Huntington's Disease: Definition, Cause and Treatment

Definition and Cause

• Huntington's disease is genetically inherited as an autosomal dominant disease
• It damages the nervous system
• It is a progressive disease with no cure
• Huntington's disease is caused by a genetic mutation in the HTT gene, which codes for the huntingtin protein
• This involves the over-duplication of the CAG segment
• Physical symptoms include loss of coordination, difficulty swallowing, slurred speech, chorea, and ataxia

Treatment

• There is currently no cure for Huntington's disease
• Treatment and support can reduce some of the problems
• Medication includes Tetrabenazine and antidepressants
• A high-calorie diet helps prevent weight loss
• Therapy includes physiotherapy and occupational therapy
• Home environment modifications manage the effects of the disease
• Support is provided through working with the patient, family, and care providers

Dental Implications

• Maintaining independence and efficient oral hygiene is difficult
• HD causes restricted motor ability of the upper extremities and a lack of coordination

Home care

• Reliance on carers is important for performing oral hygiene as the disease progresses

Communication

• Communication difficulties make it challenging for a patient to express pain
• Patients are often anxious about accessing dental services due to a lack of understanding from professionals about their condition

Oral factors and nutrition

• The composition and volume of saliva can be affected by medication, leading to xerostomia
• This increases the risk of decay and oropharyngeal candidiasis

In-chair implications

• Patients with HD usually require the use of a wheelchair

Dental Treatment Modifications

Anesthesia and Sedation

• General anesthesia and sedation techniques are used
• Focus on management of involuntary movements

Frequent Recall Appointments

• Anticipation of disease progression and risk management are important

Shortened Appointment Durations

• Accommodating increased choreiform movements

Specialized Techniques

• Modified use of Dental Operating Microscope (DOM)

Collaboration with Healthcare Providers

• Interdisciplinary coordination is important

Treatment Planning

• Incorporate strategies for managing xerostomia and choreiform movements

Incorporation of Home Care Needs

• Provide education for caregivers, focusing on practical solutions
• Adaptation to limitations

Studies

• Endodontic Treatment Challenges (Bradford et al., 2004)
• Dental Care Access and Communication (Boyle et al., 2008)