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Updated: May 24, 2026

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Published on: February 27, 2018

The sparse evidence-base for self-injurious behavior in Huntington's disease: A call to action

Inga Stewart1, Fiona Stone2, Esther Gathii2

  • 1Consultant Clinical Psychologist, Head of Progressive Neurological Conditions Research Program, St Andrews Healthcare, Northampton, UK.

Journal of Huntington'S Disease
|May 22, 2026
PubMed
Summary

No abstract available in PubMed .

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Huntington Disease l: Introduction01:21

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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,...