International Guidelines for the Treatment of Huntington's Disease

Frontiers in Neurology

Bachoud-Lévi, A. C., Ferreira, J., et al. (2019).

Frontiers in Neurology, 10, 710.

This guideline from the European Huntington's Disease Network provides recommendations on the treatment of Huntington's disease. While guidance on a variety of interventions (e.g., pharmacological, surgical) is provided, this summary highlights recommendations within the scope of speech-language pathology (e.g., swallowing, cognition, language and communication disorders, social communication impairments).

European Huntington’s Disease Network (Germany); National Reference Center for Huntington’s Disease (French Ministry of Health); NeurATRIS (France)

European Reference Network for Rare Neurological Diseases

Education and counseling on social cognition impairment in individuals with Huntington’s disease should be provided to in individuals with Huntington’s disease, family members and healthcare professions to facilitate social interaction and engagement (Professional Agreement).

For individuals with Huntington's disease who present with disturbing chorea, protective measures should be established to prevent injury or choking during meal time or other activities of daily living. Appropriate assistive technology devices and positioning techniques should be identified by rehabilitation specialists, including speech-language pathologists (Professional Agreement).

Cognitive rehabilitation strategies should be provided to individuals with Huntington’s disease to improve or maintain cognitive functions (e.g., executive functions, memory) during disease progression (Grade B Evidence).

Education and counseling pertaining to swallowing disorders should be provided to individuals with Huntington's disease. Information regarding food textures and diet modification, bolus size and placement, safe swallowing procedures, and environmental modifications should be discussed to decrease risk of aspiration ad improve swallowing function. Family and caregivers should be involved in education and counseling, especially as the disease progresses (Professional Agreement).

Oropharyngeal exercises to modify the temporal events of the swallow (e.g., supraglottic swallow) may improve swallowing difficulties in individuals with Huntington’s disease (Professional Agreement).

Cognitive stimulation may be provided to improve aspects of executive functioning, such as planning and initiation, for individuals with Huntington’s disease (Grade C Evidence).

Use of videofluoroscopic swallowing assessment can be used to help visualize the swallowing process and identify appropriate compensatory techniques for individuals with Huntington's disease (Grade C Evidence).

Education and counseling should be provided to individuals with Huntington’s disease and their communication partners. Training should be provided on the facilitation of communication strategies (e.g., gestures, repeating and rephrasing, use of yes/no questions), environmental modifications (e.g., allowing time for communication, reduction of environmental distractions and noise) and treatment options (e.g., voice therapy, augmentative alternative communication; Professional Agreement).

Early referral to a speech-language pathologist is recommended to assess and manage communication problems in individuals with Huntington's disease at all stages of the disease (Grade C Evidence).

Referral to a speech-language pathologist is recommended when individuals with Huntington's disease start experiencing swallowing disorders (Grade C Evidence).

During the progression of the disease, communication should be monitored and reassessed to plan effective management strategies for individuals with Huntington's disease. Speech and language assessment should include assessment of orofacial movements, respiratory function, breath control and coordination, phonation, articulation, intelligibility, comprehension, and communication abilities. (Grade C Evidence).

Use of augmentative and alternative communication (AAC) is recommended to compensate for communication difficulties and increase participation in daily life in individuals with Huntington’s disease. AAC strategies should be implemented based on the individual’s motivation and capacity to learn (Grade C Evidence).

Some forms of oral nutrition may be preserved to maintain pleasure and socialization associated with meals for individuals with Huntington’s disease requiring tube feeding (Professional Agreement).

Routine swallowing assessment should be provided to individuals with Huntington's disease throughout the progression of the disease (Grade C Evidence).