ArticlePLoS currents2012
The Prevalence of Juvenile Huntington's Disease: A Review of the Literature and Meta-Analysis.
Article in PLoS currents, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 69 papers, 3 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
69 citing papers in PubMed, 3 syntheses or guidelines pooled it, 141 citations in OpenAlex.
- Use of antidepressants in early Huntington's Disease: a systematic review and meta-analysis.Neurodegenerative disease management · 2026Pooled it
- Variation in the reported prevalence of Huntington's disease: a systematic review and guide to interpretation.Journal of neurology · 2025Pooled it
- Development of Research on Huntington Disease in China.Neuroscience bulletin · 2017Pooled it
- Review
- Early Manifestations, Diagnostic Pathways, and Epilepsy in Juvenile-Onset Huntington Disease: A Three-Patient Case Series and Systematic Review.Brain sciences · 2026Article
- Substantia Nigra as Brainstem Signature in Pediatric-Onset Huntington's Disease: The RAREST-JHD Study.Movement disorders clinical practice · 2026Article
- Inhibition of the NLRP3 Inflammasome With MCC950 Improves Gut Health in Huntington's Disease Mice.Journal of neurochemistry · 2026Article
- An exploration of the genetics of the mutant Huntingtin (mHtt) gene in a cohort of patients with chorea from different ethnic groups in sub-Saharan Africa.Annals of human genetics · 2025Article
- Update on the Symptomatic Treatment of Huntington's Disease: From Pathophysiology to Clinical Practice.International journal of molecular sciences · 2025Review
- Comparison of the Clinical Spectrum of Juvenile- and Adult-Onset Huntington Disease: A National Cohort and Enroll-HD Observational Study.Neurology · 2025Observational
- Brain-Derived Neurotrophic Factor (BDNF) in Huntington's Disease: Neurobiology and Therapeutic Potential.Current neuropharmacology · 2025Review
- A Practical Guide for Diagnostic Investigations and Special Considerations in Patients With Huntington's Disease in Korea.Journal of movement disorders · 2025Article
- Developmental and physiological impacts of pathogenic human huntingtin protein in the nervous system.Neurobiology of disease · 2024Article
- Medication Use and Treatment Indications in Huntington's Disease; Analyses from a Large Cohort.Movement disorders clinical practice · 2024Observational
- Association between sex and Huntington's disease: an updated review on symptomatology and prognosis of neurodegenerative disorders.Wiener medizinische Wochenschrift (1946) · 2024Review
- Huntington disease-like 2: insight into neurodegeneration from an African disease.Nature reviews. Neurology · 2024Review
- Prevalence of Juvenile-Onset and Pediatric Huntington's Disease and Their Availability and Ability to Participate in Trials: A Dutch Population and Enroll-HD Observational Study.Journal of Huntington's disease · 2024Observational
- Clinical Review of Juvenile Huntington's Disease.Journal of Huntington's disease · 2024Review
- From Pathogenesis to Therapeutics: A Review of 150 Years of Huntington's Disease Research.International journal of molecular sciences · 2023Review
- The Use of Stem Cells as a Potential Treatment Method for Selected Neurodegenerative Diseases: Review.Cellular and molecular neurobiology · 2023Review
9 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Juvenile Huntington's disease (JHD) is usually defined as Huntington's disease with an onset ≤ 20 years. The proportion of JHD cases reported in studies of Huntington's disease (HD) varies. A review of the literature found 62 studies that reported the proportion of JHD cases amongst all HD cases. The proportion of JHD cases in these studies ranged from 1% to 15%, and in a meta-analysis the pooled proportion of JHD cases was 4.92% (95% confidence interval of 4.07% to 5.84%). Limiting the analysis to the 25 studies which used multiple methods of ascertainment resulted in a similar pooled proportion of 5.32%, (95% confidence interval 4.18% to 6.60%). A small difference was observed when the meta-analysis was restricted to studies from countries defined by the World Bank as high income, that used multiple methods of ascertainment, and that were conducted since 1980 (4.81%, 95% confidence interval 3.31% to 6.58%, n=11). This contrasts with the pooled result from three post 1980 studies using multiple methods of ascertainment from South Africa and Venezuela, defined by the World Bank as upper middle income, where the estimated mean proportion was 9.95%, (95% confidence interval 6.37% to 14.22%). These results, which are expected to be more robust than those from a single study alone, may be helpful in estimating the proportion of JHD cases in a given population. Key Words: Juvenile Huntington's disease, prevalence, epidemiology.
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.