This is a degenerative disease that presents with a gradual onset of involuntary,
jerking movements (chorea) and a progressive decline in mental ability, resulting
in behavioral changes and dementia. The disease is transmitted genetically, as an
autosomal dominant trait located on chromosome 4. Family members of patients
can have genetic testing done to identify presence of the gene. The symptoms typ-
ically appear between the ages of 30 and 50 years.
PROGNOSIS
The patient may present with either abnormalities of movements or changes in
intellectual function. In time, both will be present. The mental status changes will
progress to dementia. The disease will prove to be fatal within 10 to 20 years from
the time of onset.
SIGNS AND SYMPTOMS
• Personality changes
• Irritability or moodiness
• Psychiatric disturbance
• Progressive dementia as disease causes further neurologic degeneration
• Restlessness or fidgeting due to dyskinesia
• Abnormal, jerking movements (chorea)
• Depression
TREATMENT
Huntington’s disease is progressive and while there is no cure for it, medications
can be used to control symptoms.
• Genetic counseling.
• Control dyskinesia and behavior with medication to block dopamine receptors:
• phenothiazines
• haloperidol
• reserpine.
NURSING DIAGNOSES
• Risk for injury
• Impaired physical mobility
• Ineffective health maintenance
NURSING INTERVENTION
• Provide basic needs for the patient, assist with ADLs as needed.
• Protect the patient from suicide attempts due to depression.
• Assist the patient with positioning for safety and comfort.
• Explain to the patient:
• Nature of disease.
• Genetic counseling available for family members of patients.
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