Huntington’sHuntington’s Disease Disease An OverviewAn Overview

Huntington’s Disease Association

Dee Boyd – Regional Care Advisor

Contents

• What is HD?• Statistics• Genetics• Symptoms

What is Huntington’s disease?

• A hereditary degenerative neuro-psychiatric disorder

• Symptoms of HD usually start between the ages of 30-50 years, although late onset and juvenile manifestation does occur

• The prognosis is usually between 15-20 years from onset of symptoms

• Currently no cure

Statistics

• 6,000 -10,000 – in the population of the UK• 1in 10,000

Genetics

• An inherited dominant genetic condition caused by a mutation on chromosome 4

• Each child of an affected parent has a 50:50 chance of inheriting the gene and therefore developing the disease at some stage

• Identified the gene in 1989 and a predictive test has been available from 1993

• Huntington’s disease affects the whole family• It can have an effect on generations• Sometimes a person who has the HD gene dies

before any symptoms appear. The gene may have been passed on without anyone realising it is in the family

• Anyone who inherits the gene will develop the disease unless they die before this happens

Implications

Physical problems

Cognitiveproblems

Emotional problems

These problems interact with each other

A Triad of problems with Huntington’s disease

Healthy vs. HD Brain

Typical Physical Changes

• Chorea - Often starts with fidgety movements • Might seem clumsy or stumble more than

usual • Over time movements tend to become more

noticeable• Voluntary movements also affected• Rigidity

Typical Physical Changes

• Speech might sound a bit slurred• Lack of control of volume • As the disease progresses, swallowing

problems are common• Weight Loss• Incontinence

Typical Cognitive Changes• Harder to plan and think ahead• Harder to switch from one task to another• Prefer a set routine, and want things done in a

certain way and immediately. Change to routine can cause anxiety or frustration

• Changes in behaviour may be due to medication, pain, temperature, visitors, staff, fatigue, processing skills, frustration and overload

Typical Cognitive Changes

• Lack of motivation – appear lazy• Memory and concentration problems• Inability to recognise they have a problem• Denial – or is it?• Reduced ability to read facial expression• Inability to adapt behaviour• Extra processing time is required. Longer to

receive an answer

Typical Emotional Changes

• Cognitive changes lead to frustration which in turn lead to temper outbursts

• Emotional changes often start with subtle changes to mood / behaviour

• Some people become aggressive, demanding, stubborn and self-centred.

• Depression is common, but very treatable

Typical Emotional Changes

• Again because of cognitive changes people can be impulsive or irrational, behaving in a disinhibited way or obsessive with things

• These may lead to social isolation

Points to remember

• Some people with Huntington’s disease can experience mental health issues

• People with HD are individuals and will differ in the extent and sequence with which they experience symptoms

• However, although people may move in and out of the stages, there are broad developments during the progress of the disease

Other considerations

• HD can make a person more difficult to live with

• Relationship changes• Not the person I married• Loss and bereavement

Salient Points

• Huntington’s Disease can be considered as a veil. Sometimes you can see through the veil

• The movement disorder is the most apparent• The mental health/behavioural aspects are

the most worrisome• The cognitive impairment is the most disabling• Huntington’s Disease affects every individual

in a unique way

Further Reading

• Huntington’s Disease Association Fact Sheets• A range of other books, leaflets and resources

available via the HDA Website and Head Office

Huntington’s Disease Association

• For more information contact Head Office

Suite 24Liverpool Science CentreI31 Mount PleasantLiverpool L3 5TF

Tel: 0151 331 5444

E mail: info@hda.org.uk

Web: www.hda.org.uk