Huntington Disease Dementia Symptoms

Abnormal movements or emotional or behavioral changes are usually the earliest symptoms to appear in HD. Cognitive disturbances usually appear later in the course of the disease. Emotional, behavioral, and cognitive symptoms usually appear gradually and worsen over time.

The average life expectancy from onset of symptoms is about 15 years. Early onset of the disease, before age 20 years (juvenile HD), is linked to faster progression of symptoms and shorter life expectancy (approximately 8 years). Individuals who inherit the disease from their father tend to develop symptoms much earlier in life than those who inherit it from their mother.

Emotional and behavioral disturbances occur in 35-73% of people with HD.

• Change in affect: This is a common early symptom. “Affect” means the outward expression of thoughts, feelings, and moods. For example, a person who is normally cheerful may show little feeling at all.
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• Depression: Depression is the most common emotional disturbance in HD. Symptoms include sadness, tearfulness, lethargy, apathy, withdrawal, loss of interest in activities once enjoyed, insomnia or sleeping too much, and weight gain or loss.
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• Mania: Mania is a state of extreme excitement, restlessness, or hyperactivity with insomnia, rapid speech, impulsiveness, and/or poor judgment. Increased sexual activity or arousal is sometimes a symptom. This occurs in 2-12% of people with HD.
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• Eccentricity, inappropriateness, loss of social manners
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• Excess irritability
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• Obsessive-compulsive symptoms: Some people with HD develop obsessions (uncontrolled, irrational thoughts and beliefs) and/or compulsions (odd behaviors that must be carried out to control the thoughts and beliefs). They may become preoccupied with details, rules, or orderliness to such a degree that the larger goal is lost. Lack of flexibility or inability to change is common.
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• Psychosis: The psychotic person is unable to think clearly or realistically. Symptoms such as hallucinations, delusions (false beliefs not shared by others), and paranoia (suspicious and feeling of being under outside control) are common. The person’s behavior is strange, bizarre, agitated, aggressive, or even violent.
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• Suicide: About 6% of people with HD commit suicide. The worsening dementia often does not affect insight, and a person may become suicidal when faced with the burdens of his or her disease.

Cognitive symptoms include the following:

• Forgetfulness and disorganization - Common early symptoms
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• Loss of attention, poor concentration
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• Poor judgment
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• Problems with abstract thought, reasoning, or problem solving
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• Spatial disorientation
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• Difficulties with sequencing, organizing, or planning
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• Inability to learn new things
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• Inflexibility, problems adapting to change
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• Memory loss - Typically occurs later in the disease

Most people with HD retain their insight, factual knowledge, and intelligence quotient (IQ) score relatively well into the disease process. Use of language may or may not be impaired.

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