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Huntington's Disease

Checked: 23-10-2022 by Vicky Ryan Next Review: 23-10-2024

Overview

There is some useful information on Huntington's Disease on the patient.info website.

The Huntington's Disease Association also has some useful resources for professionals.

Local neurologists advise the following comprehensive guideline which gives advice on management of some of the manifestations of Huntington's (1):

International Guidelines for the Treatment of Huntington's Disease (frontiersin.org)

Medication for Huntington's Disease

Anti-psychotics such as Olanzapine, Sulpride and Risperidone are sometimes prescribed off label for chorea which is causing the patient distress or discomfort.They should only be prescribed on the advice of a neurologist.

Tetrabenazine is licensed as a treatment for HD and is an amber drug on the BNSSG formulary so should only be initiated by a neurologist (2).

Depression is a common symptom and can be treated with anti-depressants such as SSRI, SNRI or mirtazepine (3).

Advice

Advice and Guidance

There is a local neurology advice and guidance service available via eRS

Advice is also available from the National Huntington’s disease service at UCLH. This is a nationally recognised centre of excellence for families affected by Huntington’s disease. They have a nurse led Advice and Guidance service (A&G) available via e-RS to provide support to GPs.

Referral

Referrals to Neurology

Referrals can be made to neurology via eRS at NBT.

Referrals to Allied Services

Neurophysiotherapy - see the Neurophysiotherapy page - an outpatient service for patients who have any neurological condition, either with or without a formal diagnosis. It is for all adults over 16 and there are no restrictions on location boundaries.
Speech and Language Therapy - see the Speech & Language Therapy page.
Genetics - see the Genetics - general page.
Dietician - see the Nutrition and Dietetics page.
Palliative care - see the End of Life Care and Hospice pages.

Follow up

All patients with a diagnosis of Huntington's Disease are offered follow up in the NBT neurology clinic.

The annual review involves an assessment by a neurologist and a Huntington's Disease Association carer review. A pilot to include the SLT team in this review is also due to start in 2024.

Resources

(1) Huntington's Disease Association - National guidelines (hda.org.uk)

(2) BNSSG Formulary - Movement disorders (Remedy BNSSG ICB)

(3) Frontiers | International Guidelines for the Treatment of Huntington's Disease (frontiersin.org)

Efforts are made to ensure the accuracy and agreement of these guidelines, including any content uploaded, referred to or linked to from the system. However, BNSSG ICB cannot guarantee this. This guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, in accordance with the mental capacity act, and informed by the summary of product characteristics of any drugs they are considering. Practitioners are required to perform their duties in accordance with the law and their regulators and nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties.

Information provided through Remedy is continually updated so please be aware any printed copies may quickly become out of date.