Huntington’s Disease: Symptoms and Treatment

Overview

Huntington’s disease is an inherited disease of the nervous system. It gradually destroys the nerve cells of the brain. There is no cure for
the nerve damage resulting from Huntington’s disease; it will last forever.

Huntington’s disease impacts a person’s functional and mental abilities, and usually results in movement, cognitive and psychiatric disorders. However, treatments available now can’t prevent the decline of the body functions.

Huntington’s disease is a rare disorder. Based on Huntington’s disease statistics, it is estimated that it affects about 30,000 people in the United States alone.

Causes

Huntington’s disease is caused by an inherited defect in a single gene. A person with one parent who has an abnormal copy of the gene, has a 50 percent chance of inheriting the gene and developing the disease.

The genetic mutation responsible for Huntington’s disease is different from many other mutations. An area within the gene is copied too many times. The number of repeated copies tends to increase with each generation.

Symptoms

There are two types of Huntington’s disease: adult onset and early onset.

Adult Onset

Symptoms of adult onset usually begin when people are in their 30s or 40s.

Initial symptoms include:

• depression
• irritability
• hallucinations
• psychosis
• minor involuntary movements
• poor coordination and balance
• difficulty understanding new information
• trouble making decisions

Symptoms that come out later include:

• involuntary twitching movements, called chorea
• difficulty walking
• trouble swallowing and speaking
• confusion
• memory loss
• personality changes
• speech changes
• decline in cognitive abilities
• Muscle problems, such as rigidity or muscle contracture (dystonia)
• Slow or abnormal eye movements

Early Onset

Symptoms of early onset one usually start to appear in childhood or adolescence. Early-onset Huntington’s disease causes mental, emotional, and physical changes, such as:

• drooling
• clumsiness
• slurred speech
• slow movement
• frequent falling
• contracted and rigid muscles
• seizures
• contracted and rigid muscles
• tremors or slight involuntary movements
• sudden decline in school performance
• Loss of previously learned academic or physical skills

Diagnosis

There are several steps to diagnose a Huntington’s disease:

• Neurological tests

The neurologist will ask you questions and conduct relatively simple tests to check your corresponding motor, sensory and psychiatric symptoms.

• Psychiatric tests

Psychiatric tests check your coping skills, judging abilities, emotional state, and behavioral patterns, signs of disordered thinking and evidence of substance abuse.

• Brain imaging and function

The imaging technologies may include electroencephalogram (EEG) tests, magnetic resonance imaging (MRI) scans and computed tomography (CT) scans.

• Genetic tests

Genetic tests may also help you decide whether or not to have children.

Treatment

Medications

Medications can lessen some of your physical and psychiatric symptoms.

• Tetrabenazine (Xenazine), haloperidol (Haldol) and levetiracetam(Keppra) are examples of useful medications to treat movement disorders.
• Antidepressants, antipsychotic drugs and mood-stabilizing drugs can help to treat psychiatric disorders.

Therapies

• Physical therapy

It can help improve your coordination, balance, and flexibility.

• Occupational therapy

It can be used to evaluate your daily activities and help you to practice body movements, eat and drink, bath and get dressed with devices.

• Speech therapy

It may be able to help you speak clearly and deal with swallowing and eating problems.

• Psychotherapy

It can help you work through emotional and mental problems and develop your coping skills.

Keywords: Huntington’s disease.