Huntingtons Disease


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Huntingtons Disease

  1. 1. Huntington’s chorea / Disease (HD)The term chorea means constant, uncontrollable dance like motion.DefinitionIt is progressive, degenerative neurological disease characterized by constant abnormaluncontrolled movements, emotional disturbances and intellectual decline. These movements areknown as choreiform or choreoathetoid movements.Incidence More than 150,000 Americans have HD 50% risk for developing disease for relatives.Etiology Degeneration of nerve cells in Basal Ganglia Subtle change in the cortex and cerebellum Loss of Neurons Neurotransmitter including Gamma Amino Butyric Acid (GABA) Concentration of Dopamine & Nor epinephrine Causes Excessive movement  In Parkinson’s disease dopamine causes lack of movement.Clinical Manifestations 1. Early 2. LaterPrepared by Mrs. Siji Varghese Page 1
  2. 2. Early a. Personality changes; inability/ anger/ depression/ loss of interest/ apathetic/ passive/ negativistic/ suspicions b. Cognitive ability: making decision/ learning new information/ answering questions & remembering important information. c. Mild balance problem; difficulty in walking. d. Irritability/ argumentative/ impulsive/ erratic. e. Clumsiness/ moodiness f. Involuntary facial movements: grimacing, facial twitching. g. Weight loss due to energy expenditure from choreoathetoit movement. h. Antisocial behaviors.Later a. Sudden jerky, involuntary movement throughout the body, face, trunk, fingers and feet. b. Piano playing motion of fingers c. Problems with balance & co-ordination d. Rapid eye movements e. Halting or slurred speech f. Swallowing problems g. Dementia develops as the disease progress h. Seizures i. DepressionPoints 1. Person is constantly in motion. 2. C/F usually begin before 20’s 3. Rare in childhood 4. Men & women are equally affected 5. Develops very slowly and later involves all muscles 6. Death occurs about 10-30 yrs after S/s first appear (Usually middle age) 7. Progress of disease faster in younger people 8. Movements diminishes or disappear during sleep 9. If one parent has single faulty gene chances of that offspring will also have the defect 50% 10. In 2006, researchers discovered that cholesterol accumulates in the brain can cause HD which are deposited in proper locations with improper levels. 11. Juvenile form of disease a. 10% of patients have this form of disease b. Onsel of symptoms may occur when the patient is younger than 20 yrs c. Muscular rigidity and dystonia is most common 12. Few individuals develops HD after the age of 55 yrs 13. Sometimes symptoms arising from a typical presentation of HD usually do not develop until a person is aged 35 yrs or older. By the time of diagnosis, many patients already have had children and passed the gene to another generation.Prepared by Mrs. Siji Varghese Page 2
  3. 3. How is HD diagnosed?  Family history / Medical history  Performing physical examination  Psychiatric /Neurologic examination  Laboratory test: DNA analysis (highly reliable & confirmative)  Familial screening a. Genetic test (i) Pretest counseling and (ii) Post test support  Head CT Scan/ MRI/ PET- Position Emission Tomography  Unified HD rating scale of assess S/sComplications: Dementia, Pneumonia, TB, AIDS, accidents, Suicidal attempt (depression)TreatmentAntipsychotics Haloperidol (Dopamine Receptor Blocker’s) Clonazepam ClozapineAntidepressants Fluoxetine Ant anxiety Diazepam Sertraline Nortriptyline Mood Stabilizers LithiumDrug resistant depression may yield to Electroconvulsive therapy.Tetrabenazine (Xanazine) is the first medicine to be specifically approved by Food & DrugAdministrationNursing ManagementNursing Diagnosis 1. Impaired physical mobility related to sudden jerky and involuntary movement of the body. 2. Potential for injury related to imbalance and reduced co-ordination 3. Alteration in Nutrition less than the body requirement related to difficulty in chewing and swallowing 4. Self care defect related to motor disturbances (later stages) 5. Improved verbal communication related to involuntary facial movements and slurred speechExpected Outcomes 1. Improvement of mobility 2. Avoidance of injury 3. Attainment of independence in activities of living 4. Maintenance of satisfactory nutritional status 5. Achievement of communication 6. Adequate bowel elimination 7. Development of positive coping mechanism 8. Improvement of cognitive dysfunctionsPrepared by Mrs. Siji Varghese Page 3
  4. 4. Nursing Care 1. Exercises a. Maintain fitness b. Daily regimen helps feel good 7 active 2. Physical Therapy a. Helps to keep muscles stronger & flexible b. Maintain balance, lessen the risk of falling c. Aids for ambulation such as walking behind wheel chair 3. Environmental a. Pad on wheel chair & bed b. Walking belts c. Simple & light cloths d. Keep environment free of hazard or harmful objects e. Special padding on knee/ ankles while walking 4. Speech therapy a. Poor control of oral & respiratory muscles can make communication difficult b. Nurse can assist family to develop c. Signals such as raising hand / keeping the eyes opened or closed for yes or no responses d. Cards & printed words e. Simple & unstrained communication by nurse f. Repeat words whenever necessary 5. Nutrition a. Need 5000 calories/day b. Extra vitamin and supplements c. Cutting food into small pieces prevent chocking & can swallow easily d. Soft thin food e. NG feeding f. Avoids dairy products as it causes mucus secretion & risk of chocking g. Treat incontinence & constipation h. Measure to prevent dehydration especially in hot weatherWhat kind of care does an individual with HD?Dietician Genetic CounselorPsychologist NeurologistPsychiatrist Social workerExperiments / Treatments & New Research  Combination of certain Cancer & AIDS drugs:- Halted the process of HD in fructifies  Use of Stem Cells:- Transplant of stem cells  Oct 2008 researchers began clinical trial of new drug currently known as ACR 16 – Stabilizes level of dopamineReferences  Joyce M. Black – Medical & Surgical Nursing  Brunner & Suddarth – Text Book of Adult Health Nursing  Internet Web MedicinePrepared by Mrs. Siji Varghese Page 4