Prepared By: Rozelle Mae Birador

Huntington’s DiseaseHuntington’s Disease or HD is a progressive or degenerative brain disorder or also known as a Neurodegenerative. Huntington’s slowly diminishes the ability think, walk, and talk. Chorea are jerking motions which is a developed symptom of HD. HD slowly ruins the nerves in your brain. As of the science right now Huntingtons is an incurable disease.

The Discovery of HD

HD was first described by Dr. George Huntington in 1872 as a chronic progressive neurodegenerative disorder affecting movement, cognitive function, and personality.

What causes it?Huntington’s is caused by an abnormality of

the 4th chromosome and HTT gene(makes huntingtin), and the gene mutates,making a defective protein. This flawed gene holds the blueprint for the defective version of the protein “huntingtin.” The Huntington gene defect also has 40 repeats of genetic code, unlike the normal gene that only has 17-20 repetitions.

What parts of the body are affected?

Huntington’s affects you neurons(brain cells), eyes, and muscles by destroying the interneuron,motor and sensory neurons.

Etiology and Pathophysiology

1. Inherited disorder, considered autosomal dominant

2. Progressive atrophy of the basal ganglia and some portions od the cerebral cortex

3. Appears during the middle adult years4. Onset usually occurs between the age of 30-

50, death usually follow 12 years after onset

Pathophysiology ContinuedmHtt will cause decaying of neurons in

various regions of the brain such as the basal ganglia, frontal and temporal lobe

Basal ganglia is responsible for motor function control, cognition, emotion, and learning

Sign and SymptomsSubjectiveMemory lossDisorientationEventual dementia

Sign and SymptomsObjectiveUncontrolled jerky movements of the extremities, trunk, face, or tongueDisorganized gaitUncontrolled periods of angerHesitant or explosive patterns of speechGrimacing facial movementsImpaired chewing and swallowingIncontinence

What are the Exams or Tests?

There exams or tests for Huntington’s Chorea are a test to see if you have the genetic mutation of gene HTT, and how many repeats are within the HTT gene.

Therapeutic Interventions1. Control of jerky movements with

phenothiazines, butyrophenones, and thioxanthenes

2. Reserpine may be used to decrease presynaptic dopamine and tetrabenzine to reduce dopaminergic transmission

3. Symptoms are treated as they occur

What are treatments for Chorea?Deep brain stimulation - A procedure in

which miniature electrodes that release pulses of electricity are implanted into the brain. This treatment may prove useful for reducing symptoms of uncontrollable movement known as chorea in patients with HD.

Atypical antipsychotic drugs also have been proven to help with Chorea

What is an appropriate diet?

An appropriate diet for Huntington’s Chorea is Avoid Tyramine foods (red-wine, aged cheese) Antioxidants (blueberries) Phytonutrients (Tomatoes, pink grapefruit, watermelon, guava) High Calorie Snacks Nutrient dense foods (Lobster)

Nursing care of Clients with Hungtington’s ChoreaAssessment

Neurologic status, noting uncontrolled movements and cognitive abilityFamily history of Hungtington’s chorea

Nursing care of Clients with Hungtington’s ChoreaAnalysis/ Nursing DiagnosesRisk for aspirationBody image disturbanceBowel incontinenceChronic confusionRisk for injuryAltered role performance

Planning/ ImplementationProvide emotional support for client and familyAllow client and family to express feelings about

progressive deterioration and ultimate deathEncourage family members to seek genetic

counselingModify environment to increase safetyAssess ability to swallow, provide nutritional

support as neededEncourage client to remain as active as possibleProvide respiratory support based on changing

needs of client (airway, suctioning, oxygenation

Evaluation/ OutcomesRemains free from complications of

immobilityModifies activities to prevent injuryVerbalizes feelings with family members and

other members of the health teamEncourages family members to seek genetic

Test/DiagnosisHead CT Scan/MRIPET(isotope) scan for the brainDNA testing for chromosome #4 defect