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Huntington's Disease

          Symptoms include uncontrolled movements, intellectual degradation and emotional disturbance and often appear between the ages of 35 and 44. Another rarer form of Huntington's begins earlier in childhood or adolescence. The most obvious symptom of Huntington's Disease is chorea and appears in the majority of Huntington's patients. Chorea causes patients to jerk, twist, and writhe involuntarily. Patients may abnomally move their faces and/or body. Other symptoms are irritability, paranoia, psychosis, and dementia. In children, symptoms include involuntary or slow movements

 

          Diagnosis of Huntington's Disease is most often done by a specialist. Diagnosis often occurs after a thorough examination, a look into family history, and brain imaging. Doctors can now also use genetic testing to determine if a person has Huntington's Disease thanks to the 1933 discovery of the gene that causes it. Victims of the disease usually live for about 15 to 18 years after diagnosed. To check for signs of Huntington's, doctors may do CT scans of the brain and/or head MRI scans

A recent development has introduced a technology called "CRISPR", or "Clustered Regularly Interspaced Short Palindromic Repeats". CRISPR enables scientists to edit the genome of patients by “cutting” their DNA with technology currently in development, removing unwanted sequences and inserting new and improved ones. Huntington's Disease is caused by a C-A-G repeating sequence at the end of the HD gene, which scientists can now cut out completely. This renders the gene useless and disables it from coding for proteins that would normally cause symptoms to appear, also known as gene silencing. However, the gene that codes for Huntington’s would still be present in the DNA which could be passed on to the child, so future efforts are working to find a solution to this problem.

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          Huntington's Disease is a genetic neurological disorder that causes degeneration in certain areas of the brain. It is estimated that worldwide, 5-10 people out of 100,000 will have Huntington's Disease. Although Huntington's Disease is rare, it affects both males and females equally. Since it is an autosomal, dominant disorder, if one parent has the disease, their children have 50% chances of inheriting the gene for Huntington's. Everyone with this gene will develop the disease. 

 

There is currently no cure for Huntington's Disease, nor is there a way to reverse its damage to the brain once it has been done. However, treatments include dopamine blockers, antipsychotic drugs, antiseizure drugs, antianxiety drugs, antidepressants, and therapy. These treatments listed cannot prevent the disease from getting worse, but can help reduce the symptoms that can occur randomly such as the unusual movements and behaviors. Depression and suicide is at a higher risk in patients with Huntington's disease, so they should be monitored carefully and treated regularly. There may be side effects to the treatments including anxiety, restlessness or fatigue, but it may be difficult to tell them apart from the symptoms of the disease itself.

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Removing the Huntington’s Gene Completely

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