Huntington's Disease and Dementia

Your Guide to Huntington’s Disease and Dementia

What to Know About Dementia and Huntington’s Disease

If you’ve observed signs of Huntington’s disease and Dementia in your loved one, learning more about this disease can help you cope and support them. Understanding the specifics can empower you to provide the proper assistance and create a Dementia care plan for their ongoing needs.

What is Huntington’s Disease?

Huntington’s disease, also known as Huntington’s chorea, is a hereditary neurological disorder affecting the brain’s nerve cells. It is characterized by progressive motor dysfunction, cognitive decline, and psychiatric symptoms.

Huntington’s disease is an autosomal dominant genetic disorder, meaning that if one parent carries the mutated gene, there is a 50% chance of each of their children inheriting the gene and developing the disease. Genetic testing can determine whether an individual carries the mutated gene, and counseling is often recommended for those with a family history of Huntington’s disease. 

Is Huntington’s Disease a type of Dementia?

Huntington’s disease is considered a type of dementia. It is specifically categorized as a neurodegenerative disorder that can lead to a form of dementia known as Huntington’s disease dementia or Huntington’s dementia.

Huntington’s disease is characterized by a progressive decline in cognitive function, including memory, reasoning, and judgment, which are typical features of dementia. However, Dementia and Huntington’s disease often occur in conjunction with significant motor symptoms, like involuntary movements (chorea), muscle rigidity, and difficulties with coordination and balance.

While Huntington’s disease primarily affects motor function, the cognitive and psychiatric symptoms become increasingly prominent as the disease progresses. These cognitive impairments can lead to significant functional impairment and impact a person’s daily life. Not all people with Huntington’s disease will develop Dementia, and the specific symptoms and their severity can vary among those affected.

Causes of Huntington’s Disease and Dementia

  • Huntington’s disease is caused by a genetic mutation in the HTT gene, which leads to the production of a mutated protein called huntingtin. This abnormal protein accumulates in the brain and damages nerve cells, particularly in the basal ganglia and cortex.

Symptoms of Huntington’s Disease with Dementia

Symptoms of Huntington’s disease usually develop in adulthood, between the ages of 30 and 50, although they can appear earlier or later. Common symptoms include:

  • – Involuntary jerking movements (chorea)
  • – Muscle rigidity
  • – Difficulty with coordination and balance
  • – Cognitive impairment
  • – Mood swings
  • – Psychiatric symptoms like depression and anxiety


As the disease progresses, people with Huntington’s disease with Dementia may experience significant disability and eventually require full-time care.

Diagnosis of Huntington’s Disease

The diagnosis of Huntington’s disease with Dementia includes a combination of steps including:

  • Clinical Evaluation: A neurologist or specialist assesses medical history and symptoms and conducts a physical examination.
  • Genetic Testing: A blood test confirms the presence of the mutated HTT gene, often with genetic counseling.
  • Cognitive Assessment: Neuropsychological tests measure cognitive function and memory.
  • Brain Imaging: MRI or PET scans detect brain abnormalities.
  • Motor Symptom Evaluation: Assessing motor symptoms like chorea, rigidity, and coordination issues.
  • Psychiatric Assessment: Evaluating mood swings, depression, anxiety, and personality changes.
  • Differential Diagnosis: Ruling out other conditions with similar symptoms.
  • Family History: Considering a positive family history of Huntington’s disease.


How Fast Does Huntington’s Disease Progress?

Huntington’s disease (HD) is a progressive neurodegenerative disorder, and the rate of progression can vary from person to person. Several factors influence the progression of HD, making it challenging to predict precisely how fast the disease will advance in an individual. Some of the key factors affecting the rate of progression include:

  • Age of Onset: HD can manifest at different ages. People with juvenile-onset HD tend to experience a more rapid progression of symptoms compared to those with adult-onset HD.
  • Genetic Factors: The specific genetic mutation responsible for HD can vary in its length, which can influence the age of onset and the rate of progression. Longer mutations are generally associated with earlier onset and a faster disease course.
  • Individual Variation: Each person’s experience with HD is unique. Some individuals may progress more slowly, while others may experience a more rapid decline in motor, cognitive, and psychiatric functions.
  • Medications and Treatment: Medications and therapies help manage certain symptoms of Huntington’s Dementia, potentially slowing down progression or improving the person’s quality of life.
  • Overall Health and Lifestyle: General health, nutrition, physical activity, and overall lifestyle choices can impact the progression of HD symptoms.


In general, Huntington’s Dementia progresses over several years to decades, with distinct stages of the disease. People with Huntington’s Dementia and their families should work closely with healthcare professionals, including neurologists to manage the disease’s progression effectively. Support services can help improve your loved one’s quality of life and provide valuable assistance throughout the disease. Participating in clinical trials and research studies can contribute to a better understanding of it and the development of potential treatments.

Huntington’s Dementia is an unpredictable disease, and the rate of progression can vary even among those with the same genetic mutation. Early diagnosis and intervention are crucial for providing the best possible care and support for those affected by this disease. 

Will all patients with Huntington’s Disease develop dementia?

Not all patients with Huntington’s Disease will develop Dementia. While cognitive impairments are a common symptom, the extent and timing of cognitive decline can vary significantly. Some people with the disease may experience mild cognitive changes, while others may develop more severe dementia over time. The progression and severity of cognitive symptoms can be influenced by genetics and the age of onset. While dementia is a frequent feature of this disease, it is not a guaranteed outcome for every patient.

Are there different stages of Huntington’s?

Huntington’s disease with Dementia typically progresses through several stages, which can vary in duration and intensity. These stages are:

Early Stage:

In the early stage of  Huntington’s disease with Dementia, people often experience subtle motor symptoms, such as mild chorea (involuntary jerking movements), along with mood swings and cognitive changes. Daily activities are generally still manageable, and many individuals may continue to work or engage in social activities.

Middle Stage:

The middle stage is marked by more pronounced motor symptoms, including increased chorea, muscle rigidity, and coordination difficulties. Cognitive impairments become more apparent, with difficulties in memory, attention, reasoning, and language skills. Daily functioning becomes increasingly challenging, and individuals may require assistance with various activities of daily living.

Late Stage:

The late stage of Huntington’s disease with Dementia is characterized by severe motor dysfunction, including significant chorea and muscle stiffness. Cognitive decline reaches a critical point, often resulting in profound memory loss and an inability to communicate effectively. People in this stage become non-ambulatory, and they may require full-time care and assistance with all aspects of daily life.

The progression of  Huntington’s disease with Dementia can vary from person to person, and some may experience a slower or faster decline through these stages. Additionally, managing symptoms and care needs become increasingly complex as the disease advances, emphasizing the importance of comprehensive and personalized care planning.

Treatment and Outcomes of Huntington’s Disease and Dementia

Currently, there is no cure for Huntington’s disease, but there are treatments available to manage its symptoms and provide support for affected individuals and their families.

Once the diagnosis of Huntington’s disease is confirmed, the healthcare team can provide appropriate medical management, symptom relief, and support for both the affected person and their family members. Early diagnosis and intervention can help improve their quality of life and facilitate care planning for those with Huntington’s disease and Dementia.

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