Med Surg Case Study #24: Huntington Disease

Jared Kensington | 40 years old

Chief Complaint: Jared presents with involuntary choreiform movements and cognitive impairment.

Medical History:

• Positive family history of Huntington disease

• No other significant medical history reported

Open each expandable tab to get more information.

0900

Nurse's Note: Jared exhibits rapid, jerky, involuntary movements consistent with chorea. He appears agitated and demonstrates difficulty in maintaining attention during conversation. Family members report progressive decline in cognitive function over the past year.

Vital Signs:

• Blood pressure: 130/85 mmHg

• Heart rate: 90 bpm

• Respiratory rate: 18 breaths/min

• Temperature: 98.5°F (36.9°C)

Provider Orders:

• Perform neurological assessment.

• Order MRI of the brain.

• Start tetrabenazine (Xenazine) 25 mg orally twice daily for chorea.

1200

Nurse's Note: Jared undergoes MRI imaging, which reveals symmetrical striatal atrophy consistent with Huntington disease. He continues to exhibit choreiform movements and demonstrates impaired judgment during interactions.

Vital Signs

• Blood pressure: 128/82 mmHg

• Heart rate: 95 bpm

• Respiratory rate: 20 breaths/min

• Temperature: 98.6°F (37°C)
  

Provider Orders:

• Increase tetrabenazine (Xenazine) dose to 50 mg orally twice daily.

• Consult with neurology team for further management.

• Initiate referral to genetic counselor for family members.

1530

Nurse's Note: Jared's chorea shows partial response to tetrabenazine, with a decrease in the frequency and intensity of involuntary movements. However, he remains emotionally labile and demonstrates poor insight into his condition.

Vital Signs:

• Blood pressure: 125/80 mmHg

• Heart rate: 88 bpm

• Respiratory rate: 22 breaths/min

• Temperature: 98.7°F (37.1°C)
  

Provider Orders:

• Continue tetrabenazine (Xenazine) 50 mg orally twice daily.

• Prescribe selective serotonin reuptake inhibitor (SSRI) for control of psychiatric symptoms.

• Arrange for psychotherapy consultation to address anxiety and stress management.

Question 1: What are the characteristic symptoms of Huntington disease?

Question 2: Why is tetrabenazine prescribed for chorea in Huntington disease?

Question 3: What is the role of genetic counseling in Huntington disease?

Answers to Questions Below

ULTIMATE Med Surg Notebook

Buy Now

Answers

Question 1: What are the characteristic symptoms of Huntington disease?

Huntington disease is characterized by a triad of symptoms: motor dysfunction (including chorea), cognitive impairment, and behavioral changes such as apathy and emotional lability.

Question 2: Why is tetrabenazine prescribed for chorea in Huntington disease?

Tetrabenazine is the only medication approved by the FDA to treat the symptom of chorea in Huntington disease. It works by depleting presynaptic dopamine stores, thereby reducing excessive dopamine neurotransmission and alleviating choreiform movements.

Question 3:What is the role of genetic counseling in Huntington disease?

Genetic counseling is crucial in Huntington disease to provide information about the risk of inheritance and help individuals and their families make informed decisions about genetic testing, family planning, and coping strategies. It also addresses concerns related to employment and healthcare discrimination.

Med Surg Case Study