Evidence map›Paper›PMID 26908513›Full record

SynthesisBMJ open2016

Incidence of adult Huntington's disease in the UK: a UK-based primary care study and a systematic review.

Nancy S Wexler, Laura Collett, Alice R Wexler, Michael D Rawlins, Sarah J Tabrizi, Ian Douglas, Liam Smeeth, Stephen J Evans

Abstract readSystematic Review
In one paragraph

Synthesis in BMJ open, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 3 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
23citing papers in PubMed, 3 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

23 citing papers in PubMed, 3 syntheses or guidelines pooled it.

  1. Pooled it
  2. Prevalence and Incidence of Huntington's Disease: An Updated Systematic Review and Meta-Analysis.Movement disorders : official journal of the Movement Disorder Society · 2022
    Pooled it
  3. Distribution of theClinical medicine & research · 2020
    Pooled it
  4. Review
  5. Review
  6. Unraveling Molecular Targets for Neurodegenerative Diseases ThroughInternational journal of molecular sciences · 2025
    Review
  7. Article
  8. Article
  9. Article
  10. Article
  11. Geographic differences in the incidence of Huntington's disease in Sardinia, Italy.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2021
    Article
  12. Article
  13. Huntington's disease among immigrant groups and Swedish-born individuals: a cohort study of all adults 18 years of age and older in Sweden.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2021
    Article
  14. Review
  15. Article
  16. Observational
  17. Article
  18. Article
  19. Article
  20. Huntington's disease in the United States: Variation by demographic and socioeconomic factors.Movement disorders : official journal of the Movement Disorder Society · 2019
    Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors.

Nancy S WexlerDepartment of Neurology and Psychiatry, Columbia University, New York, New York, USA Hereditary Disease Foundation, New York, New York, USA.
Laura CollettDepartment of Medical Statistics, London School of Hygiene and Tropical Medicine, London, UK.
Alice R WexlerHereditary Disease Foundation, New York, New York, USA.
Michael D RawlinsDepartment of Non-Communicable Disease Epidemiology, London School of Hygiene and Tropical Medicine, London, UK.
Sarah J TabriziDepartment of Neurodegenerative Diseases, Institute of Neurology, University College London, London, UK.
Ian DouglasDepartment of Non-Communicable Disease Epidemiology, London School of Hygiene and Tropical Medicine, London, UK.
Liam SmeethDepartment of Non-Communicable Disease Epidemiology, London School of Hygiene and Tropical Medicine, London, UK.
Stephen J EvansDepartment of Medical Statistics, London School of Hygiene and Tropical Medicine, London, UK.

Funding

Medical Research Council G0802403Medical Research Council MR/K006584/1Medical Research Council MR/L012936/1Medical Research Council MR/L02053X/1Wellcome Trust 200181/Z/15/Z
6 · The paper itself

Abstract

objectivesThe prevalence of Huntington's disease (HD) recorded in the UK primary care records has increased twofold between 1990 and 2010. This investigation was undertaken to assess whether this might be due to an increased incidence. We have also undertaken a systematic review of published estimates of the incidence of HD.

settingIncident patients with a new diagnosis of HD were identified from the primary care records of the Clinical Practice Research Datalink (CPRD). The systematic review included all published estimates of the incidence of HD in defined populations.

participantsA total of 393 incident cases of HD were identified from the CPRD database between 1990 and 2010 from a total population of 9,282,126 persons. PRIMARY AND SECONDARY OUTCOME MEASURES: The incidence of HD per million person-years was estimated. From the systematic review, the extent of heterogeneity of published estimates of the incidence of HD was examined using the I(2) statistic.

resultsThe data showed that the incidence of HD has remained constant between 1990 and 2010 with an overall rate of 7.2 (95% CI 6.5 to 7.9) per million person-years. The systematic review identified 14 independent estimates of incidence with substantial heterogeneity and consistently lower rates reported in studies from East Asia compared with those from Australia, North America and some--though not all--those from Europe. Differences in incidence estimates did not appear to be explained solely by differences in case ascertainment or diagnostic methods.

conclusionsThe rise in the prevalence of diagnosed HD in the UK, between 1990 and 2010, cannot be attributed to an increase in incidence. Globally, estimates of the incidence of HD show evidence of substantial heterogeneity with consistently lower rates in East Asia and parts of Europe. Modifiers may play an important role in determining the vulnerability of different populations to expansions of the HD allele.

Indexed as

AdultAge of OnsetElectronic Health RecordsHumansHuntington DiseaseIncidenceMiddle AgedPrevalencePrimary Health CareUnited KingdomEPIDEMIOLOGYGENETICS

Identifiers

PMID26908513
PMCPMC4769413

What OpenQuestion holds

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Registered trials

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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.