An Incomplete Guide

What is Huntington's Disease Dementia (HDD)?

Huntington’s Disease (HD) is not as common as other types of dementia, but it significantly impacts those affected. Accounting for less than 1% of dementia cases, it is most known for its early onset and severe motor symptoms. Let’s dive into what Huntington’s Disease Dementia is, how it affects people, and why it is almost always inherited.

What is Huntington’s Disease Dementia (HDD)?

What is Huntington’s Disease Dementia?

Huntington’s Disease Dementia is a neurodegenerative disorder caused by a mutation in the HTT gene, which affects both movement and cognitive functions. While rare, it is well-known for causing movement disorders like stiffness, slurred speech, and problems with balance. Later stages of the disease can also affect cognitive abilities, including attention, memory, and executive function.

Symptoms of Huntington’s Disease Dementia

Huntington’s symptoms typically begin in a person’s 30s or 40s, which is much earlier than most dementias. Early signs usually involve problems with movement, but as the disease progresses, cognitive decline becomes more apparent. Here are some common symptoms:

  • Movement Disorders: Stiffness in the arms or legs, difficulty with balance, and frequent falls.
  • Slurred Speech: Difficulty forming words clearly due to motor dysfunction.
  • Swallowing Problems: Difficulty swallowing, especially in later stages.
  • Cognitive Decline: Issues with memory, attention, and executive functions (planning, decision-making) typically appear later.

When Do Symptoms Appear?

Symptoms of Huntington’s Disease can develop at any time but typically present in the patient’s 30s or 40s. This early onset makes it unique among other dementias, many of which tend to occur later in life. Unfortunately, because it develops early, it can significantly impact a person’s quality of life for many years.

Is Huntington’s Disease Inherited?

Yes, Huntington’s Disease is inherited and is caused by a mutation in the HTT gene. The mutation leads to an excessive repetition of DNA building blocks, which causes degeneration of neurons in the brain. If a parent has the disease, there is a 50% chance that it will be passed on to their children. Unlike other genetic conditions, Huntington’s does not skip generations—if a child does not inherit the mutation, the cycle stops.

Does Huntington’s Skip Generations?

There is a common misconception that Huntington’s Disease can skip generations. However, this is not the case. If the HTT gene mutation is not passed to the next generation, it cannot be passed to future generations, and the disease does not “reappear” later. Once the mutation is no longer present, it’s gone for good.

Wrapping Up…

Huntington’s Disease Dementia is a rare but severe form of dementia that affects both movement and cognitive abilities. Its early onset and genetic nature make it unique among dementias, and while there is no cure, early diagnosis and treatment can help manage symptoms and improve quality of life. If Huntington’s runs in your family, genetic testing may help assess your risk.

If Huntington’s Disease runs in your family or you’re noticing any symptoms, consult with a genetic counselor or cognitive health specialist for testing options. Stay informed and check back for more guides as we explore other types of dementia and cognitive health issues.

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