Huntington's Disease

By: Mackenzie Fraitzl

What is it?

Huntington's Disease is an inherited disease that causes the progressive breakdown of nerve cells in the brain. It has a broad impact on a person's functional abilities and usually results in movement, physical, and psychiatric disorders.

How is it inherited?

Huntington's Disease is caused by an inherited defect in a single gene. Huntington's Disease is an autosomal dominant disorder, which means that a person needs only one copy of the defective gene to develop this disorder.

What are the symptoms?

Can cause movement, cognitive, and psychiatric disorders.

Movement:

-Involuntary jerking or writhing movements

-Muscle problems

-Slow or abnormal eye movements

-Impaired gait, posture and balance

-Difficulty with the physical production of speech or swallowing

Cognitive:

-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

Psychiatric:

-Feelings or irritability, sadness or apathy

-Social withdrawal

-Insomnia

-Fatigue and loss of energy

-Frequent thoughts of death, dying or suicide

What are the treatments?

No treatment can alter the course of Huntington's disease, however, medications can lessen some of the symptoms of the disease. Multiple interventions can help a person adapt to changes in his/her abilities for a certain amount of time.

Medications to help movement disorders:

-Tetrabenazine

-Antipsychotic drugs

Medications to help psychiatric disorders:

-Antidepressants

-Antipsychotic drugs

-Mood-stabilizing drugs

Is there available help for families?

There are support services for people with Huntington's disease and their families. Nonprofit agencies such as the Huntington's Disease Society of America, provide caregiver education, referrals to outside service and support groups for people with the disease and caregivers. Local and state health or social service agencies may provide daytime care for people with the disease, meal assistance programs or respite for caregivers.

Who should be tested?

A genetic test can be given to someone who has a family history of the disease but shows no signs or symptoms. I think that only people who have a family history of the disease need to be tested.

What is the procedure for testing?

The neurologist will ask you questions and conduct relatively simple tests in the office to judge:

Motor Symptoms:

-Reflexes

-Muscle strength

-Muscle tone

-Coordination

-Balance

Sensory Symptoms:

-Sense of touch

-Vision and eye movement

-Hearing

Psychiatric Symptoms:

-Memory

-Reasoning

-Mental agility

-Language functioning

-Spatial reasoning