Management of dysphagia in Huntington’s disease: a descriptive review

Pizzorni, Nicole; Pirola, Francesca; Ciammola, Andrea; Schindler, Antonio

doi:10.1007/s10072-020-04265-0

Cited by 9 publications

(11 citation statements)

References 43 publications

(62 reference statements)

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“…Besides chorea, the hallmark motor symptom in HD, other typical motor disorders include dystonia, incoordination, Parkinsonism, and ideomotor apraxia. When these heterogeneous movement disorders involve the oropharyngeal musculature, swallowing difficulties ensue 3 .…”

Section: Introductionmentioning

confidence: 99%

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

Schindler

Pizzorni

Sassone

et al. 2020

Sci Rep

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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.

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Section: Introductionmentioning

confidence: 99%

“…Dysphagia in HD has been investigated by subjective and objective swallowing evaluation tests and described in case reports and case series 1 , 3 – 6 . The Huntington’s Disease Dysphagia Scale (HDDS), a self-report questionnaire specifically designed to assess swallowing in HD, has demonstrated good construct.…”

Section: Introductionmentioning

confidence: 99%

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

Schindler

Pizzorni

Sassone

et al. 2020

Sci Rep

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“…'Involvement of speech-language therapist', 'Supervision when eating or drinking' and 'Adapted consistencies of food and drinks' were the measures most frequently used to manage dysphagia in this study; in most patients, a combination was applied. On the basis of a review of the scarce literature about management of dysphagia in HD, compensation strategies are recommended; also, pharmacological and rehabilitation treatments should be further investigated (36). No information was found about treatments to cope with FoC.…”

Section: Discussionmentioning

confidence: 99%

Dysphagia, Fear of Choking and Preventive Measures in Patients with Huntington's Disease: The Perspectives of Patients and Caregivers in Long-Term Care

Kalkers

Schols

Zwet

et al. 2022

The Journal of nutrition, health and aging

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Objectives To explore the prevalence of dysphagia and fear of choking in patients with Huntington’s disease (HD) as well as preventive measures, both those applied and those not included in managing dysphagia. Also, to investigate related problems encountered by their formal and informal caregivers. Design A multi-center observational cross-sectional study Setting and Participants 158 HD patients, recruited from six Dutch nursing homes specialized in HD, and their formal and informal caregivers Measurements Patients were assessed by means of questionnaires enquiring about dysphagia, fear of choking and measures to manage dysphagia. Also, questionnaires were administered about awareness of dysphagia symptoms, cognition and anxiety. Because we expected individuals with greater care dependency to have a higher severity of dysphagia, we distinguished between a care-independent and a care-dependent group of HD patients. Results In the total group, 90.5% of HD patients had one or more dysphagia symptoms. The prevalence of FoC in HD patients and the formal and informal caregivers’ fears about choking in HD patients was 45.7%, 19.0% and 59.5%, respectively, for care-independent patients and 58.7%, 50.1% and 77.5% for care-dependent patients. The score on the Huntington’s Disease Dysphagia Scale was a predictor for fear of FoC in care-independent patients. Speech-language therapy, supervision during eating and drinking and adaptation of food and drink consistency were the most frequently applied measures to manage dysphagia, a combination was used in most HD patients. Conclusions In HD patients, the prevalence of dysphagia is high and fear of choking is common among both patients and caregivers. A more severe degree of dysphagia is a predictor of FoC in care-independent HD patients. A combination of measures was used to manage dysphagia in most HD patients.

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“…Dysphagia symptoms in individuals with HD can be hyperkinetic or akinetic-rigid motor symptoms, varying according to the stage of HD [15,30]. Involuntary movements observed in HD can complicate the eating and drinking process by making postural control challenging, increasing the risk of aspiration [15,31]. Rapid lingual chorea and lingual dysfunction associated with swallowing coordination issues result in problems with bolus preparation and delivery [15,32,33].…”

Section: Prevalence Pathophysiology and Symptoms-findings Of Huntingt...mentioning

confidence: 99%

Dysphagia in Indivuduals with Huntington's Disease: A Narrative Review

Sapmaz Atalar,

Oğuz,

Genç

2024

Eur J Ther

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Huntington's disease (HD) is a neurodegenerative autosomal dominant condition characterized by motor, behavioral, and cognitive symptoms. Aspiration pneumonia stands out as a leading cause of death in HD, primarily attributed to dysphagia, which gets more noticeable as the disease progresses. Dysphagia symptoms in individuals with HD are compounded by noticeable movement problems, including Chorean or rigid-bradykinetic patterns. These symptoms manifest in every phase of swallowing and fluctuate with the progression of HD. Lingual chorea, delayed swallowing initiation, and impaired swallowing-respiratory coordination are key indicators of dysphagia in HD individuals. The negative impact on eating behaviors is further exacerbated by concurrent cognitive and sensory deficits. Consequently, dysphagia leads to social isolation, restrictions on activities and involvement, and a diminished quality of life for individuals with HD. To minimize these adverse effects, a referral to a speech-language therapist (SLT) for swallowing assessment should be initiated immediately upon the diagnosis of HD by a neurologist. Starting from the earliest stages of the disease, both clinical and instrumental swallowing assessments should be employed to minimize the detrimental consequences of dysphagia. Depending on the assessment results, compensatory and/or rehabilitative (restitutive) strategies can be recommended for treatment. Furthermore, the SLT actively collaborates with other team members, including individuals with HD, caregivers, neurologists, otolaryngologists, gastroenterologists, and others, contributing collectively to the decision-making process regarding both oral and non-oral feeding considerations. Despite negative impact of dysphagia on individuals with HD and its significant role in individuals’ deterioration, the evidence for specific dysphagia interventions remains limited. Clinicians, therefore, rely on well-established general swallowing therapy practices. There is a pressing need for evidence-based research on dysphagia in HD. In this study, the literature on dysphagia in HD will be examined, with a focus on its pathophysiology and the role of SLT in diagnostic and intervention techniques.

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Management of dysphagia in Huntington’s disease: a descriptive review

Cited by 9 publications

References 43 publications

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

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

Dysphagia, Fear of Choking and Preventive Measures in Patients with Huntington's Disease: The Perspectives of Patients and Caregivers in Long-Term Care

Dysphagia in Indivuduals with Huntington's Disease: A Narrative Review

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