The Huntington's disease dysphagia scale

Heemskerk, Anne‐Wil; Verbist, Berit M.; Marinus, Johan; Heijnen, Bas J.; Sjögren, Elisabeth V.; Roos, Raymund A.C.

doi:10.1002/mds.25922

Cited by 17 publications

(15 citation statements)

References 21 publications

(42 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In fact, despite dysphagia is a frequent and potentially invalidating symptom in HD patients, its assessment is not included in the current clinical evaluation of this condition. A recent study stated the validity of a questionnaire for patients and caregivers in detecting this aspect of HD [13] . However, the cognitive decline and the behavioural disturbances characterising HD patients may reduce the reliability of a self-reported scale, and in fact, in that study, a low agreement between patients and caregivers emerged.…”

Section: Discussionmentioning

confidence: 99%

“…The DOSS, preceded by BSAS, indicated that dysphagia is a prevalent motor symptom of HD, specially correlated with global motor slowness and impairment of mouth-tongue motor control. The concordance with the HDDS questionnaire [13] may be further obtained, though the direct patient observation by SLTs should be provided in order to improve early diagnosis and treatment recommendations such as modification of diet. The major limitation of the study is its small group size, which is a frequent problem in rare disorders, while multicentre evaluations may confirm the significance of clinical correlations.…”

Section: Discussionmentioning

confidence: 99%

“…Recently, a patient-reported outcome measure, namely, the Huntington's Disease Dysphagia Scale (HDDS), was proposed in order to measure and to monitor the severity of dysphagia in all stages of the disease. This 11-item scale demonstrated reproducibility and construct validity, though such self-assessment tool would be limited by the patient's cognitive decline and caregiver subjective reports [13] .…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Dysphagia in Huntington's Disease: Correlation with Clinical Features

et al. 2015

View full text Add to dashboard Cite

Huntington's disease (HD) is an inherited neurodegenerative disorder characterised by motor impairment, cognitive decline and psychiatric disorders. Dysphagia is a pathologic condition that increases morbidity and mortality of the affected people. Our aim was to evaluate dysphagia in a group of HD patients in view of motor, cognitive and functional decline. Thirty-seven genetically confirmed HD patients were submitted to clinical evaluations of swallowing. Bedside Swallowing Assessment Scale (BSAS) was used. Dysphagia Outcome and Severity Scale (DOSS) was applied for a preliminary classification of swallowing difficulties. All patients were also evaluated by the Unified Huntington's Disease Rating Scale (UHDRS). A group of 39 controls comparable for sex and age were recruited for BSAS scores normalisation. The BSAS scores indicated that in our HD cohort, 32.4% presented relevant or severe dysphagia. The DOSS levels were significantly correlated with main clinical features, such as age, disease duration and motor impairment, with special regard to lingual protrusion ability, dysarthria and bradykinesia. The total functional capacity (TFC) and cognitive scales did not show significant correlation with DOSS levels. The results of clinical examination of swallowing indicated that dysphagia is a prevalent motor symptom of HD.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Dysphagia in Huntington's Disease: Correlation with Clinical Features

et al. 2015

View full text Add to dashboard Cite

show abstract

“…Worsening of neurological condition may cause an individual to become distracted and overwhelmed by mealtime activity. In 2014 Heemskerk et al developed a valid and reliable 11-item scale to measure the severity of dysphagia in HD [19]. This Swallowing Disturbance Questionnaire could be very helpful when making decision about referral to a speechlanguage therapist (SLT) and further assessment including clinical observation for signs of aspiration when different consistencies of food/liquids are consumed, or instrumental assessment like video fluoroscopy.…”

Section: Dysphagiamentioning

confidence: 99%

Huntington Disease – Principles and practice of nutritional management

Żukiewicz-Sobczak

Król

Wróblewska

et al. 2014

Neurologia i Neurochirurgia Polska

View full text Add to dashboard Cite

Huntington disease (HD) is a degenerative brain disease clinically manifested by the characteristic triad: physical symptoms including involuntary movements and poor coordination, cognitive changes with less ability to organize routine tasks, and some emotional and behavioral disturbances. For patients with HD, feeding is one of the problems they have to face. People with HD often have lower than average body weight and struggle with malnutrition. As a part of therapy, good nutrition is an intervention maintaining health and functional ability for maximally prolonged time. In the early stages of HD, small amounts of blenderized foods given orally are recommended. In more advanced stages, enteral nutrition is essential using gastric, or jejunal tubes for short term. Most severe cases require gastrostomy or gastrojejunostomy. Although enteral feeding is well tolerated by most of the patients, a number of complications may occur, including damage to the nose, pharynx, or esophagus, aspiration pneumonia, sinusitis, metabolic imbalances due to improper nutrient and fluid supply, adverse effects affecting gastrointestinal system, and refeeding syndrome.

show abstract

“…For example, a recent 11-item scale developed to assess dysphagia in HD [17] evaluates the presence or absence of dysphagia-related problems such as drooling, nasal regurgitation, coughing, and choking. However, this scale does not the affect that dysphagia has on quality of life.…”

mentioning

confidence: 99%

HDQLIFE: the development of two new computer adaptive tests for use in Huntington disease, Speech Difficulties, and Swallowing Difficulties

et al. 2016

View full text Add to dashboard Cite

Purpose Huntington disease (HD) is an autosomal dominant neurodegenerative disease which results in several progressive symptoms, including bulbar dysfunction (i.e., speech and swallowing difficulties). Although difficulties in speech and swallowing in HD have a negative impact on health-related quality of life, no patient-reported outcome measure exists to capture these difficulties that are specific to HD. Thus, we developed a new patient reported outcome measure for use in the Huntington Disease Health-Related Quality of Life (HDQLIFE) Measurement System that focused on the impact that difficulties with speech and swallowing have on HRQOL in HD. Methods Five hundred seven individuals with prodromal and/or manifest HD completed 47 newly developed items examining speech and swallowing difficulties. Unidimensional item pools were identified using exploratory and confirmatory factor analyses (EFA and CFA, respectively). Item response theory (IRT) was used to calibrate the final measures. Results EFA and CFA identified two separate unidimensional sets of items: Speech Difficulties (27 items) and Swallowing Difficulties (16 items). Items were calibrated separately for these two measures and resulted in item banks that can be administered as computer adaptive tests (CATs) and/or 6-item, static short forms. Reliability of both of these measures was supported through high correlations between the simulated CAT scores and the full item bank. Conclusions CATs and 6-item calibrated short forms were developed for HDQLIFE Speech Difficulties and HDQLIFE Swallowing Difficulties. These measures both demonstrate excellent psychometric properties, and may have clinical utility in other populations where speech and swallowing difficulties are prevalent.

show abstract

The Huntington's disease dysphagia scale

Abstract: We developed a valid and reliable 11-item scale to measure the severity of dysphagia in Huntington's disease.

Cited by 17 publications

References 21 publications

Dysphagia in Huntington's Disease: Correlation with Clinical Features

Dysphagia in Huntington's Disease: Correlation with Clinical Features

Huntington Disease – Principles and practice of nutritional management

HDQLIFE: the development of two new computer adaptive tests for use in Huntington disease, Speech Difficulties, and Swallowing Difficulties

Contact Info

Product

Resources

About