QUALITY OF LIFE IMPAIRMENTS IN HUNTINGTON'S DISEASE (LITERATURE REVIEW AND OWN DATA)

Authors

  • Usmanov Firdavs Bakhtiyorovich Author

Keywords:

Keywords: Huntington's disease, chorea, anticipation phenomenon, dementia, apathy

Abstract

 
Abstract: Huntington's disease (HD) is a rare, incurable, hereditary neurological 
disorder that causes the progressive breakdown of nerve cells in the brain. Huntington's 
chorea is a progressive neurodegenerative hereditary disorder that is transmitted from 
generation to generation in an autosomal dominant manner. Huntington's chorea occurs 
in 4-6 cases per 100,000 population (4-6:100,000). The classic symptom is chorea, 
which is the appearance of hyperkinesis, i.e. uncontrolled movements. This is one of 
the  main  features  of  the  disease,  which  is  considered  to  be  the  anticipation 
phenomenon, i.e. the disease progresses gradually and begins at a young age [1]. The 
mutant huntingtin gene has a repeat of the trinucleotide sequence CAG from 40 to 80. 
The  pathogenesis  of  HD  is  multifaceted  and  involves  glutamine  aggregation, 
mitochondrial and oxidative stress, mitochondrial-related pathway dysfunction, and 
DNA damage. Currently, two drugs, tetrabenazine and deutetrabenazine, are approved 
for the treatment of HD. 

References

References

1. Ayala-Pena, S. (2012). The role of oxidative DNA damage in mitochondrial

dysfunction and the pathogenesis of Huntington's disease. P. 102-110.

2. Bhattacharyya, B.K. (2016). George Huntington and his disease 19(1), 25-28.

3. Bilal, H., Warren, N., Dahanayake, P., Kelso, V., Farrand, S., & Stout, J.C.

(2022). The experience of depression in survivors of Huntington's disease: 1(3),

321-335.

4. Chen, I.W., Chen, C.M., Wu, Y.R., & Hua, M.S. (2017). Patterns of faulty

memory in patients with Huntington's disease. 32(4), 391-400.

5. Costa, V., & Scorrano, L. (2012). Elucidating the role of mitochondria in the

pathogenesis of Huntington's disease. EMBO Journal, 31(8), 1853-1864.

Published

2025-05-26

How to Cite

Usmanov Firdavs Bakhtiyorovich. (2025). QUALITY OF LIFE IMPAIRMENTS IN HUNTINGTON’S DISEASE (LITERATURE REVIEW AND OWN DATA) . Ta’lim Innovatsiyasi Va Integratsiyasi, 46(1), 99-101. https://scientific-jl.com/tal/article/view/16486