Movement disorder - HUNTINGTON'S DISEASE(Chorea)

Increased movement that can be voluntary (intentional) or involuntary (unintended) Decreased or slow voluntary movement.

1. MOVEMENT DISORDERS Mr.Sachin Dwivedi M.SC. Medical surgical Nursing K.G.M.U Institute of Nursing , Lucknow

2. MOVEMENT DISORDERS Movement disorders are neurologic conditions that cause problems with movement, such as Increased movement that can be voluntary (intentional) or involuntary ( unintended) Decreased or slow voluntary movement.

3. DEFINITION Movement disorders  are clinical syndromes with either an excess of movement or a paucity of voluntary and involuntary movements, unrelated to weakness or spasticity .

4. MOVEMENT DISORDERS Ataxia.( Impaired balance or coordination, ) Dystonia (Repetitive muscle contractions ) Huntington's disease , (Chorea) Parkinson's disease (Damaged Nerve cell ) Tourette syndrome , Tremor ( shaking movements  of body parts.)

5. CAUSES Genetics- Infections Damage to the brain, spinal cord, or peripheral nerves Metabolic disorders Stroke and vascular diseases Toxins .

6. HUNTINGTON'S DISEASE(Chorea) Huntington’s disease is a chronic, progressive, hereditary disease of the nervous system that results in progressive involuntary choreiform movement and dementia . Huntington's disease is an inherited disease that causes the progressive breakdown (degeneration) of nerve cells in the brain. 

7. FACTS HD is named after George Huntington, the physician who described it as hereditary chorea in 1872.   Characteristic features of HD include involuntary movements, dementia, and behavioral changes.

8. CAUSES The huntingtin gene defect involves extra repeats of one specific chemical code in one small section of chromosome 4. The normal huntingtin gene includes 17 to 20 repetitions of this code among its total of more than 3,100 codes. The defect that causes Huntington's disease includes 40 or more repeats. Genetic tests for Huntington's disease measure the number of repeats present in an individual's huntingtin protein gene.

9. PATHOPHYSIOLOGY

10. CLINICAL MANIFESTATION Neurologic manifestation: I nvoluntary jerking movements (chorea) Muscle problems, such as rigidity or muscle contracture (dystonia) Slow or abnormal eye movements Impaired gait, posture and balance Difficulty with production of speech or swallowing

11. COGNITIVE MANIFESTATION Difficulty organizing, prioritizing or focusing on tasks Lack of flexibility or the tendency to get stuck on a thought, behavior or action . Lack of awareness of one's own behaviors and abilities Slowness in processing thoughts or ''finding'' words Difficulty in learning new information

12. PSYCHIATRIC MANIFESTATION Most common - Depression Feelings of irritability, sadness or apathy Social withdrawal Insomnia Fatigue and loss of energy

13. DIAGNOSTIC EVALUATION

14. NON-PHARMACOLOGICAL MANAGEMENT Nutritional therapy Psychotherapy Speech therapy Physical therapy Occupational therapy

15. PHARMACOLOGICAL MANAGEMENT T etrabenazine ( reduces dopaminergic transmission). Antidepressants  include such drugs as citalopram ( Celexa ), Antipsychotic drugs : such as quetiapine (Seroquel), risperidone ( Risperdal) may suppress violent outbursts, agitation. A nticonvulsants , such as valproate ( Depacon ), carbamazepine ( Carbatrol )

16. Nursing Management Risk for injury from falls and possible skin breakdown ( pressure ulcers , abrasions), resulting from constant movement. Imbalanced nutrition, less than body requirements, due to inadequate intake. Anxiety and impaired communication from excessive grimacing and unintelligible speech. Disturbed thought processes and impaired social interaction.