Huntington`s disease

Codes

ICD-10: G10

ORPHA: 399

General information

Estimated occurrence

6-12:100,000 inhabitants.

Cause

Autosomal dominant inheritance. The disease is caused by a mutation in the gene on the short arm of chromosome 4.

General symptoms

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.

Synonyms

Huntington chorea, Juvenile Huntington disease

Orofacial/odontological symptoms

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.

Advice on follow-up and treatment

Continuous intensified preventive oral care is very important.
People with Huntington disease may need reminding about times for treatment.
A soft 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.

Sources

Sällsynta hälsotillstånd, Socialstyrelsen

Updated: 2019-11-12 14:48