ReviewJournal of molecular neuroscience : MN2016
Onset Manifestations of Spinal and Bulbar Muscular Atrophy (Kennedy's Disease).
Review in Journal of molecular neuroscience : MN, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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.
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.
Who cites it
18 citing papers in PubMed, 44 citations in OpenAlex.
- Tongue volume in spinal and bulbar muscular atrophy (SBMA): an AI-assisted automatic MRI analysis.Journal of neurology · 2026Article
- Heterogeneous phenotype and cardiovascular comorbidities in Swedish patients with spinobulbar muscular atrophy.Journal of neurology · 2026Article
- A systematic review of the association between the age of onset of spinal bulbar muscular atrophy (Kennedy's disease) and the length of CAG repeats in the androgen receptor gene.eNeurologicalSci · 2024Review
- Psychotherapeutic Change Mechanisms and Causal Psychotherapy: Applications to Child Abuse and Trauma.Journal of child & adolescent trauma · 2022Article
- Enhanced Neural Empathic Responses in Patients with Spino-Bulbar Muscular Atrophy: An Electrophysiological Study.Brain sciences · 2020Article
- The French national protocol for Kennedy's disease (SBMA): consensus diagnostic and management recommendations.Orphanet journal of rare diseases · 2020Article
- Preliminary design and validation of the "6-K-scale" for bulbar symptoms evaluation in SBMA.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2019Article
- DNA methylation inhibitor attenuates polyglutamine-induced neurodegeneration by regulating Hes5.EMBO molecular medicine · 2019Article
- Clinical manifestations and AR gene mutations in Kennedy's disease.Functional & integrative genomics · 2019Article
- Kennedy's disease (spinal and bulbar muscular atrophy): a clinically oriented review of a rare disease.Journal of neurology · 2019Review
- Empathy In Neurodegenerative Diseases: A Systematic Review.Neuropsychiatric disease and treatment · 2019Review
- Unimpaired Neuropsychological Performance and Enhanced Memory Recall in Patients with Sbma: A Large Sample Comparative Study.Scientific reports · 2018Article
- X-linked spinal and bulbar muscular atrophy (Kennedy's disease): the first case described in the Brazilian Amazon.Einstein (Sao Paulo, Brazil) · 2018Article
- [Genetics of tremor].Der Nervenarzt · 2018Review
- Biomarkers of Spinal and Bulbar Muscle Atrophy (SBMA): A Comprehensive Review.Frontiers in neurology · 2018Review
- Emergencies in motoneuron disease.Internal and emergency medicine · 2017Review
- Validation of the Italian version of the SBMA Functional Rating Scale as outcome measure.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2016Article
- Introduction to the Special Issue on Spinal and Bulbar Muscular Atrophy.Journal of molecular neuroscience : MN · 2016Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Spinal and bulbar muscular atrophy (SBMA) is regarded as a disorder with adult onset between third and fifth decade of life. However, there is increasing evidence that SBMA may start already before adulthood. The present study investigated the following: (1) Which clinical manifestations have been described so far in the literature as initial manifestations? (2) Which was the age at onset of these manifestations? and (3) Is age at onset dependent on the CAG-repeat length if non-motor manifestations are additionally considered? Data for this review were identified by searches of MEDLINE using appropriate search terms. Onset manifestations in SBMA can be classified as frequent, rare, motor, non-motor, or questionable. Frequent are muscle weakness, cramps, fasciculations/twitching, tremor, dysarthria, dysphagia, or gynecomastia. Rare are myalgia, easy fatigability, exercise intolerance, polyneuropathy, hyper-CKemia, under-masculinized genitalia, scrotal hypospadias, microphallus, laryngospasm, or oligospermia. Questionable manifestations include sensory disturbances, cognitive impairment, increased pituitary volume, diabetes, reduced tongue pressure, elevated creatine-kinase, or low androgens/high estrogens. Age at onset is highly variable ranging from 4-76 years. Non-motor manifestations develop usually before motor manifestations. Age at onset depends on what is considered as an onset manifestation. Considering non-motor onset manifestations, age at onset is independent of the CAG-repeat size. In conclusion, age at onset of SBMA depends on what is regarded as onset manifestation. If non-motor manifestations are additionally considered, age at onset is independent of the CAG-repeat length. Since life expectancy is hardly reduced in SBMA, re-investigation of patients from published studies with regard to their initial disease profiles is recommended.
Indexed as
Identifiers
What OpenQuestion holds
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.