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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 judgement, 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 that develop the disease from sporadic mutations in the chromosome 4. The effect of the faulty gene leads to the destruction of brain neurons.

Medical Author:
Medically Reviewed on 3/19/2019


Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.