Evidence map›Paper›PMID 29353237›Full record

ReviewJournal of neurology, neurosurgery, and psychiatry2018

Beyond motor neurons: expanding the clinical spectrum in Kennedy's disease.

Raquel Manzano, Gianni Sorarú, Christopher Grunseich, Pietro Fratta, Emanuela Zuccaro, Maria Pennuto, Carlo Rinaldi

Open access · hybridAbstract readReview
In one paragraph

Review in Journal of neurology, neurosurgery, and psychiatry, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
39citing papers in PubMed, 2 pooled it
2.7field-weighted citation impact, top 10% 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

39 citing papers in PubMed, 2 syntheses or guidelines pooled it, 65 citations in OpenAlex.

  1. CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne · 2026
    Guideline
  2. Best practice recommendations for the clinical care of spinal bulbar muscular atrophy.CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne · 2025
    Guideline
  3. Trial
  4. Frequency and Circumstances of Falls Events in People Living With Spinal and Bulbar Muscular Atrophy: A Cross-Sectional Survey.Physiotherapy research international : the journal for researchers and clinicians in physical therapy · 2026
    Article
  5. Review
  6. Article
  7. Review
  8. Article
  9. Article
  10. Article
  11. Article
  12. Article
  13. Association of serum Spp1 levels with disease progression in ALS and SBMA.Annals of clinical and translational neurology · 2024
    Article
  14. Article
  15. Article
  16. Article
  17. Review
  18. Article
  19. Bicalutamide and Trehalose Ameliorate Spinal and Bulbar Muscular Atrophy Pathology in Mice.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2023
    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

7 authors at 5 institutions in 3 countries.

Raquel ManzanoDepartment of Physiology, Anatomy and Genetics, University of Oxford, Oxford, UK.
Gianni SorarúDepartment of Neurosciences, Neuromuscular Center, University of Padova, Padova, Italy.
Christopher GrunseichNeurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA.
Pietro FrattaDepartment of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.
Emanuela ZuccaroDepartment of Biomedical Sciences, University of Padova, Padova, Italy.
Maria PennutoDepartment of Biomedical Sciences, University of Padova, Padova, Italy.
Carlo RinaldiDepartment of Physiology, Anatomy and Genetics, University of Oxford, Oxford, UK.
University of Oxford · GBUniversity of Padua · ITDulbecco Telethon Institute · ITNational Institutes of Health · USUniversity College London · GB

Funding

Studies of Hereditary Neurological Disease: Disease MechanismsZIANS003038 · NINDS · NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE · PI FISCHBECK, KENNETH · 2009 to 2023
$19.5M
Studies Of Hereditary Neurological Disease: Disease Gene IdentificationZIANS002974 · NINDS · NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE · PI FISCHBECK, KENNETH · 2009 to 2023
$9.8M
Medical Research Council MR/M008606/1Motor Neurone Disease Association FRATTA/JAN15/946-795Wellcome Trust 205162/Z/16/Z
6 · The paper itself

Abstract

Kennedy's disease, or spinal and bulbar muscular atrophy (SBMA), is an X-linked neuromuscular condition clinically characterised by weakness, atrophy and fasciculations of the limb and bulbar muscles, as a result of lower motor neuron degeneration. The disease is caused by an abnormally expanded triplet repeat expansions in the ubiquitously expressed androgen receptor gene, through mechanisms which are not entirely elucidated. Over the years studies from both humans and animal models have highlighted the involvement of cell populations other than motor neurons in SBMA, widening the disease phenotype. The most compelling aspect of these findings is their potential for therapeutic impact: muscle, for example, which is primarily affected in the disease, has been recently shown to represent a valid alternative target for therapy to motor neurons. In this review, we discuss the emerging study of the extra-motor neuron involvement in SBMA, which, besides increasingly pointing towards a multidisciplinary approach for affected patients, deepens our understanding of the pathogenic mechanisms and holds potential for providing new therapeutic targets for this disease.

Indexed as

Autonomic Nervous System DiseasesBulbo-Spinal Atrophy, X-LinkedHumansMotor NeuronsMuscular AtrophyPhenotypeTrinucleotide Repeat ExpansionUrinary Bladder Neck Obstructionmotor neuron diseaseneurogeneticsneuromuscular

Identifiers

PMID29353237
PMCPMC6204939
OpenAlexW2785231623

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
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.