Abstract

Huntington's disease (HD) is an autosomal dominantneurodegenerative disorder, which occurs in patients witha mutation in the IT15 gene (huntingtin). Clinically, thedisease presents itself with not only motor symptoms butalso dementia. The aim of this study is to review andpresent all recent data, specifically those related to theneuropathology, biochemistry and diagnostic methods ofdementia in HD.

Highlights

  • Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder, which occurs in patients with a mutation in the IT15 gene

  • The aim of this study is to review and present all recent data, those related to the neuropathology, biochemistry and diagnostic methods of dementia in HD

  • Recent advances in molecular and genetic research of HD, derived from articles published in journals indexed in PubMed and other Entrez medical databases are being reviewed in an effort to elucidate mechanisms of cell death in the striatum and disruption of cortical-striatal circuitry

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Summary

Objectives

The aim of this study is to review and present all recent data, those related to the neuropathology, biochemistry and diagnostic methods of dementia in HD

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Results
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