What is Huntington's Disease

What is Huntington's Disease

Huntington's disease (HD) is another rare, incurable brain disorder passed down in families through a defective gene. It’s a condition that causes irreversible dementia and affects the central part of the brain, causing a decline in a person's physical, emotional, and mental abilities.

Huntington’s disease can develop in children and young adults in their 20s. This condition is called juvenile HD (JHD). Nearly 10% develop the symptoms before they reach 20 years old and around 10% after 60. Individuals who develop the adult-onset of Huntington's disease live about 15 to 20 years after the symptoms emerge. Other sources mentioned that a person could live about 10 to 30 years.

What causes Huntington's Disease

Huntington's disease (HD) occurs when a child inherits a parent's defective gene. DNA is composed of thousands of genes, and a person with HD is known to have a small error in a gene called huntingtin. HD is an autosomal dominant disorder, which means a person only needs one copy of the faulty gene to acquire the condition. If a mother has this defective gene, there's a 50% chance that she'll pass the genetic disorder to her child. The child will also have a 50% chance of passing the disease, affecting several generations.

Symptoms

Since this condition is inherited, symptoms can emerge at any time. In many instances, the first signs appear in people in their 30s or 40s. But they can also surface as early as age 2 to 80.

Symptoms of HD can vary, and the disease progression may be slow or rapid. The most common symptoms are:

  • Difficulties with planning
  • Depression, mood swings, and social withdrawal
  • Personality changes
  • Impaired judgment and forgetfulness
  • Involuntary movements (chorea), such as involuntary jerking and muscle problems
  • Weight loss and balance issues
  • Slurred speech
  • Psychiatric disorders, such as obsessive-compulsive disorder and bipolar disorder

Symptoms for individuals with juvenile Huntington's disease (JHD) are slightly different. Early signs include:

  • A rapid drop in school performance
  • Contracted muscles that affect gait
  • Difficulty paying attention
  • Behavioral changes
  • Seizures
  • Tremors
  • Frequent falls

Unlike other types of dementia, a person with Huntington’s disease can understand language and recognize families and friends.

Treatment

There's no cure for Huntington's disease nor treatment that can reverse the disease progression. A combination of treatments, such as medications, physical and occupational therapy, and counseling can help manage the symptoms. Nutritionists and speech pathologists can assist with difficulty in eating and nutrition.

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