The Huntingtons Disease management clinic is hosted within Neurology. Other relevant clinical services are hosted outwith Neurology, and are briefly signposted below.
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).
- Problems including psychiatric ill-health may not always be directly due to Huntingtons 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.
See one useful treatment algorithm https://bestpractice.bmj.com/topics/en-gb/513 (BMJ Best Practice).
HD management clinic.
Peter Foley (Neurology consultant) runs a ~weekly Huntingtons management clinic, working closely with Huntingtons Disease Specialists who are employed by the Scottish Huntingtons Association (SHA) charity.
The HD management 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.
Scottish Huntington’s Lothian team
- ‘SHA Huntingtons Specialists’ have a community-based outreach support role with extensive experience of supporting people with HD, and will have previous experience in a health or social care role.
- SHA Youth workers may be placed to support family members ≤25 years old.
- SHA Financial wellbeing team (which can advise on benefits optimisation) is not currently operational.
Multidisciplinary team
We work closely with a variety of other teams including psychiatry and neurorehabilitation. Close psychiatry input may be particularly important for a few people with HD. Multidisciplinary team input may be important.
Detailed guidance on available multidisciplinary support in Lothian is available on the Lothian Care Framework for HD.
Contacts;
We are happy to discuss cases where the best approach or route of referral is uncertain
Lothian Huntington’s Disease Management service: Please email huntingtons@nhslothian.nhs.scot.uk (checked in office hours, weekdays).
M.A & P.F 16-08-24
Who to refer:
- Adults with known Huntington’s Disease wishing ongoing input from Huntington’s Disease management 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.
- Usually refer to neurology movement disorder clinic, or general neurology clinic.
- If a test for Huntington’s Disease is carried out and is positive, patients will then usually be referred to Huntington’s management clinic for ongoing care.
- Testing where it is not clear if patient is symptomatic or not of Huntingtons (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’s 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 management clinic does not typically see people who are resident in Nursing Homes.
How to refer: (e.g. SCI & which option etc)
Sci-gateway> Royal Infirmary> Neurology
Primary Care Management
(Information to help GP’s treat patient without hospital referral (where appropriate)
- Pharmacological and overall management of Huntingtons 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’s 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).
- Consider MDT input, falls risk mitigation and other general support
- Please see BMJ best practice for more detailed advice on specific symptoms and management
- Detailed multidisciplinary input may be appropriate particularly in complex or more advanced cases – see link to Lothian Care Framework above for multidisciplinary team contacts, and please discuss if required.
BMJ Best Practice-Huntington’s disease BMJ paper.pdf
Scottish Huntingtons Association- Lothian Care Framework Lothian Care Framework for HD.
