Evidence map›Paper›PMID 22850483›Full record

ArticleNeurobiology of disease2013

Limited regional cerebellar dysfunction induces focal dystonia in mice.

Robert S Raike, Carolyn E Pizoli, Catherine Weisz, Arn M J M van den Maagdenberg, H A Jinnah, Ellen J Hess

Open access · greenAbstract read
In one paragraph

Article in Neurobiology of disease, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.

0numbers the graph read from it
0cells of the map it votes in
41citing papers in PubMed
5.5field-weighted citation impact, top 4% of its field
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

41 citing papers in PubMed, 71 citations in OpenAlex.

  1. Trial
  2. Cerebellar α1Cellular and molecular life sciences : CMLS · 2025
    Article
  3. Article
  4. Article
  5. Discovery of the First Selective MACS pharmacology & translational science · 2021
    Article
  6. Article
  7. Lessons learned from the syndrome of oculopalatal tremor.Journal of computational neuroscience · 2021
    Review
  8. Dystonia and Cerebellum: From Bench to Bedside.Life (Basel, Switzerland) · 2021
    Review
  9. Article
  10. Article
  11. Article
  12. Article
  13. Article
  14. Dopamine Receptor Agonist Treatment of Idiopathic Dystonia: A Reappraisal in Humans and Mice.The Journal of pharmacology and experimental therapeutics · 2018
    Article
  15. Article
  16. Review
  17. Physiology of midbrain head movement neurons in cervical dystonia.Movement disorders : official journal of the Movement Disorder Society · 2017
    Article
  18. Article
  19. Article
  20. 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

6 authors at 3 institutions in 2 countries.

Robert S RaikeDepartment of Pharmacology, Emory University School of Medicine, Atlanta, GA 30322, USA.
Carolyn E PizoliDepartment of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
Catherine WeiszDepartment of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA; Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
Arn M J M van den MaagdenbergDepartment of Human Genetics, Leiden University Medical Centre, 2300 RC Leiden, The Netherlands; Department of Neurology, Leiden University Medical Centre, 2300 RC Leiden, The Netherlands.
H A JinnahDepartment of Neurology, Emory University School of Medicine, Atlanta, GA 30322, USA; Department of Human Genetics, Emory University School of Medicine, Atlanta, GA 30322, USA.
Ellen J HessDepartment of Pharmacology, Emory University School of Medicine, Atlanta, GA 30322, USA; Department of Neurology, Emory University School of Medicine, Atlanta, GA 30322, USA. Electronic address: ejhess@emory.edu.
Emory University · USJohns Hopkins Medicine · USLeiden University Medical Center · NL

Funding

ENNCF - Viral Vector CoreP30NS055077 · NINDS · EMORY UNIVERSITY · PI LEVEY, ALLAN I · 2008 to 2018
$6.8M
Pathophysiology of dystonia: animal modelsR01NS033592 · NINDS · JOHNS HOPKINS UNIVERSITY · PI HESS, ELLEN J. · 2001 to 2010
$3.5M
NEUROBIOLOGY OF DYSTONIAR01NS040470 · NINDS · JOHNS HOPKINS UNIVERSITY · PI JINNAH, HYDER A · 2000 to 2008
$2.5M
Cerebellar stimulation for the treatment of dystonia: preclinical studiesR21NS077905 · NINDS · EMORY UNIVERSITY · PI HESS, ELLEN J. · 2012 to 2013
$422k
NINDS NIH HHS P30 NS055077NINDS NIH HHS R01 NS033592NINDS NIH HHS R01 NS040470NINDS NIH HHS R01 NS33592NINDS NIH HHS R01 NS40470NINDS NIH HHS R21 NS077905
6 · The paper itself

Abstract

Dystonia is a complex neurological syndrome broadly characterized by involuntary twisting movements and abnormal postures. The anatomical distribution of the motor symptoms varies among dystonic patients and can range from focal, involving an isolated part of the body, to generalized, involving many body parts. Functional imaging studies of both focal and generalized dystonias in humans often implicate the cerebellum suggesting that similar pathological processes may underlie both. To test this, we exploited tools developed in mice to generate animals with gradients of cerebellar dysfunction. By using conditional genetics to regionally limit cerebellar dysfunction, we found that abnormalities restricted to Purkinje cells were sufficient to cause dystonia. In fact, the extent of cerebellar dysfunction determined the extent of abnormal movements. Dysfunction of the entire cerebellum caused abnormal postures of many body parts, resembling generalized dystonia. More limited regions of dysfunction that were created by electrical stimulation or conditional genetic manipulations produced abnormal movements in an isolated body part, resembling focal dystonia. Overall, these results suggest that focal and generalized dystonias may arise through similar mechanisms and therefore may be approached with similar therapeutic strategies.

Indexed as

AgingAnimalsCalbindinsCalcium Channels, N-TypeCerebellar DiseasesCerebellumDystonic DisordersExtremitiesFemaleMaleMiceMice, Inbred C3HMice, Inbred C57BLMice, TransgenicPosturePurkinje CellsCalbindinsCalcium Channels, N-TypeRNA, MessengerCerebellumCre recombinaseElectrical stimulationFocal dystoniaLentivirus

Identifiers

PMID22850483
PMCPMC3567246
OpenAlexW1998722246

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

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.