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| George Huntington |
Huntington's disease is a hereditary disease which rare, with a medical condition which is characterized by the progressive degeneration of certain nerve cells in the brain , and is the result of a genetic defect that is carried on chromosome 4. Named after an American doctor " George Huntington " who first wrote this disease in 1872 .
The initial name of this disease is "chorea" Huntington , from the Greek word meaning "dance". Chorea is described as a circular motion, twisting, uncontrolled and constant deteriorating progressively in line with the development of the disease . However, some patients with adult - onset Huntington experiencing severe rigidity and not able to move the weight but not chorea, so the dominant symptoms is akinesia. Other symptoms may include hallucinations, memory loss and poor self- assessment .
The only risk factor is having one or both parents who carry the disease gene, as this is a genetic disease. Unfortunately no one can cure this disease and the prognosis is always bad because its symptoms grow worse over time; patient will usually die within 15 to 20 years after onset, but this number can vary depending on the severity of disability. Any individual with symptoms or a parent with this condition should immediately consult a doctor.
Huntington disease occurred in the decade of the 4th and 5th . The prevalence of this disease is 5-10 people per 100,000 population . The research at the National Institute of Neurological Disorder and Stroke (NINDS, 2000) estimates that more than 30,000 people in the U.S. suffer of Huntington, or 1:10,000 people .
Huntington minority of cases may be sporadic occurrence in the absence of a family history of hereditary. Such cases may be due to a new genetic mutation in the gene that occurs during the process of sperm development which will bring the risk factors trinucleotide repeat CAG ( cysteine - adenosine - guanine) , which became the cause of Huntington .
Etiology
What is causes of Huntington's disease? Huntington's disease is caused by degeneration of neurons that are genetically programmed in the basal ganglia are associated with defects in chromosome 4 in the form of propagation of disturbances that are autosomal dominant trinucleotide . Genes on chromosome 4 that produces the protein huntington is widely diespresikan the central nervous system. This gene contains a CAG trinucleotide repeat that is normally only amounted to 26 pieces. However, in patients with Huntington's disease, CAG trinucleotide repeat can reach more than 36 pieces. Repetition over 100 usually occurs in childhood ( juveinile ) derived by her father
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| Huntington's Disease Pathway Genetic |
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1. Picture: http://digitaljournal.com/image/66144
Related Articles
1. Pathophysiology of Huntington's Disease
2. Clinical Manifestations of Huntington's Disease
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4. Medical Treatment and Therapy of Huntington's Disease
Related Articles
1. Pathophysiology of Huntington's Disease
2. Clinical Manifestations of Huntington's Disease
3. Diagnosis and Differential Diagnosis of Huntington's Disease
4. Medical Treatment and Therapy of Huntington's Disease
