Huntington's Disease

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Signs and Symptoms

Huntington's disease produces three types of symptoms: movement, cognitive, and psychiatric. The sequence in which symptoms develop varies from person to person.

Movement

Uncontrolled movement, or tics, may develop in the fingers, feet, face, or trunk. This is the beginning stage of chorea—involuntary, rapid, ceaseless movement. Chorea can become more intense when the person is anxious or disturbed. Over time other symptoms, such as the following, emerge:

• Clumsiness
  
• Jaw clenching (bruxism)
  
• Loss of coordination and balance
  
• Slurred speech
  
• Swallowing and/or eating difficulty
  
• Uncontrolled continual muscular contractions (dystonia)
  
• Walking difficulty, stumbling, falling
  

Cognitive

Over time judgment, memory, and other cognitive functions begin to deteriorate into dementia. As Huntington's disease progresses, the ability to concentrate becomes more difficult. The person may have difficulty driving, keeping track of things, making decisions, answering questions, and may lose the ability to recognize familiar objects.

Psychiatric

Early psychiatric symptoms of Huntington's disease are subtle, varied, and easily overlooked or misinterpreted. Depression is the most common psychiatric symptom of Huntington's and often develops early in the course of the disease. Signs of depression include:

• Hostility/irritability
  
• Inability to take pleasure in life (anhedonia)
  
• Lack of energy
  

Some people develop manic-depression, or bipolar disorder, during the course of the disease.

A person with Huntington's also may exhibit psychotic behavior:

• Delusions
  
• Hallucinations
  
• Inappropriate behavior (e.g., unprovoked aggression)
  
• Paranoia
  

In late-onset disease (after age 50), the patient may suffer depression rather than experience sudden anger or irritability, and their memory, reasoning, and problem-solving skills may remain sharp.

Early signs of juvenile Huntington's disease often include subtle changes in handwriting and a rapid decline in school performance. The child may develop seemingly minor movement disorders, such as slowness, rigidity, tremor, or rapid muscle twitching. Other early signs of disease may include these changes:

• Behavioral changes
  
• Difficulty learning new things
  
• Speech difficulties
  

Children under the age of 15 may experience recurrent seizures and akinesia—muscle rigidity and stiffness. Children from 15 to 18 years of age tend to manifest the same symptoms of Huntington's disease as adults.

Complications

Lack of physical activity, dietary problems, and eating and swallowing problems can cause constipation, incontinence, and weight loss. Psychiatric and cognitive problems can lead to social isolation and deep depression.

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