Treatment for dysphagia (swallowing difficulties) in hereditary ataxia

Vogel, Adam P.; Keage, Megan; Schalling, Ellika

doi:10.1002/14651858.cd010169.pub2

Cited by 22 publications

(12 citation statements)

References 47 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Dysphagia significantly impacts QOL in individuals with FRDA, and management should reflect and address the significant burden associated with swallowing impairment in these individuals. This characterization work lays the foundation for future targeted clinical trials given there is an absence of high quality evidence supporting the use of any dysphagia treatment in ataxia (37).…”

Section: Resultsmentioning

confidence: 99%

Dysphagia in Friedreich Ataxia

et al. 2017

View full text Add to dashboard Cite

The objective of the study was to comprehensively characterise dysphagia in Friedreich ataxia (FRDA) and identify predictors of penetration/aspiration during swallowing. We also investigated the psychosocial impact of dysphagia on individuals with FRDA. Sixty participants with FRDA were screened for dysphagia using a swallowing quality of life questionnaire (Swal-QOL) and case history. Individuals reporting dysphagia underwent a standardised oromotor assessment (Frenchay Dysarthria Assessment, 2, FDA-2) and videofluoroscopic study of swallowing (VFSS). Data were correlated with disease parameters (age at symptom onset, age at assessment, disease duration, FXN intron 1 GAA repeat sizes, and Friedreich Ataxia Rating Scale (FARS) score). Predictors of airway penetration/aspiration were explored using logistic regression analysis. Ninety-eight percent (59/60) of participants reported dysphagia, of whom 35 (58.3%) underwent FDA-2 assessment, and 38 (63.3%) underwent VFSS. Laryngeal, respiratory, and tongue dysfunction was observed on the FDA-2. A Penetration-Aspiration Scale score above 3 (deemed significant airway compromise based on non-clinical groups) was observed on at least one consistency in 13/38 (34.2%) participants. All of those who aspirated (10/38, 26.3%) did so silently, with no overt signs of airway entry such as reflexive cough. Significant correlations were observed between dysphagic symptoms and disease duration and severity. No reliable predictors of penetration or aspiration were identified. Oropharyngeal dysphagia is commonly present in individuals with FRDA and worsens with disease duration and severity. Individuals with FRDA are at risk of aspiration at any stage of the disease and should be reviewed regularly. Instrumental analysis remains the only reliable method to detect aspiration in this population. Dysphagia significantly affects the quality of life of individuals with FRDA.

show abstract

Section: Resultsmentioning

confidence: 99%

Dysphagia in Friedreich Ataxia

et al. 2017

View full text Add to dashboard Cite

show abstract

“…» There is an absence of any significant evidence supporting the use of dysphagia intervention in hereditary ataxia. (Vogel et al 2015) These 'no evidence' and 'absence of evidence' statements appear to provide the weakest basis of all the statements that we have examined for a conclusion based on ignorance. For while there is some evidence that p, even some weak evidence that p, there appears to be little rational warrant for drawing the conclusion that not-p.…”

Section: Weakly Warranted Argument From Ignorancementioning

confidence: 87%

Arguing from Ignorance

Cummings

2020

Fallacies in Medicine and Health

View full text Add to dashboard Cite

“…Videofluoroscopy can detect even subclinical swallowing difficulties and thus leads to higher prevalence estimates [5]. Systematic evaluation of diagnostic procedures and treatment is lacking [29].…”

Section: Discussionmentioning

confidence: 99%

“…The rate of patients experiencing recurrent bronchitis and pneumonia due to aspiration in a long-term study of 23 patients suffering from different forms of cerebellar ataxia was up to 50% and 13% of the patients died of aspiration pneumonia [31]. Thus, the correct diagnosis and recognition of dysphagia cannot be underestimated [29]. For detection of dypshagic symptoms, Swal-QOL and clinical case history should be considered and the Swal-QOL should be used as an additional tool to detect impact on HRQOL.…”

Section: Discussionmentioning

confidence: 99%

Dysphagia Affecting Quality of Life in Cerebellar Ataxia—a Large Survey

et al. 2020

View full text Add to dashboard Cite

Dysphagia is a common symptom in neurodegenerative disorders and is generally associated with increased mortality. In the clinical care setting of ataxia patients, no systematical and standardized assessment of dysphagia is employed. Its impact on patients' healthrelated quality of life is not well understood. To assess the impact of dysphagia in ataxia patients on diet, body weight, and healthrelated quality of life. We conducted a large survey using self-reported questionnaires for swallowing-related quality of life (Swal-QOL) and a food frequency list in combination with retrospective clinical data of 119 patients with cerebellar ataxia treated in the neurological outpatient clinic of a large German university hospital. Seventeen percent of ataxia patients suffered from dysphagia based on the Swal-QOL score. Less than 1% of all patients reported dysphagia as one of their most disabling symptoms. Dysphagia was associated with unintentional weight loss (p = 0.02) and reduced health-related quality of life (p = 0.01) but did not affect individual nutritional habits (p > 0.05; Chi-squared test). Dysphagia is a relevant symptom in cerebellar ataxia. A systematic screening for dysphagia in patients with cerebellar ataxia would be desirable to enable early diagnosis and treatment. Keywords Dysphagia . Cerebellar ataxia . Health-related quality of life . Spinocerebellar ataxia . Nutrition M. Rönnefarth and N. Hanisch contributed equally to this work.

show abstract

Treatment for dysphagia (swallowing difficulties) in hereditary ataxia

Cited by 22 publications

References 47 publications

Dysphagia in Friedreich Ataxia

Dysphagia in Friedreich Ataxia

Arguing from Ignorance

Dysphagia Affecting Quality of Life in Cerebellar Ataxia—a Large Survey

Contact Info

Product

Resources

About