The Huntington Disease management service is co-led by Dr Niall Campbell, Consultant Liaison Psychiatrist and Dr Myles Connor, Consultant Neurologist.
Information
What is Huntington’s disease (HD)?
HD is a relatively rare genetic disorder most often diagnosed in middle age. It is inherited in an autosomal dominant pattern. It progresses slowly over years, with survival typically over 10 years from diagnosis, though this varies. People with HD are usually affected by varying combinations of;
psychiatric problems
commonly depression or apathy sometimes accompanied by self-harm, more rarely psychosis, aggression or other features
cognitive problems
often progressing to dementia in later disease.
Decision-making capacity may be affected in more advanced illness but not in all cases
movement disorder (usually chorea ie restless semi-purposeful movements)
and other physical difficulties such as imbalance, falls, dysarthria and dysphagia
Usually definite HD is diagnosed if there is a positive genetic test along with a compatible movement disorder (usually chorea).
Genetic testing may be carried out prior to symptoms developing (“predictive” testing) after review by the Clinical Genetics service.
Problems including psychiatric ill-health may not always be directly due to Huntington Disease
Treatment
Typically management approach, and medications used, overlap strongly with those used in other conditions. Depression is common and the risk of suicide should be borne in mind.
There is no treatment currently for HD itself. There is ongoing research. There are symptomatic therapies available, often with limited evidence.
Dr Niall Campbell, Consultant Liaison Psychiatrist and Dr Myles Connor, Consultant Neurologist, run a multidisciplinary HD clinic between 2 and 4 times a year depending on need. The HD clinic broadly focuses on providing and signposting holistic support for people with definite symptomatic HD, to maximise quality of life, and support independent living in the community.
Contacts;
We are happy to discuss cases where the best approach or route of referral is uncertain.
Who to refer, who not to refer, how to refer
Who to refer:
Adults with known Huntington Disease wishing ongoing input from Huntington Disease clinic.
Who not to refer:
There are a range of teams involved in supporting people with HD, often based outside Neurology. Sometimes a specific team/service may be better placed to support than the HD management clinic. The following is not exhaustive. Please be in touch to discuss specific situations as needed.
Predictive HD gene testing – in other words testing for people at risk of developing HD in the future, but not currently suspecting that they are affected.
Please refer directly to clinical genetics for age 18 and older.
Clinical genetics also may be appropriate for issues including pregnancy planning in affected families. Specialised approaches may be considered.
HD gene testing where symptomatic HD is suspected (usually in context of a movement disorder or neurological disability) – For instance suspected chorea in presence of family history, or other high index of suspicion.
Refer to neurology
If a test for Huntington Disease is carried out and is positive, patients will then usually be referred to Huntington Disease clinic for ongoing care.
Testing where it is not clear if patient is symptomatic or not of Huntington Disease (for example known pre-existing psychiatric ill-health and no clear movement disorder)
Please discuss as required. Where there is uncertainty, counselling and testing through clinical genetics may often be most appropriate.
Significant psychiatric issues developing in known Huntington’s Disease.
Most often, patients in this position will be known to the Huntington service and psychiatry team already involved.
If this is not the case consider direct psychiatry discussion or referral, usually through CMHT.
However please discuss if required.
Nursing home resident
Given the focus of the HD management clinic on holistic support to enable independent living in the community, and because of multidisciplinary support available in the Nursing Home environment, the HD clinic does not typically see people who are resident in Nursing Homes.
How to refer:
SCI gateway
Primary care management
Pharmacological and overall management of Huntington Disease overlaps substantially with management of other more common neurodegenerative conditions such as Alzheimers or vascular dementia. Many principles of management are similar.
Pharmacological management of psychiatric symptoms overlaps with management in non-HD cases.
Patients with Huntington disease should be monitored for signs of depression and can respond well to typical anti-depressants
Rapid clinical changes are not typical and often suggest intercurrent issues (including infection and medication).
Please see BMJ best practice for more detailed advice on specific symptoms and management
Borders Ref Help Toolkit
