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Record W2341818933 · doi:10.1212/nxg.0000000000000069

Clinical and genetic features of cervical dystonia in a large multicenter cohort

2016· article· en· W2341818933 on OpenAlex

Why this work is in the frame

A frame that forgets how it found something cannot be audited. These are the routes that admitted this work.

fundA Canadian funder is recorded on the work.
no affNo Canadian affiliation: this work is invisible to an affiliation-only frame.
No Canadian affiliation. An affiliation-only frame, the usual design, would never have seen this work. It is one of the works that make the case for inverting the frame.

Bibliographic record

VenueNeurology Genetics · 2016
Typearticle
Languageen
FieldMedicine
TopicBotulinum Toxin and Related Neurological Disorders
Canadian institutionsnot available
FundersDystonia CoalitionNational Institute of Neurological Disorders and StrokeParkinsonfondenHealth Science Center, University of TennesseeSt. Jude MedicalNational Institutes of HealthUniversity of TorontoOmeros CorporationSpastic Paraplegia FoundationAdamas PharmaceuticalsIpsenProthenaPsyadon PharmaceuticalsACADIA PharmaceuticalsParkinson Study GroupMerz PharmaceuticalsUltragenyx PharmaceuticalMedical University of South CarolinaUniversity of LouisvilleUniversity of MissouriPfizerNational Parkinson FoundationUniversity of RochesterUCB PharmaMichael J. Fox Foundation for Parkinson's ResearchEmory UniversityCHDI FoundationMedtronicAmerican Parkinson Disease AssociationDystonia Medical Research FoundationParkinson's Disease FoundationNational Center for Advancing Translational SciencesTeva Pharmaceutical IndustriesUniversity of South CarolinaUS WorldMedsMayo ClinicUniversity of MichiganAcorda TherapeuticsDana FoundationIpsen BiopharmaceuticalsAlberta InnovatesNeurocrine BiosciencesBenign Essential Blepharospasm Research FoundationAllerganInternational Parkinson and Movement Disorder SocietyH. Lundbeck A/SAmerican Academy of Neurology
KeywordsCervical dystoniaDystoniaMedicineMissense mutationCohortInternal medicinePediatricsGeneticsBiologyMutationPsychiatryGene

Abstract

fetched live from OpenAlex

OBJECTIVE: To characterize the clinical and genetic features of cervical dystonia (CD). METHODS: Participants enrolled in the Dystonia Coalition biorepository (NCT01373424) with initial manifestation as CD were included in this study (n = 1,000). Data intake included demographics, family history, and the Global Dystonia Rating Scale. Participants were screened for sequence variants (SVs) in GNAL, THAP1, and Exon 5 of TOR1A. RESULTS: The majority of participants were Caucasian (95%) and female (75%). The mean age at onset and disease duration were 45.5 ± 13.6 and 14.6 ± 11.8 years, respectively. At the time of assessment, 68.5% had involvement limited to the neck, shoulder(s), and proximal arm(s), whereas 47.4% had dystonia limited to the neck. The remaining 31.5% of the individuals exhibited more extensive anatomical spread. A head tremor was noted in 62% of the patients. Head tremor and laryngeal dystonia were more common in females. Psychiatric comorbidities, mainly depression and anxiety, were reported by 32% of the participants and were more common in females. Family histories of dystonia, parkinsonian disorder, and tremor were present in 14%, 11%, and 29% of the patients, respectively. Pathogenic or likely pathogenic SVs in THAP1, TOR1A, and GNAL were identified in 8 participants (0.8%). Two individuals harbored novel missense SVs in Exon 5 of TOR1A. Synonymous and noncoding SVs in THAP1 and GNAL were identified in 4% of the cohort. CONCLUSIONS: Head tremor, laryngeal dystonia, and psychiatric comorbidities are more common in female participants with CD. Coding and noncoding variants in GNAL, THAP1, and TOR1A make small contributions to the pathogenesis of CD.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.007
Threshold uncertainty score0.372

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.014
GPT teacher head0.297
Teacher spread0.284 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it