Evidence map›Paper›PMID 24614516›Full record

ReviewNature reviews. Neurology2014

Huntington disease: natural history, biomarkers and prospects for therapeutics.

Christopher A Ross, Elizabeth H Aylward, Edward J Wild, Douglas R Langbehn, Jeffrey D Long, John H Warner, Rachael I Scahill, Blair R Leavitt, Julie C Stout, Jane S Paulsen and 5 more

Registry-linked trialAbstract readReview
PubMed Publisher
In one paragraph

Review in Nature reviews. Neurology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03252535 (Dose-Response Evaluation of the Investigational Product Cellavita HD After Intravenous Administration in Patients With Huntington's Disease), which is not on this map. Cited by 503 papers, 4 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
503citing papers in PubMed, 4 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.

NCT03252535 phase2completednot on this mapstarted 2018, after this paper: background citation

Dose-Response Evaluation of the Investigational Product Cellavita HD After Intravenous Administration in Patients With Huntington's Disease

TypeinterventionalSponsorAzidus BrasilRan2018 to 2021Enrolled49ConditionsHuntington DiseaseArmsCellavita HD lower dose, Cellavita HD higher dose, Placebo
3 · Its place in the literature

Who cites it

503 citing papers in PubMed, 4 syntheses or guidelines pooled it.

  1. Pooled it
  2. Systematic Review with Meta-Analysis of Biofluid Markers for Huntington's Disease.Movement disorders : official journal of the Movement Disorder Society · 2025
    Pooled it
  3. Pooled it
  4. Pooled it
  5. Trial
  6. Trial
  7. Trial
  8. Trial
  9. Trial
  10. Review
  11. Stage-dependent tau-PET signatures in Huntington's disease revealed by [¹⁸F]PI-2620.European journal of nuclear medicine and molecular imaging · 2026
    Article
  12. Article
  13. Review
  14. Article
  15. Treatment of Huntington's disease with a pan-HTT-targeting CRISPR nuclease.Molecular therapy : the journal of the American Society of Gene Therapy · 2026
    Article
  16. Silencing of humanJournal of Huntington's disease · 2026
    Article
  17. Review
  18. Article
  19. Article
  20. Article

443 more citing papers are in PubMed but not listed here.

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

15 authors.

Christopher A RossDivision of Neurobiology, Johns Hopkins University, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Elizabeth H AylwardSeattle Children's Research Institute, USA.
Edward J WildUniversity College London, UK.
Douglas R LangbehnUniversity of Iowa, USA.
Jeffrey D LongUniversity of Iowa, USA.
John H WarnerCHDI Management/CHDI Foundation, USA.
Rachael I ScahillUniversity College London, UK.
Blair R LeavittUniversity of British Columbia, Canada.
Julie C StoutMonash University, Australia.
Jane S PaulsenUniversity of Iowa, USA.
Ralf ReilmannGeorge-Huntington-Institute, Germany.
Paul G UnschuldUniversity of Zürich, Switzerland.
Alice WexlerUniversity of California, Los Angeles, USA.
Russell L MargolisDivision of Neurobiology, Johns Hopkins University, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Sarah J TabriziUniversity College London, UK.

Funding

NEUROBIOLOGICAL PREDICTORS OF HUNTINGTON'S DISEASER01NS040068 · NINDS · UNIVERSITY OF IOWA · PI PAULSEN, JANE S · 2001 to 2013
$48.5M
Restoring FMRP Phenotypes Frpm Temporal Regional and Splice- Isoforms VarationsP30HD002274 · NICHD · UNIVERSITY OF WASHINGTON · PI GURALNICK, MICHAEL J · 1985 to 2013
$33.6M
NINDS NIH HHS R01 NS040068
6 · The paper itself

Abstract

Huntington disease (HD) can be seen as a model neurodegenerative disorder, in that it is caused by a single genetic mutation and is amenable to predictive genetic testing, with estimation of years to predicted onset, enabling the entire range of disease natural history to be studied. Structural neuroimaging biomarkers show that progressive regional brain atrophy begins many years before the emergence of diagnosable signs and symptoms of HD, and continues steadily during the symptomatic or 'manifest' period. The continued development of functional, neurochemical and other biomarkers raises hopes that these biomarkers might be useful for future trials of disease-modifying therapeutics to delay the onset and slow the progression of HD. Such advances could herald a new era of personalized preventive therapeutics. We describe the natural history of HD, including the timing of emergence of motor, cognitive and emotional impairments, and the techniques that are used to assess these features. Building on this information, we review recent progress in the development of biomarkers for HD, and potential future roles of these biomarkers in clinical trials.

Indexed as

BiomarkersBrainHumansHuntington DiseaseMagnetic Resonance ImagingNeuroimagingRadionuclide ImagingBiomarkers

Identifiers

PMID24614516

What OpenQuestion holds

Textmetadata
Read underepoch 390

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.