Summary of Huntington's Disease

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Disease Summary Huntington’s disease is of great concern because it is a genetic disease that affects many people worldwide. Huntington’s is described by Wider and Luthi-Carter (2006) as the most prevalent inherited neurodegenerative disorder in humans, affecting between two to eight per 100,000 inhabitants of Western countries. Huntington’s also has a slow onset with an average age of onset around 40 (Wider & Luthi-Carter, 2006). Wider and Luthi-Carter (2006) note the cause of this disease to be a mutation in the huntingtin gene, which can be characterized by distinct symptoms. Chorea, from the Greek “to dance”, is the main distinguishing feature of this mutation and is described by Wider and Luthi-Carter (2006) as rapid involuntary movements that manifest as eyelid elevation, head bobbing, facial grimacing, and jerking of the limbs. Chorea is also noticeable in the way one walks, making an individual move in a zigzag pattern and appear to be thrown off balance by involuntary movements (Wider & Luthi-Carter, 2006). The disease duration is between ten and thirty years and is often first noticed in the early stages by symptoms including attention disorders, personality changes, and alterations in motor control (Wider & Luthi-Carter, 2006). Though Huntington’s is caused by a mutation, research is still being focused on its pathogenesis to gain a better understanding of the disease. Research done by Wider and Luthi-Carter (2006) shows that the main pathological causes of Huntington’s are atrophy and gliosis of the caudate nucleus and the putamen, which become noticeable and statistically significant around ten years prior to disease onset. The research completed by Wider and Luthi-Carter (2006), also shows a significant loss of GA... ... middle of paper ... ...2013) chose to only use male mice in order to avoid the female estrus cycle. Using female mice would enhance this study because females certainly differ biologically from males and may therefore experience different effects of Huntington’s. The knowledge gained as a result of these differences may be beneficial in treating Huntington’s in male and female patients. Works Cited Wider, C., & Lüthi-Carter, R. (2006). Huntington's disease: Clinical and aetiologic aspects. Schweizer Archiv Für Neurologie Und Psychiatrie, 157(8), 378-383. Retrieved from Abada, Y. K., Schreiber, R., & Ellenbroek, B. (2013). Motor, emotional and cognitive deficits in adult BACHD mice: A model for Huntington’s disease. Behavioural Brain Research, 238, 243-251. doi:

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