Huntington's disease (HD) behaviors involve significant personality changes, mood swings (depression, irritability, anger), apathy (loss of motivation), impulsivity, obsessive-compulsive patterns, and sometimes psychosis, stemming from brain damage, leading to social withdrawal, aggression, poor judgment, and difficulty with daily tasks, with symptoms often appearing as early as middle age and worsening as the disease progresses.
The movement disorders associated with Huntington's are usually noticeable in involuntary movements, such as of the head, hands, arms, legs, and trunk, and also in tic-like muscle twitches such as blinking of the eyes or a contortion of the mouth. The dance-like gait is typical.
People with Huntington's disease also may not be able to control voluntary movements. This can have a greater impact than the involuntary movements caused by the disease. Having trouble with voluntary movements can affect a person's ability to work, perform daily activities, communicate and remain independent.
The disease attacks areas of the brain that help to control voluntary (intentional) movement, as well as other areas. People living with HD develop uncontrollable dance-like movements (chorea) and abnormal body postures, as well as problems with behavior, emotion, thinking, and personality.
With dominant diseases like Huntington's Disease (HD), it is usually pretty easy to figure out risks. Generally if one parent has it then each child has a 50% chance of having it too. And if neither parent has the disease, then odds are that none of the kids will either. Huntington's is a dominant genetic disease.
Early Huntington's disease symptoms are often subtle and can include behavioral challenges or a decline in school performance in children. Someone with HD might have difficulty thinking or experience mood changes, work challenges and strained relationships with others.
Being at risk
Every child conceived naturally to a parent who carries the Huntington's gene has a 50% chance of inheriting it. Although it is a rare disease, other people live with this risk and you are not alone. Living with the knowledge that you are at risk can be very worrying.
HD affects the whole brain, but certain areas are more vulnerable than others. Pictured above in blue is the striatum – an area deep in the brain that plays a key role in movement, mood, and behavior control. The striatum is the part of the brain that is most affected by HD.
Of all the psychiatric manifestations of HD, the executive dysfunction syndrome of HD, while difficult to define and characterize, may be the most common. Individuals with this syndrome may become apathetic, irritable, disinhibited, impulsive, obsessional, and perseverative.
The neuropsychiatric symptoms of Huntington's disease often mimics those of bipolar disorder, leading to frequent misdiagnosis. A comprehensive clinical evaluation that includes genetic testing, neuroimaging, and consideration of both neuropsychiatric and motor symptoms is crucial for accurate diagnosis.
Huntington's disease impairs the functioning of the brain, which can result in apathy, trouble organizing, impulsivity, irritability and anger, unawareness, disinhibition, preservation, and other psychiatric symptoms. These emotional and behavioral symptoms can further complicate the caregiver's role.
How can we help the person affected?
Eye symptoms associated with Huntington's disease include ocular motility problems, like different characteristics of saccades, pursuit and fixation abnormalities, as well as retinal thinning.
And for social and cultural as well as medical and scientific reasons, it played a far more important role in defining the discrete clinical entity that soon came to be known as 'Huntington's chorea' and by the late 1960s, as 'Huntington's disease'.
What are neurological symptoms that should never be ignored? Key symptoms to watch for include sudden severe headaches, unexplained numbness or tingling, vision changes, cognitive decline, muscle weakness, tremors, balance problems, speech difficulties, and seizures.
Early signs that you might be having a balance or walking problem are trips and stumbles, and difficulty maintaining balance when standing on one leg or while you are doing two or more things at the same time, such as walking and talking or walking while holding something like a laundry basket in your arms.
The hallmark symptom of Huntington's disease is chorea, uncontrollable and often painful involuntary movement. The cognitive and behavioral symptoms of dementia due to Huntington's include depression, memory problems, impaired judgment, problems with short-term memory, organizing, coping, and concentrating.
Persons with HD should engage in aerobic activities ideally for at least 150 minutes a week. Walking is a good aerobic exercise and still possible for the person in the middle stage of HD to perform. If available, stationary bikes are an excellent means of providing aerobic activity.
Huntington's disease brain changes lead to alterations in mood, especially depression, anxiety, and uncharacteristic anger and irritability. Another common symptom is obsessive-compulsive behavior, leading a person to repeat the same question or activity over and over.
People can start to show the symptoms of Huntington's disease at almost any age. Most will develop problems between the ages of 30 and 50. The condition gradually gets worse for around 10-25 years, until the person dies.
Medicines to control movement include tetrabenazine (Xenazine), deutetrabenazine (Austedo) and valbenazine (Ingrezza). They have been approved by the Food and Drug Administration to suppress involuntary jerking and writhing movements, known as chorea. Chorea can happen as a result of Huntington's disease.
At this stage, a person with Huntington's is no longer able to do their own personal care and domestic responsibilities, and will have difficulty with mobility, needing to be in a chair or bed most of the time. Swallowing may be difficult and there may be significant weight loss.
The 4-wheeled rollator has been shown to be the safest walking device for people with HD (1). The 4-wheeled walker produced safer and less variable gait when compared to other devices (1).
Speech changes are typically mild initially but can get worse over time. Speech can become slurred (if muscles in the face and tongue become weak) or lose its natural rhythm and sound 'jerky' (if you have difficulty coordinating your breathing with speech).
Choose soft, easy-to-chew and easy-to-swallow foods (aim for the consistency of porridge). Use plenty of sauces and gravies to help make main meals easier to swallow. Add plenty of custards, ice-cream and cream to desserts. Avoid hard foods such as nuts and lollies.