Dataset Information

Fiberoptic endoscopic evaluation of swallowing in early-to-advanced stage Huntington's disease.

ABSTRACT: Huntington's disease (HD) is a neurodegenerative disorder characterized by motor disturbances, cognitive decline, and behaviour changes. A well-recognized feature of advanced HD is dysphagia, which leads to malnutrition and aspiration pneumonia, the latter being the primary cause of death in HD. Previous studies have underscored the importance of dysphagia in HD patients with moderate-to-advanced stage disease, but it is unclear whether dysphagia affects patients already at an early stage of disease and whether genetic or clinical factors can predict its severity. We performed fiberoptic endoscopic evaluation of swallowing (FEES) in 61 patients with various stages of HD. Dysphagia was found in 35% of early-stage, 94% of moderate-stage, and 100% of advanced-stage HD. Silent aspiration was found in 7.7% of early-stage, 11.8% of moderate-stage, and 27.8% of advanced-stage HD. A strong correlation was observed between disease progression and dysphagia severity: worse dysphagia was associated with worsening of motor symptoms. Dysphagia severity as assessed by FEES correlated with Huntington's Disease Dysphagia Scale scores (a self-report questionnaire specific for evaluating swallowing in HD). The present findings add to our understanding of dysphagia onset and progression in HD. A better understanding of dysphagia onset and progression in HD may inform guidelines for standard clinical care in dysphagia, its recognition, and management.

SUBMITTER: Schindler A

PROVIDER: S-EPMC7499207 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature

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Fiberoptic endoscopic evaluation of swallowing in early-to-advanced stage Huntington's disease.

Schindler Antonio A Pizzorni Nicole N Sassone Jenny J Nanetti Lorenzo L Castaldo Anna A Poletti Barbara B Solca Federica F Pirola Francesca F Lazzari Laura L Stramba-Badiale Marco M Rossi Agnese A Silani Vincenzo V Mariotti Caterina C Ciammola Andrea A

Scientific reports 20200917 1

Huntington's disease (HD) is a neurodegenerative disorder characterized by motor disturbances, cognitive decline, and behaviour changes. A well-recognized feature of advanced HD is dysphagia, which leads to malnutrition and aspiration pneumonia, the latter being the primary cause of death in HD. Previous studies have underscored the importance of dysphagia in HD patients with moderate-to-advanced stage disease, but it is unclear whether dysphagia affects patients already at an early stage of dis ...[more]

PMID: 32943712

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Project description:BackgroundWith longer life spans and medical advancements, the rising number of patients with advanced-stage Parkinson's disease (PD) warrants attention. Current literature predominantly addresses dementia and fall management in these patients. However, exploring the impact of swallowing function on patients with advanced PD is crucial. Previous research has demonstrated notable enhancements in the quality of life related to voice for participants following a group singing-intervention program. To further elucidate the effect of individual singing-induced swallowing exercises, our study aimed to investigate the quantitative and qualitative effects of therapeutic singing on swallowing function in patients with advanced PD in comparison to a matched usual care control group. The hypothesis of this study is that therapeutic singing-induced swallowing exercises can assist to maintain swallowing function in patients with advanced PD.MethodsThis prospective matched control study compared the effects of a 6-week therapeutic singing-based swallowing intervention on swallowing function and quality of life in patients with advanced PD. The intervention group received individual sessions with a music therapist and conventional individual physical therapy. The control group received the same standard physical therapy for 6 weeks without music intervention. The primary outcome measure was Video Fluoroscopic Dysphagia Scale (VDS).ResultsThe study revealed that the intervention group maintained swallowing function, whereas the control group experienced deterioration, indicating significant time-dependent changes in Penetration-Aspiration Scale (PAS), National Institutes of Health-Swallowing Safety Scale (NIH-SSS), and VDS. Analysis of PAS and NIH-SSS liquid food scores in both groups showed significant time effects. However, the intervention group exhibited no significant differences between the pre- and post-tests, indicating preservation of the swallowing function. VDS of liquid food indicated an interaction effect between time and group in the pharyngeal phase and total scores. The Swallowing-Quality of Life showed significant time-effect improvement in the intervention group.ConclusionTherapeutic singing exercises may help maintain swallowing function in advanced PD patients, potentially enhancing quality of life related to swallowing in those with advanced-stage diseases.Clinical trial registrationhttps://cris.nih.go.kr/cris/search/listDetail.do, identifier KCT0008644.

Project description:BackgroundRemoval of a tracheostomy tube in critically ill neurologic patients is a critical issue during intensive care treatment, particularly due to severe dysphagia and insufficient airway protection. The "Standardized Endoscopic Evaluation for Tracheostomy Decannulation in Critically Ill Neurologic Patients" (SESETD) is an objective measure of readiness for decannulation. This protocol includes the stepwise evaluation of secretion management, spontaneous swallowing, and laryngeal sensitivity during fiberoptic endoscopic evaluation of swallowing (FEES). Here, we first evaluated safety and secondly effectiveness of the protocol and sought to identify predictors of decannulation success and decannulation failure.MethodsA prospective observational study was conducted in the neurological intensive care unit at Münster University Hospital, Germany between January 2013 and December 2017. Three hundred and seventy-seven tracheostomized patients with an acute neurologic disease completely weaned from mechanical ventilation were included, all of whom were examined by FEES within 72 h from end of mechanical ventilation. Using regression analysis, predictors of successful decannulation, as well as decannulation failure were investigated.ResultsTwo hundred and twenty-seven patients (60.2%) could be decannulated during their stay according to the protocol, 59 of whom within 24 h from the initial FEES after completed weaning. 3.5% of patients had to be recannulated due to severe dysphagia or related complications. Prolonged mechanical ventilation showed to be a significant predictor of decannulation failure. Lower age was identified to be a significant predictor of early decannulation after end of weaning. Transforming the binary SESETD into a 4-point scale helped predicting decannulation success in patients not immediately ready for decannulation after the end of respiratory weaning (optimal cutoff ≥1; sensitivity: 64%, specifity: 66%).ConclusionsThe SESETD showed to be a safe and efficient tool to evaluate readiness for decannulation in our patient collective of critically ill neurologic patients.

Project description:BackgroundIn Huntington's disease clinical trials, recruitment and stratification approaches primarily rely on genetic load, cognitive and motor assessment scores. They focus less on in vivo brain imaging markers, which reflect neuropathology well before clinical diagnosis. Machine learning methods offer a degree of sophistication which could significantly improve prognosis and stratification by leveraging multimodal biomarkers from large datasets. Such models specifically tailored to HD gene expansion carriers could further enhance the efficacy of the stratification process.ObjectivesTo improve stratification of Huntington's disease individuals for clinical trials.MethodsWe used data from 451 gene positive individuals with Huntington's disease (both premanifest and diagnosed) from previously published cohorts (PREDICT, TRACK, TrackON, and IMAGE). We applied whole-brain parcellation to longitudinal brain scans and measured the rate of lateral ventricular enlargement, over 3 years, which was used as the target variable for our prognostic random forest regression models. The models were trained on various combinations of features at baseline, including genetic load, cognitive and motor assessment score biomarkers, as well as brain imaging-derived features. Furthermore, a simplified stratification model was developed to classify individuals into two homogenous groups (low risk and high risk) based on their anticipated rate of ventricular enlargement.ResultsThe predictive accuracy of the prognostic models substantially improved by integrating brain imaging features alongside genetic load, cognitive and motor biomarkers: a 24 % reduction in the cross-validated mean absolute error, yielding an error of 530 mm3/year. The stratification model had a cross-validated accuracy of 81 % in differentiating between moderate and fast progressors (precision = 83 %, recall = 80 %).ConclusionsThis study validated the effectiveness of machine learning in differentiating between low- and high-risk individuals based on the rate of ventricular enlargement. The models were exclusively trained using features from HD individuals, which offers a more disease-specific, simplified, and accurate approach for prognostic enrichment compared to relying on features extracted from healthy control groups, as done in previous studies. The proposed method has the potential to enhance clinical utility by: i) enabling more targeted recruitment of individuals for clinical trials, ii) improving post-hoc evaluation of individuals, and iii) ultimately leading to better outcomes for individuals through personalized treatment selection.

Dataset Information

Fiberoptic endoscopic evaluation of swallowing in early-to-advanced stage Huntington's disease.

Publications

Fiberoptic endoscopic evaluation of swallowing in early-to-advanced stage Huntington's disease.

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