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Brain SPECT imaging in Huntington's disease before and after therapy with olanzapine. Case report

E C Etchebehere1, M C Lima, W Passos

  • 1Department of Radiology, Faculdade de Ciências Médicas (FCM) da Universidade Estadual de Campinas (UNICAMP), Brazil. elba@mn-d.com

Arquivos De Neuro-Psiquiatria
|February 7, 2001
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Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...