Evidence map›Paper›PMID 33692166›Full record

ArticleNeurology2021

Association of CAG Repeat Length in the Huntington Gene With Cognitive Performance in Young Adults.

Jordan L Schultz, Carsten Saft, Peggy C Nopoulos

Abstract read
In one paragraph

Article in Neurology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.

0numbers the graph read from it
0cells of the map it votes in
17citing papers in PubMed
–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

17 citing papers in PubMed.

  1. Article
  2. Review
  3. Sertraline treatment prevents motor dysfunction in a Huntington's disease mouse model and functional decline in patients.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2025
    Article
  4. Review
  5. Review
  6. Article
  7. Article
  8. Article
  9. Observational
  10. Article
  11. Review
  12. Impairments to executive function in emerging adults with Huntington disease.Journal of neurology, neurosurgery, and psychiatry · 2023
    Article
  13. Article
  14. Article
  15. Article
  16. Article
  17. Review
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

3 authors.

Jordan L SchultzFrom the Departments of Psychiatry (J.L.S., P.C.N.) and Neurology (J.L.S., P.C.N.), Carver College of Medicine at the University of Iowa; Division of Pharmacy Practice and Sciences (J.L.S.), University of Iowa College of Pharmacy, Iowa City; Department of Neurology (C.S.), Huntington Center NRW, Ruhr-University Bochum, St Josef-Hospital, Bochum, Germany; and Stead Family Children's Hospital at the University of Iowa (P.C.N.), Iowa City. Jordan-schultz@uiowa.edu.
Carsten SaftFrom the Departments of Psychiatry (J.L.S., P.C.N.) and Neurology (J.L.S., P.C.N.), Carver College of Medicine at the University of Iowa; Division of Pharmacy Practice and Sciences (J.L.S.), University of Iowa College of Pharmacy, Iowa City; Department of Neurology (C.S.), Huntington Center NRW, Ruhr-University Bochum, St Josef-Hospital, Bochum, Germany; and Stead Family Children's Hospital at the University of Iowa (P.C.N.), Iowa City.
Peggy C NopoulosFrom the Departments of Psychiatry (J.L.S., P.C.N.) and Neurology (J.L.S., P.C.N.), Carver College of Medicine at the University of Iowa; Division of Pharmacy Practice and Sciences (J.L.S.), University of Iowa College of Pharmacy, Iowa City; Department of Neurology (C.S.), Huntington Center NRW, Ruhr-University Bochum, St Josef-Hospital, Bochum, Germany; and Stead Family Children's Hospital at the University of Iowa (P.C.N.), Iowa City.

Funding

University of Iowa Hawkeye Intellectual and Developmental Disabilities Research Center (Hawk-IDDRC)P50HD103556 · NICHD · UNIVERSITY OF IOWA · PI EDWIN TED G. ABEL, Lane Strathearn · 2021 to 2026
$8.5M
Investigating Autonomic Dysfunction as an Early Pathologic Feature of Huntington’s DiseaseK23NS117736 · NINDS · UNIVERSITY OF IOWA · PI SCHULTZ, JORDAN · 2021 to 2025
$895k
NICHD NIH HHS P50 HD103556NINDS NIH HHS K23 NS117736
6 · The paper itself

Abstract

objectiveTo investigate the relationships between CAG repeat length in the huntingtin gene and cognitive performance in participants above and below the disease threshold for Huntington disease (HD), we performed a cross-sectional analysis of the Enroll-HD database.

methodsWe analyzed data from young, developing adults (≤30 years of age) without a history of depression, apathy, or cognitive deficits. We included participants with and without the gene expansion (CAG ≥36) for HD. All participants had to have a Total Functional Capacity Score of 13, a diagnostic confidence level of zero, and a total motor score of <10 and had to be >28.6 years from their predicted motor onset. We performed regression analyses to investigate the nonlinear relationship between CAG repeat length and various cognitive measures controlling for age, sex, and education level.

resultsThere were significant positive relationships between CAG repeat length and the Symbol Digit Modalities, Stroop Color Naming, and Stroop Interference test scores. There were significant negative relationships between CAG repeat length and scores on Parts A and B of the Trails Making Test ( DISCUSSION: An increasing number of CAG repeats in the huntingtin gene below disease threshold and low pathologic CAG ranges were associated with some improvements in cognitive performance. These findings outline the relationship between CAG repeats within the huntingtin gene and cognitive development. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that CAG repeat length is positively associated with cognitive function across a spectrum of CAG repeat lengths.

Indexed as

AdolescentAdultCognitionCross-Sectional StudiesDatabases, GeneticFemaleHumansHuntingtin ProteinHuntington DiseaseMaleTrinucleotide Repeat ExpansionYoung AdultHTT protein, humanHuntingtin Protein

Identifiers

PMID33692166
PMCPMC10508647

What OpenQuestion holds

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Registered trials

None linked

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.