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Huntingtons disease (chorea)

Chorea, the Greek word meaning dance. It is described as involuntary movements of the body. Is a progressive neurodegenerative genetic disorder that affects muscle coordination.

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Symptoms are treated as they occur.

Nursing Interventions 1. 2. Provide emotional support for client and family. Allow client and family to express feelings about progressive deterioration and ultimate death. Encourage family members to seek genetic counseling. Modify environment to increase safety. Assess ability to swallow. Provide nutritional support as needed. Encourage client to remain as active as possible. Provide respiratory support based on changing needs of client such as airway, suctioning, oxygenation). Utilize community agencies to provide emotional support.

Etiology and Pathophysiology Memory loss Disorientation Eventual dementia Uncontrolled jerky movements of the extremities, trunk, face, or tongue. Disorganized gait Uncontrolled periods of anger Hesitant or explosive pattern of speech Grimacing facial movements Impaired chewing and swallowing Incontinence

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Complications Assessment 1. 2. Neurologic status, noting uncontrolled movements and cognitive ability. Family history of Huntingtons chorea. Weight loss Pneumonia Congestive heart failure Incapacitated Bed sore

Nursing Diagnoses 1. 2. 3. 4. 5. Risk for aspiration Body image disturbance Bowel incontinence Risk for injury Altered nutrition less than body requirements 6. Self-care deficit 7. Risk for impaired skin integrity 8. Impaired swallowing 9. Altered thought process 10. Impaired verbal communication Therapeutic Interventions 1. Control of jerky movements with phenothiazines, butyrophenones, and thioxanthenes. Reserpine may be used to decrease presynaptic dopamine and tetrabenzine to reduce dopaminergic transmission.

Parkinsons disease
Is a progressive neurologic disease affecting the brain centers responsible for control and regulation of movement. A deficiency of dopamine due to degenerative changes in the substantia nigra produces tremor, bradykinesia, rigidity, and autonomic dysfunction. The cause is not known. Complications of Parkinsons disease include dementia, aspiration, and injury from falls. The incidence of Parkinsons disease increases with age; approximately 1% of the population older than age 60 has this disorder.

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Assessment 1. Characteristic resting tremor of the extremities (may be worse on one side), and possibly affecting the head and neck. Bradykinesia (slowness of movement). Muscle rigidity in performing all movements, as well as rest. Verbal fluency may be impaired. Signs of autonomic dysfunction (sleeplessness, salivation, sweating, orthostatic hypotension). Depression, dementia. Masklike facies. Poor balance, gait disturbances, speech problems.

inhibitors, as adjunct therapy in combination with levodopa therapy; COMT is an enzyme that eliminates dopamine from the brain. Surgical Interventions 1. 2. Medical pallidotomy to improve dyskinesia, rigidity, and tremor. Chronic deep brain stimulation through electrodes implanted into the thalamus or globus pallidus to decrease tremor. Brain tissue transplants through the use of stem cells and genetically engineered animal cells are a promising area of research.

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Nursing Intervention Diagnostic Evaluation 1. 1. Diagnosis is based on observation of clinical symptoms and consideration of patients age and history, confirmed by favorable response to levodopa therapy. CT scanning and MRI may be performed to rule out other disorder. Monitor drug treatment to note adverse reactions and allow for dosage adjustments. Monitor for liver function changes and anemia during drug therapy. 2. Monitor the patients nutritional intake and check weight regularly. 3. Monitor the patients ability to perform activities of daily living. 4. To improve mobility, encourage the patient to participate in daily exercise, such as walking, riding stationary bike, swimming, or gardening. 5. Advise the patient to perform stretching and postural exercises as outlined by a physical therapist. 6. Teach the patient walking techniques to offset parkinsonian shuffling gait and tendency to lean forward. 7. Encourage the patient to take warm baths and massage muscles to help relax muscles. 8. Instruct the patient to rest often to avoid fatigue and frustration. 9. To improve the patients nutritional status, teach the patient to think through the sequence of swallowing. 10. Urge the patient to make a conscious effort to control accumulation of saliva (drooling) by holding head upright and swallowing periodically. Be alert for aspiration hazard. 11. Have the patient use secure, stabilized dishes and eating utensils.

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Pharmacologic Interventions 1. Various drugs can be used, often in combination to prolong effectiveness because tolerance develops. o Anticholinergics to reduce activation of cholinergic pathways, which are thought to be overactive in dopamine deficiency. o Amantadine, which may improve dopamine release in the brain. o Levodopa, a dopamine precursor, combined with carbidopa, a decarboxylase inhibitor, to inhibit destruction of L-dopa in the bloodstream, making more available to the brain. o Bromocriptine, a dopaminergic agonist that activates dopamine receptors in the brain. o Monoamine oxidase inhibitors as adjunct to levodopa therapy. o Catecholamine-Omethyltransferase (COMT)

12. Suggest the patient eat smaller meals and additional snacks. 13. To prevent constipation, encourage patient to consume foods containing moderate fiber content (whole grains, fruits, and vegetables), and to increase his or her water intake. 14. Obtained a raised toilet seat to help the patient sit and stand. 15. Teach the patient facial exercises and breathing methods to obtain appropriate pronunciation, volume, and intonation. 16. Teach the patient about the medication regimen and adverse reaction.

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