Huntington's Disease

HUNTINGTON’S DISEASE

Kendall Strobel

Multiple Select

What do you know about Huntington's?

• It has a juvenile form as well.

• It affects the cognitive function.

• It cannot be cured or stopped by any treatment.

• It is rare.

Huntington’s Disease is a progressive brain disorder caused by a defective gene on chromosome 4. It begins when an individual is in their 30s or 40s. It impairs one’s movement, cognitive ability, and mental health.

Multiple Choice

What type of disease is Huntington’s?

• Bacterial

• Genetic

• Autoimmune

• Environmental

The nerve cells of specific regions of the brain gradually break down and die. The mutation occurs in a gene responsible for a protein called huntingtin. This causes cytosine, adenine, and guanine (CAG) to repeat over and over. 27 to 35 repetitions may not cause a person to develop the disease but they can be a carrier for future generations. Individuals with HD often have 36 or more repetitions.

Fill in the Blank

What is the first letter of each nucleotide base of the sequence that is repeated?

​cognitive

Most with HD will develop chorea, involuntary movements. More severe HD can cause impairment to voluntary movements as well which can affect an individuals speech, swallowing, ability to perform daily activities, and more.

​movement

Symptoms/Effects

​Mental health

It’s very common for cognitive function to be affected. An individual with HD may struggle with impulse control, focus, slowed processing of thoughts, difficulty learning, and lack of personal awareness of behavior and actions.

The damage to the brain and changes in function are linked to causing, most commonly, depression and other conditions such as OCD, mania, or bipolar disorder. Some may just experience fatigue, insomnia, social withdrawal, or irritability.

Multiple Select

Which of the following are symptoms of Huntington’s Disease?

• Insomnia

• Chorea

• Impaired speech

• Mania

Diagnosing Huntington’s

There are many tests that determine if an individual has HD. A combination of neurological examination, medical history, diagnostic imaging and genetic tests are used. Family history is the easiest to determine as a child is 50% likely to develop HD if a parent has HD. However, sporadic HD exists and occurs when an individual with HD has no family history of the disease. A direct genetic test is most effective and accurate. It only requires a blood sample in which DNA will be extracted from.

​(Ask Alex if you have any questions)

Fill in the Blank

What is the percent change a child has of receiving the gene mutation if one of their parents has HD? (00 to 99)

There is no cure or anything that can stop HD. However, there are methods to relieve symptoms. The drugs tetrabenazine and deuterabenazine can treat chorea. Hallucinations, delusions, and violent outbursts can be minimized with antipsychotic drugs. Depression and anxiety may also call for a prescription that can help.

Treatment

Multiple Choice

True or False: There is a cure for Huntington’s Disease.

• True

• False

A Nursing Assistant may need to help an individual with Huntington’s Disease by being supportive and encouraging, using KISS if the resident is having difficulty understanding, ensuring the individual is consuming small bites of food and is seated up to aid in swallowing, assisting with daily activities, and more.

Statistics

• Around 41,000 people in the United States are affected.

• After an individual experiences symptoms, they typically live for only 15 to 20 years. (due to choking or affects of immobility)

• https://www.ucsfhealth.org/conditions/huntingtons-disease

• https://www.mayoclinic.org/diseases-conditions/huntingtons-disease/symptoms-causes/syc-20356117

• https://www.ninds.nih.gov/health-information/disorders/huntingtons-disease