The oral manifestations of Huntington's disease: A systematic review of prevalence

Munhoz, Luciana; Jabbar, None Ashjan Qasim; Filho, William José e Silva; Nagai, Aline Yukari; Arita, Emiko Saito

doi:10.1111/odi.14076

Cited by 3 publications

(5 citation statements)

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“…Dysphagia has been reported more frequently in HD patients than in controls 86 (Table 1), and is a leading cause of hospitalization in HD. 87 A strong correlation was also observed between disease progression and dysphagia severity on fiberoptic endoscopic evaluation of swallowing.…”

Section: Gastrointestinal Disorders Dysphagiamentioning

confidence: 99%

Systemic Symptoms in Huntington's Disease: A Comprehensive Review

Mehanna,

Jankovic

2024

Movement Disord Clin Pract

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BackgroundAlthough Huntington's disease (HD) is usually thought of as a triad of motor, cognitive, and psychiatric symptoms, there is growing appreciation of HD as a systemic illness affecting the entire body.ObjectivesThis review aims to draw attention to these systemic non‐motor symptoms in HD.MethodsWe identified relevant studies published in English by searching MEDLINE (from 1966 to September 2023), using the following subject headings: Huntington disease, autonomic, systemic, cardiovascular, respiratory, gastrointestinal, urinary, sexual and cutaneous, and additional specific symptoms.ResultsData from 123 articles were critically reviewed with focus on systemic features associated with HD, such as cardiovascular, respiratory, gastrointestinal, urinary, sexual and sweating.ConclusionThis systematic review draws attention to a variety of systemic and autonomic co‐morbidities in patients with HD. Not all of them correlate with the severity of the primary HD symptoms or CAG repeats. More research is needed to better understand the pathophysiology and treatment of systemic and autonomic dysfunction in HD.

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Section: Gastrointestinal Disorders Dysphagiamentioning

confidence: 99%

Systemic Symptoms in Huntington's Disease: A Comprehensive Review

Mehanna,

Jankovic

2024

Movement Disord Clin Pract

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“…HD include gum inflammation, tooth decay, poor oral hygiene, and bruxism in a recent review [39]. Additionally, many medications used for psychiatric symptoms induce dry mouth [40].…”

Section: Common Oral Health Issues Observed Among Individuals Withmentioning

confidence: 99%

“…Additionally, many medications used for psychiatric symptoms induce dry mouth [40]. Early referral to a dentist is essential for maintaining oral health, as dental procedures become more challenging as the disease progresses [39].…”

Section: Common Oral Health Issues Observed Among Individuals Withmentioning

confidence: 99%

“…Until advanced stages, oral feeding is generally feasible in HD.Even in advanced stages, efforts can be made to continue diet modifications, compensatory, and rehabilitative swallowing maneuvers as much as possible. Additionally, maintaining oral hygiene and ensuring that caregivers possess knowledge about safe nutrition for individuals are emphasized[19,39].Medical interventions encompass pharmacological treatmentsand non-oral nutrition alternatives. Pharmacological treatments (e.g., antipsychotics, valbenazine, and tetrabenazine) can be advantageous in managing motor and behavioral symptoms in HD.…”

mentioning

confidence: 99%

“…Generally, in scenarios where oral intake becomes problematic, and airway protection is uncertain, nutritional options like NG or PEG are discussed with the collaboration of other team members. Dysphagia intervention recommendations for individuals with HD are given in Figure3[19,27,39,[52][53][54].…”

mentioning

confidence: 99%

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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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What we don't need to prove but need to do in multidisciplinary treatment and care in Huntington's disease: a position paper

Mühlbäck¹,

Walsem²,

Nance³

et al. 2023

Orphanet J Rare Dis

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Background Huntington’s disease is a complex neurodegenerative hereditary disease with symptoms in all domains of a person’s functioning. It begins after a healthy start in life and leads through the relentless progression over many years to complete care dependency and finally death. To date, the disease is incurable. The long progressive complex nature of the disease demands multiple disciplines for treatment and care of patient and family. These health care providers need inter- and multidisciplinary collaboration to persevere and be efficacious in this devastating disease trajectory. Discussion The position paper outlines current knowledge and experience alongside the experience and consensus of a recognised group of HD multidisciplinary experts. Additionally the patient’s voice is clear and calls for health care providers with a holistic view on patient and family. Building long-term trust is a cornerstone of the network around the patient. This paper describes a managed care network comprising all the needed professionals and services. In the health care system, the role of a central coordinator or case manager is of key importance but lacks an appropriate guideline. Other disciplines currently without guidelines are general practitioners, nurses, psychologists, and social workers. Guidelines for neurologists, psychiatrists, geneticists, occupational therapists, speech and language therapists, physiotherapists, dieticians, and dentists are being discussed. Apart from all these profession-specific guidelines, distinctive inter- and multidisciplinary collaboration requirements must be met. Conclusions and recommendations The complex nature of Huntington's disease demands multidisciplinary treatment and care endorsed by international regulations and the lay association. Available guidelines as reviewed in this paper should be used, made available by a central body, and updated every 3–5 years. Time needs to be invested in developing missing guidelines but the lack of this ‘proof’ should not prevent the ‘doing’ of good care.

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The oral manifestations of Huntington's disease: A systematic review of prevalence

Cited by 3 publications

References 48 publications

Systemic Symptoms in Huntington's Disease: A Comprehensive Review

Systemic Symptoms in Huntington's Disease: A Comprehensive Review

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

What we don't need to prove but need to do in multidisciplinary treatment and care in Huntington's disease: a position paper

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