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Record W4226425030 · doi:10.1055/s-0042-1746208

The Epileptology of Aicardi–Goutières Syndrome: Electroclinical Phenotype and Clinical–Radiological Correlation

2022· article· en· W4226425030 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.

affAt least one author lists a Canadian institution in the pinned OpenAlex snapshot.

Bibliographic record

VenueNeuropediatrics · 2022
Typearticle
Languageen
FieldImmunology and Microbiology
Topicinterferon and immune responses
Canadian institutionsUniversité de MontréalMontreal Neurological Institute and Hospital
Fundersnot available
KeywordsMedicinePhenotypeEpilepsyNeuroscienceElectroencephalographyClinical phenotypePediatricsElectrophysiologyPsychiatryPsychologyGeneticsInternal medicineBiologyGene

Abstract

fetched live from OpenAlex

Objectives: Aicardi–Goutières syndrome (AGS) is a monogenic interferonopathy. Most individuals affected by AGS show various degrees of neurological symptoms. Although at least a quarter of patients with AGS experiences epileptic seizures, their phenotypic and electrophysiological characterizations remain elusive. To encompass this lack of knowledge, we collected patients with established diagnosis of typical AGS and detailed electroclinical data with the aim to characterize epilepsy phenotypes and electroencephalographic patterns and identify possible correlations between them and clinical, genetic, and neuroradiologic features.

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)0.001
metaresearch head score (Gemma)0.002
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.436
Threshold uncertainty score0.426

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0010.002
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.000
Science and technology studies0.0000.001
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.001
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 head0.268
Teacher spread0.254 · 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