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Huntington’s Disease (Huntington’s Chorea)

  • - Huntington's Disease
  • Jun 25, 2011
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  • Viewed: 9099
Tags: | dementia | huntington's chorea | huntington's disease | inheritable dominant disease |

Treatment of involuntary movements

Tetrabenazine (Xenazine) was developed specifically to reduce the severity of the jerky/involuntary movements occurring in Huntington’s disease. Other drugs are also used to help reduce involuntary movements -  these include neuroleptics and benzodiazepines. The rigidity experienced by some patients can be treated with antiparkinsonian drugs. 

Treatment of psychological effects

Selective serotonin reuptake inhibitors and the serotonin receptor antagonist mirtazapine can be used to treat depression, while atypical antipsychotic drugs can be taken to manage psychosis and behavioural problems.  Involvement with Huntington’s disease and other disease support groups and communities can also very helpful in managing the psychological effects of Huntington’s for both the affected individual and their family.  There is a National Huntington’s Disease Association and also various State-based networks.  Links to the State-based Huntington’s Disease Associations can be found on the National website. A website has also been established which specifically handles Juvenile Huntington’s.

Weight loss and eating difficulties due to swallowing difficulties and lack of other muscle coordination make management of nutrition important. Thickening agents can be added to liquids to make the fluids easier and safer to swallow and reduce the chance of fluids entering the lungs.  If eating becomes too dangerous or uncomfortable, a feeding tube can be used.

In combination with adding thickening agents to liquids, nutrition can be managed by feeding individuals soups and purees of fruits, vegetables and meats.  There are a number of recipe books available that are specifically tailored to individuals with feeding difficulties.  For tips, guidelines regarding fluid and calorie requirements and recipe ideas for maintaining adequate nutrition in individuals with swallowing diffculties, visit the following website: http://www.gicare.com/Diets/Dysphagia.aspx.

Often the behavioural changes that occur in Huntington’s individuals are the most worrying for the patient and their families.  These include lack of concentration, memory lapses, depression, mood swings and aggression. These behavioural changes together with the progressive loss of mobility and independence place a lot of pressure and stresses on the individual’s family. In the late stages of the disease, full-time nursing care is required. Most often the individual’s spouse provides the nursing care. The primary carers can often feel or become isolated due to having less contact with friends and society in general. Since people more peripheral to the disease do not understand the behavioural changes that occur in Huntington’s, the carer is often embarrassed or reluctant to socialise with their affected spouse. It is very important that the individuals responsible for care-giving receive breaks from their care-giving duties and manage the psychological burden the disease places on them. Tapping into support networks to ease the psychological burden and isolation, and specific equipment that can be used to ease the physical burden can help individuals cope with their care-giving role. 

Physical and speech therapy

Huntington’s diseasePhysical and speech therapy can be very useful in the management of Huntington’s disease and has been shown to maintain cognitive and motor function. Physical therapy is particularly important in helping the patient to adjust to their reducing motor control and instability. These include making postural changes, balance transfer, preventing falls, learning how to use walking aids and other assistance devices.

Speech therapists can assist with maintaining breathing capacity and control of swallowing and eating muscles. These can all help to maintain quality of life.   

Potential therapies for the future and getting involved with clinical trials

A number of compounds that have shown promise in animal studies and in pilot clinical trials are currently under investigation for the treatment of Huntington’s disease. To keep up to date with the latest developments with clinical trials, these websites might be useful:

  * European Huntington’s Disease Network
  * Huntington Study Group
  * Huntington’s Disease Society of America

Gene therapies to reduce the amount of mutant huntingtin protein in the brain have shown great promise in the laboratory setting. However, it is likely to take sometime before clinical trials of gene therapies are underway.

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Source: Neurological Rehabilitation and Movement Disorders.

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References


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