INFORMATION SHEET

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Huntington’s disease is a rare, inherited disease that causes the progressive breakdown (degeneration) of nerve cells in the brain. Huntington’s disease has a broad impact on a person’s functional abilities and usually results in movement, thinking (cognitive) and psychiatric disorders.
Huntington’s disease symptoms can develop at any time, but they often first appear when people are in their 30s or 40s. If the condition develops before age 20, it’s called juvenile Huntington’s disease. When Huntington’s develops early, symptoms are somewhat different and the disease may progress faster.

Medications are available to help manage the symptoms of Huntington’s disease, but treatments can’t prevent the physical, mental and behavioral decline associated with the condition.

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Symptoms

Huntington’s disease usually causes movement, cognitive and psychiatric disorders with a wide spectrum of signs and symptoms. Which symptoms appear first varies greatly from person to person. Some symptoms appear more dominant or have a greater effect on functional ability, but that can change throughout the course of the disease.

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Movement disorders

The movement disorders associated with Huntington’s disease can include both involuntary movement problems and impairments in voluntary movements, such as: involuntary jerking or writhing movements; muscle problems, such as rigidity or muscle contracture; slow or abnormal eye movements; impaired gait, posture and balance; difficulty with speech or swallowing. Impairments in voluntary movements may have a greater impact on a person’s ability to work, perform daily activities, communicate and remain independent.

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Cognitive disorders

Cognitive impairments often associated with Huntington’s disease include: difficulty organizing, prioritizing or focusing on tasks; lack of flexibility or the tendency to get stuck on a thought, behavior or action; lack of impulse control that can result in outbursts, acting without thinking and sexual promiscuity; lack of awareness of one’s own behaviors and abilities; slowness in processing thoughts or ”finding” words; difficulty in learning new information.

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Psychiatric disorders

The most common psychiatric disorder associated with Huntington’s disease is depression. This isn’t simply a reaction to receiving a diagnosis of Huntington’s disease. Instead, depression appears to occur because of injury to the brain and subsequent changes in brain function. Signs and symptoms may include: feelings of irritability, sadness or apathy; social withdrawal; insomnia; fatigue and loss of energy; frequent thoughts of death, dying or suicide.
Other common psychiatric disorders include: Obsessive-compulsive disorder, a condition marked by recurrent, intrusive thoughts and repetitive behaviors; mania, which can cause elevated mood, overactivity, impulsive behavior and inflated self-esteem; bipolar disorder, a condition with alternating episodes of depression and mania. In addition to the above disorders, weight loss is common in people with Huntington’s disease, especially as the disease progresses.

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Diagnosis

A preliminary diagnosis of Huntington’s disease is based primarily on answers to questions, a general physical exam, a review of family medical history, and neurological and psychiatric examinations. The doctor may order brain-imaging tests for assessing the structure or function of the brain. The imaging technologies may include MRI or CT scans that show detailed images of the brain.

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Treatment

There are no treatments which can prevent the physical, mental and behavioral decline associated with the condition. Current treatment strategies involve the use of various medications to treat specific symptoms such as abnormal movements and behaviors. Depression and suicide are more common among affected people, so caregivers should monitor for associated symptoms and seek help if necessary. As symptoms of the disease worsen, affected people need more assistance, supervision, and care.

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Prognosis

The duration of the disease (from onset until death) varies considerably, with an average of approximately 19 years. Most people with HD survive for 10-25 years after the onset of symptoms. The average age at death ranges from 51-57 years, but the range may be broader. Pneumonia and cardiovascular (heart) disease were the most common primary causes of death in one study.

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PATIENT CARE

Optimize Quality of Life

Caring for people with HD is both challenging and rewarding. At times, the lack of definitive treatments can be frustrating, but careful attention to the changing symptoms and good communication between professionals, family members, and affected individuals can contribute to the successful management of the disease. Because there are no treatments which can slow, halt, or reverse the course of the disease, the goals of treatment are to reduce the burden of symptoms, maximize function, and optimize quality of life.

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Tailor Treatment to the Individual

Symptoms of HD evolve over time as a person passes through different stages of the disease. Symptoms also vary from individual to individual, even within a family. For example, one person may develop a severe mood disorder, requiring multiple hospitalizations, but have little motor disability at the time. The individual’s brother may have debilitating motor symptoms at the same disease duration, but no mood disturbance at all. Thus, interventions need to be tailored to individual symptoms.

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Support Organizations

Support and advocacy groups can help the patient connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics.

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Helpful resource:
Huntington’s Disease Society of America: http://www.hdsa.org/

 

Sources:

• Huntington’s Disease Society of America

• National Institute of Neurological Disorders and Stroke

• Mayo Clinic

• US Department of Health and Human Services

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