Lancet Neurology: The biological and clinical characteristics of gene carriers are far from the rate expected in the Huntington's Disease Youth Study (HD-YAS): a cross-sectional analysis

Huntington's disease is an anthromosomal explicit genetic neurodegenerative disease caused by the CAG amplification of the HTTP gene, which is considered to be the main toxic factor.
, Huntington's disease is characterized by a gradual deterioration of motor and cognitive function and neuropsychiaction.
In an era of new treatments targeting DNA and RNA, a single known genetic cause of Huntington's disease provides an attractive target for such treatments, and several drugs to reduce Huntington's disease are currently being tested in humans.
detailed description of the pre-onset of Huntington's disease is critical to the stageing of the disease, the telling of the best time to start treatment, and the identification of biomarkers for future trials in patients with pre-onset of Huntington's disease (preHD).
paper, using the latest methods, focuses on potential group differences in health control groups and preHD queues away from predicted onset in multiple areas.
The study aims to assess how to identify early changes associated with the disease (i.e., when measurable biomarkers of early neurodegeneration are present, but clinical function remains intact), and which indicators are most sensitive to early preHD.
study of young people with Huntington's disease (HD-YAS) was conducted in the UK.
study recruited young people with preHD and a control group that matched preHD's age, education, and gender.
control group asked that either have a family history of Huntington's disease, but the gene test is negative, or have no known family history of Huntington's disease.
participants underwent detailed neuropsychological and cognitive assessments.
imaging checks include MRI, diffusion imaging, and multi-parameter mapping.
use blood and CSF to examine the healthy bioflow markers of neurons.
cross-sectional analysis was performed using the general least-two linear model to assess differences between groups and their relationship to age and CAG length, which were associated with the predicted clinical age of onset.
data collection period is from 2 August 2017 to 25 April 2019.
we recruited 64 young people with preHD and 67 control groups.
age in the preHD group and the control group was 29.0 years (SD 5.6) and 29.1 years (5.7), respectively.
We note that there was no evidence of significant cognitive or mental disorders in preHD participants who were 23.6 years of age (standard deviation 5.8) (0.22-0.87 for cognitively measured FDRs and 0.31-0.91 for neuropsychiastic measurements).
the shell nucleation of the preHD group is slightly smaller (FDR=0.03), this does not appear to be closely related to the predicted age of onset (FDR=0.54).
no intergroup differences with other brain imaging indicators (FDR;0.16).
compared to the control group, CSF neural silk light protein (NfL), plasma NfL, and CSF YKL-40 increased in the preHD queue (FDR was 0.0001, 0.01 and 0.03, respectively).
NfL elevation of cerebrospinal fluid is more likely to occur in individuals close to the expected clinical onset (FDR-lt;0.0001).
, by identifying a group of preHD individual queues that did not detect functional impairment but began to show a slight increase in certain neurodegenerative biological indicators, we identified key points in the early stages of the disease process.
intervention at this stage may delay or prevent further neurodegeneration in the case of functional integrity, allowing gene carriers to extend their lives without injury.
Scahill, Rachael I et al. Biological and clinical characteristics of gene carriers far from predicted onset in the Huntington's disease Young Adult Study (HD-YAS): a cross-sectional analysis. The Lancet Neurology, Volume 19, Issue 6, 502 - 512MedSci Original Source: MedSci Original Copyright Notice: All text, images and audio and video materials on this website that indicate "Source: Met Medical" or "Source: MedSci Original" are owned by Mets Medical and are not authorized to reproduce, and any media, website or individual must indicate "Source: Mays Medicine".
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