Early Stage: In this stage patients can still perform most of their usual activities. They may still be working and may still be able to drive. Involuntary movements are mild and infrequent, speech is still clear, and dementia, if present at all, is mild.
Once motor symptoms appear, Huntington's is commonly divided into five stages of disease progression. Note that symptoms can vary, both from person to person and within any one individual throughout the course of the disease.
Lots of people at risk of Huntington's disease decide they'd rather not know until any symptoms appear. If you do want to know, ask your GP for a referral to a genetic counsellor. You'll have several appointments with the counsellor. It's only done once all the benefits and risks have been explained.
Stage 1: Preclinical Stage
In the Preclinical stage, an individual will start to develop mild symptoms, such as anxiety, unusual irritability, poor coordination, difficulty learning new things and issues with making decisions.
After Huntington's disease starts, a person's functional abilities gradually worsen over time. The rate of disease progression and duration varies. The time from the first symptoms to death is often about 10 to 30 years. Juvenile Huntington's disease usually results in death within 10 years after symptoms develop.
Familial prion disease may produce a diverse range of phenotypes, even within the same pedigree. It may resemble HD with prominent personality change, psychiatric symptoms and cognitive decline, chorea, rigidity, and dysarthria. Limb and truncal ataxia and seizures may be present.
There's currently no cure for Huntington's disease or any way to stop it getting worse.
Huntington's disease is a condition that stops parts of the brain working properly over time. It's passed on (inherited) from a person's parents. It gets gradually worse over time and is usually fatal after a period of up to 20 years.
Symptoms of Huntington's disease
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.
Average age of onset is about 40 years old, with a range from childhood to the eighth decade [2,3]. It has been suggested that older onset patients are more likely to present with motor manifestations, and younger patients with psychiatric/behavioral manifestations [4].
Medical imaging techniques, such as computerized tomography (CT) and magnetic resonance imaging (MRI) can reveal atrophy of the caudate nuclei, which is observed in the early stages of Huntington's disease.
Early signs and symptoms can include irritability, depression, small involuntary movements, poor coordination, and trouble learning new information or making decisions. Many people with Huntington disease develop involuntary jerking or twitching movements known as chorea.
Parkinson's disease and Huntington's disease are both model diseases. Parkinson's disease is the most common of several akinetic-rigid syndromes and Huntington's disease is only one of an ever growing number of trinucleotide repeat disorders.
Typically, HD progresses for 10 to 30 years. Most people with HD die from complications related to the disease. There is no cure for HD, and current medications can only relieve its symptoms, not slow or delay the progression. If Huntington's disease runs in your family, you may want to have genetic testing.
Huntington's disease has been around for a long time, but in 1872, George Huntington was the first to describe it entirely and observe how it affected families genetically. Despite his work, HD patients still tended to be misdiagnosed with something else. The most famous example is Woody Guthrie.
The disease gets worse over time. Early signs of HD can vary, but often include mild clumsiness or problems with balance or movement, cognitive or psychiatric symptoms (problems with thinking or emotion), and changes in behavior.
As the disease progresses, a variety of motor, emotional/behavioral, and cognitive symptoms are experienced, including unsteadiness, trouble holding onto things, trouble walking, changes in sleeping patterns, delusions and hallucinations, intellectual decline, and memory loss.
If you have Huntington disease, it is important to closely monitor you condition, and contact your healthcare provider if you notice any of the following symptoms: Increasing difficulty with motor tasks such as walking. Trouble swallowing. Mood changes, such as increasing symptoms of depression or feelings of suicide.
Mood and behavioral changes
Living with Huntington's can induce feelings of anxiety, depression, apathy, and frustration. Such emotions may occur as the result of the diagnosis, or develop over time as a consequence of the disease process in the brain.
Although stress does not play a direct role in the onset and development of Huntington´s disease (HD) itself, it does have an influence on the course of the disease.
Research studies have suggested that exercise is beneficial for reducing symptoms and maximizing function in persons with HD. Maintaining a healthy heart is important to the health of all persons, no matter their disability. Persons with HD should engage in aerobic activities ideally for at least 150 minutes a week.
While treatments exist to manage the symptoms of the disease, nothing has proven to be curative – yet.
In Australia it is estimated that over 1,800 people have Huntington's Disease and approximately 9,000 are at risk (Huntington's NSW ACT website, 2019) with a reported prevalence rate in Australia which ranges from 4.5 per 100,000 to 6.5 per 100,000 (Pringsheim et al., 2012).