ArticleMovement disorders : official journal of the Movement Disorder Society2023
Longitudinal Clinical and Biological Characteristics in Juvenile-Onset Huntington's Disease.
Article in Movement disorders : official journal of the Movement Disorder Society, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 15 citations in OpenAlex.
- Nuclear membrane disruption in neurodegenerative diseases: Emerging perspectives.Neural regeneration research · 2026Article
- Observational
- Childhood to Adult Neurodevelopment in Gene-Expanded Huntington's Disease (ChANGE-HD) study protocol: A Prospective Longitudinal Neurodevelopmental Study of Huntington's Disease.medRxiv : the preprint server for health sciences · 2025Article
- Cortical and Striatal Functional Connectivity in Juvenile-Onset Huntington's Disease.Brain sciences · 2025Article
- A Practical Guide for Diagnostic Investigations and Special Considerations in Patients With Huntington's Disease in Korea.Journal of movement disorders · 2025Article
- Pediatric Huntington Disease Brains Have Distinct Morphologic and Metabolic Traits: the RAREST-JHD Study.Movement disorders clinical practice · 2024Article
- Evaluating motor progression of juvenile-onset Huntington's Disease: An Enroll-HD analysis.Parkinsonism & related disorders · 2024Article
- Serum metabolomic signatures of patients with rare neurogenetic diseases: an insight into potential biomarkers and treatment targets.Frontiers in molecular neuroscience · 2024Article
- Clinical Review of Juvenile Huntington's Disease.Journal of Huntington's disease · 2024Review
- Metabolomics: An Emerging "Omics" Platform for Systems Biology and Its Implications for Huntington Disease Research.Metabolites · 2023Review
- Longitudinal changes in white matter as measured with diffusion tensor imaging in adult-onset myotonic dystrophy type 1.Neuromuscular disorders : NMD · 2023Article
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
Abstract
backgroundJuvenile-onset Huntington's disease (JOHD) is a rare form of Huntington's disease (HD) characterized by symptom onset before the age of 21 years. Observational data in this cohort is lacking.
objectivesQuantify measures of disease progression for use in clinical trials of patients with JOHD.
methodsParticipants who received a motor diagnosis of HD before the age of 21 were included in the Kids-JOHD study. The comparator group consisted of children and young adults who were at-risk for inheriting the genetic mutation that causes HD, but who were found to have a CAG repeat in the non-expanded range (gene non-expanded [GNE]).
resultsData were obtained between March 17, 2006, and February 13, 2020. There were 26 JOHD participants and 78 GNE participants who were comparable on age (16.03 vs. 14.43, respectively) and sex (53.8% female vs. 57.7% female, respectively). The mean annualized decrease in striatal volume in the JOHD group was -3.99% compared to -0.06% in the GNE (mean difference [MD], -3.93%; 95% confidence intervals [CI], [-4.98 to -2.80], FDR < 0.0001). The mean increase in the Unified Huntington's Disease Rating Scale Total Motor Score per year in the JOHD group was 7.29 points compared to a mean decrease of -0.21 point in the GNE (MD, 7.5; 95% CI, [5.71-9.28], FDR < 0·0001).
conclusionsThese findings demonstrate that structural brain imaging and clinical measures in JOHD may be potential biomarkers of disease progression for use in clinical trials. Collaborative efforts are required to validate these results in a larger cohort of patients with JOHD. © 2022 International Parkinson and Movement Disorder Society.
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