Huntington's Disease

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Huntington’s Disease

Definition- Huntington’s Disease is an inherited disease that causes the progressive breakdown of nerve cells in the brain.

Classification- This disease affects the musculoskeletal system due to loss of brain cells that control the initiation, coordination and fluidity of movement. This disease also affects the digestive system due to the loss of muscle control affects chewing and swallowing. The American Speech-Language-Hearing Association notes that people with Huntington's disease typically have difficulty holding food and liquids in the mouth, initiating swallowing and clearing food from the mouth. Loss of muscle control usually leads to an inability to control the passage of stool. Finally, Huntington’s affects the respiratory system. The disordered swallowing associated with Huntington's disease commonly interferes with the coordinated closure of the airway, causing frequent episodes of choking and contamination of the airway with ingested food and liquids. The Huntington's Outreach Project for Education at Stanford University reports that pneumonia is the leading cause of death among people with Huntington's.

Signs and Symptoms- Behavior changes may occur before movement problems, and can include: behavioral disturbances, hallucinations, irritability, and moodiness. Abnormal and unusual movements include: slow, uncontrolled movements, and facial movements. Dementia that slowly gets worse, including: personality changes, speech changes, and confusion.

Diagnosis- Either fluid from around the fetus, or chorionic villus sampling (CVS—a sample of fetal cells), will indicate whether the baby has inherited the gene for Huntington‘s.

Etiology- Nerve cells become damaged, causing various parts of the brain to deteriorate. The faulty gene that causes Huntington's disease is found on chromosome number 4. A normal copy of the gene produces huntingtin, a protein. The faulty gene is larger than it should be and produces a larger form...
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