99 kg/m2, are age independent, and are the same for both sexes.23 Our BMI cutoff can be explained by the transversal design of the present study, the inability to detect a weight loss in process, and weight loss occurring in a previously obese patient, as well as the population‐based shift among Brazilians toward being overweight.8 In this scenario, we believe that BMI distributions and cutoffs are population related and are not generalizable to other countries.
Several motor and sensory dysfunctions can be detrimental to the ingestive process in SCA3/MJD: weakness and ataxia of the jaw muscles, facial hypokinesia, orofacial dystonia, reduced pharyngeal reflexes, and slowed lingual and faringeal movements, among others.6 Eating might turn into a difficult, time‐consuming task. In line with this, our subjects with worse PAS and DOSS complained of an increased eating time than others, as measured by the eating duration of SWAL‐QOL. This domain includes two sentences with which the subject should agree or not (five alternatives: “Very much true,” “Quite a bit true,” “Somewhat true,” “A little true,” and “Not at all true”): “It takes me longer to eat than other people” and “it takes me forever to take a meal.” The two most negative replies to these statements equal 100%; the two most affirmative replies equal 0%. Again, the distribution of this domain suggested, and the ROC curve confirmed, that a cutoff could be used to decide when to perform VF (Figs. 2C and 3).
VF is a gold‐standard test for dysphagia, but there is no guideline about the proper time to perform it, in SCAs. We have tested the sensitivity and specificity of these three measurements—SARA, BMI, and eating duration of SWAL‐QOL—to detect significant dysphagia in VFs. All three are very easy to obtain in a clinical setting and do not depend on any special equipment. We have measured two scales considered the gold standard, PAS and DOSS. In line with this, ROC curves have been built for SARA, BMI, or eating duration‐SWAL‐QOL, all giving consistent AUCs. Given that our aim was to suggest cutoffs for use as screening tests for significant dysphagia, we used these ROC curves to find the values with the highest sensitivity in order to detect all patients with significant risk for aspiration. Our most conservative results showed that, if a physician uses a SARA score of 15 points or an eating duration‐SWAL‐QOL of 60% as cut‐off values to indicate a VF, he or she would be unable to overlook any single SCA3/MJD subject with significant dysphagia (Fig. 3).
In conclusion, dysphagia is an important, though sometimes forgotten, complication of SCA3/MJD that evolves alongside disease progression, as measured by NESSCA and SARA. As a consequence, some weight loss takes place. Subjective information, the most common method of assessing dysphagia on clinical setting, was not correlated with VF nor with other clinical parameter in the present study, except for the eating duration domain of SWAL‐QOL. Therefore, health professionals that assist SCA3/MJD patients should rely directly on the objective evaluation of swallowing—the VF. SARA, BMI, and the eating duration domain of SWAL‐QOL might help in the decision‐making process. They should be routinely assessed in the care of SCA3/MJD patients. Whereas the cutoff of BMI should be determined in each population of origin, SARA scores greater than 15 points or eating duration‐SWAL‐QOL lower than 60% should urge VF studies, in order to detect significant dysphagia and aspiration in SCA3/MJD patients.
Author Roles
(1) Research Project: A. Conception, B. Organization, C. Execution; (2) Statistical Analysis: A. Design, B. Execution, C. Review and Critique; (3) Manuscript Preparation: A. Writing of the First Draft, B. Review and Critique.
A.D.R.: 1A, 1B, 1C, 2C, 3B
E.R.R.: 1C, 3B
A.C.K.‐S. 1C, 3B
M.C.A.: 1C, 3B
B.S.: 1C, 3B
C.D.F.: 1C, 3B
V.L.T.: 2A, 2B, 3B
M.‐L.S.‐P.: 1B, 3B
J.A..S.: 1B, 2A, 2C, 3B
L.B.J.: 1A, 1B, 2A, 2B, 2C, 3A, 3B
Disclosures
Funding Sources and Conflicts of Interest: This study was funded by Fundo de Incentivo à Pesquisa, Hospital de Clínicas de Porto Alegre (FIPE‐HCPA; register no.: 12‐0476). The authors report no conflicts of interest.
Financial Disclosure for previous 12 months: E.R.R. received an undergraduate research grant by the Brazilian agency CNPq, PIBIC CNPq UFRGS. A.C.K‐S. received an undergraduate research grant by the Brazilian agency FAPERGS, PROBIC HCPA. M.C.A. received an undergraduate research grant by the Brazilian agency FAPERGS, PPSUS 1209‐2551/13‐4. L.B.J. received research grants by the Brazilian agencies CNPq (Universal 478057/2012‐1; Universal 478888/2010‐4; and PVE 402968/2012‐3) and FAPERGS (PPSUS 1209‐2551/13‐4 and PPSUS 09/0078‐5). M.‐L.S.‐P. and L.B.J. are suportedd by CNPq.
Supporting information
A video accompanying this article is available in the supporting information here.
Figure S1. Distribution of VF severity scores DOSS and PAS in the present sample
Video 1. X‐ray video captured from a VF study of a SCA3/MJD patient. Side view: After ejection from the oral cavity to the oropharynx, the liquid content surpasses glottis and a tracheal aspiration occurs. No effort was made to eject. PAS score of 8.
Acknowledgments
The authors thank the patients who participated in the study. The authors thank HCPA and its Postgraduate Research Group (GPPG). This study was supported by FIPE (12‐0476). M.‐L.S.‐P. and L.B.J. were supported by CNPq.
Relevant disclosures and conflicts of interest are listed at the end of this article.
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Supplementary Materials
A video accompanying this article is available in the supporting information here.
Figure S1. Distribution of VF severity scores DOSS and PAS in the present sample
Video 1. X‐ray video captured from a VF study of a SCA3/MJD patient. Side view: After ejection from the oral cavity to the oropharynx, the liquid content surpasses glottis and a tracheal aspiration occurs. No effort was made to eject. PAS score of 8.