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Self-Assessment Questions


Answer 8
  1. Huntington’s disease. Huntington’s disease is a degenerative, progressive, hereditary (autosomal dominant) disorder that is characterized by a movement disorder (usually chorea), dementia, and a personality disorder. The Huntington’s disease gene is located on the short arm of chromosome 4 (4p16.3) and contains extra copies of trinucleotide repeats of cytosine-adenine-guanine. Imaging with MRI or computed tomography will show dilatation of the frontal horns of the lateral ventricles due to caudate nucleus atrophy. Treatment is based on the patient’s symptoms. For treating depression and psychosis related to Huntington’s disease, antidepressants and antipsychotic agents can be used, respectively. The choreiform movements can be controlled by typical neuroleptic agents, such as haloperidol and perphenazine, or presynaptic dopamine depleters, such as reserpine and tetrabenazine.

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