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ECT in genetically confirmed Huntington's disease

C F Lewis1, J R DeQuardo, R Tandon

  • 1University of Michigan Department of Psychiatry, Ann Arbor 48109, USA.

The Journal of Neuropsychiatry and Clinical Neurosciences
|January 1, 1996
PubMed

Abstract:

The authors report successful ECT treatment of a severely depressed man with genetically confirmed Huntington's disease. He responded well to treatment and showed no abnormal movements or worsening in his cognitive status.

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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,...

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