Reports from the MHC data base
- Huntington chorea
- Juvenile Huntington disease
6-12:100 000 inhabitants
Autosomal dominant inheritance. The disease is caused by a mutation in the gene on the short arm of chromosome 4. The gene controls the formation of a protein, huntingtin.
Huntington disease is a progressive neurological and neuropsychiatric disorder that entails motor function, cognitive and psychiatric symptoms. The first sign of the disease usually become noticeable between 30 and 50 years of age. An unusual juvenile form occurs which appears before the age of 20. The disease is characterised by difficulties with balance and random, jerky movements of arms and legs that increase in time. Complications from the respiratory organs are common.
Neurological symptoms often start with involuntary, scarcely noticeable movements of fingers, toes and tongue. There is a risk of bite damage to tongue and cheeks. Increased grimacing may occur. However, the facial expression is flattened as control of the muscles deteriorates. Oral motor difficulties affect the ability to clean the mouth. The impact on the oral motor function also affects the ability to speak, chew and swallow.
- Continuous intensified preventive oral care is very important
- People with Huntington disease may need reminding about times for treatment
- A soft plastic splint that protects against bite damage may be needed
- In the long term relatives and personal assistants need instruction on how to help with oral hygiene
- Dental treatment may be facilitated if the patient is helped to sit/lie in the dentist’s chair as relaxed as possible – for example on Tumle pillows
- Avoid large amounts of water if there is a risk of aspiration.
- The rare disease database of the Swedish National Board of Health and Welfare.
- The MHC database - The Mun-H-Center database of on oral health and orofacial function in rare diseases.
- Huntington disease – a practical guide on CD.