Symptoms and Signs of Huntington Disease Dementia

Doctor's Notes on Huntington Disease Dementia

Huntington disease (Huntington's disease or HD) is a familial genetic disorder that affects a person's ability to move, feel, and think. The person with one parent with HD has a 50% chance of developing HD, but it often begins in middle age although it can begin at almost any age with the problem that earlier the onset of symptoms, the more quickly the disease advances. Signs and symptoms vary widely from person to person, but mood swings are usually the first noticed. The mood swings may abate or develop into depression or even hostility. The person's judgment, memory, and concentration become faulty. In others, chorea (uncontrolled movements) may begin as clumsy movements that can progress to causing falls. As the disease progresses, a decline in the ability to speak, swallow, eat, and walk occurs and dementia develops.

The cause of HD is usually an inherited faulty chromosome 4 from one parent; each child of an HD parent will have a 50% chance of having the disease. There are a few patients who develop the disease from sporadic mutations in the chromosome 4. The effect of the faulty gene leads to the destruction of brain neurons.

What Are the Treatments for Huntington's Disease (HD)?

No treatments can alter this genetic disease. However, medications may reduce symptoms, although side effects may limit their use.

• Medications for movement disorders
  • Tetrabenazine
  • Deutetrabenazine
• Antipsychotic drugs
  • Haloperidol
  • Risperidone
  • Olanzapine
  • Quetiapine
• Medications for psychiatric disorders
  • Antidepressants like fluoxetine, sertraline
  • Antipsychotics like olanzapine, citalopram
  • Mood stabilizers like carbamazepine, divalproex

In addition, patients may benefit from several types of therapy such as

• psychotherapy,
• speech therapy, and
• physical therapy.

The needs of the patient change over the years. The medical team can help the patient adjust to those problems as they develop.