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Record W4417274172 · doi:10.3174/ajnr.a9127

Toward a Neuroimaging Consensus for the Work-up of Adult Genetic Leukoencephalopathies on Behalf of the White Matter Rounds Network: State of Practice

2025· article· en· W4417274172 on OpenAlexafffund
Laura Airas, Enrique Álvarez, Jack P. Antel, David Araújo, Geneviève Bernard, Hayet Boudjani, Bernard Brais, Sirio Cocozza, John R. Corboy, Giulia Fadda, Jaime Imitola, Erin E. Longbrake, Gabrielle Macaron, Sridar Narayanan, Jennifer Orthmann‐Murphy, Johanna Ortiz Jiménez, Natalia Shor, C. Uggetti, Sunita Venkateswaran, Nagwa Wilson, Elka Miller, Roberta La Piana

Bibliographic record

VenueAmerican Journal of Neuroradiology · 2025
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicRNA regulation and disease
Canadian institutionsHospital for Sick ChildrenWestern UniversityQueen's UniversityJewish General HospitalUniversity of OttawaMontreal Neurological Institute and HospitalOttawa HospitalChildren's Hospital of Eastern OntarioCentre Hospitalier de l’Université de MontréalMcGill University
FundersCanadian Institutes of Health Research
KeywordsNeuroimagingFluid-attenuated inversion recoveryWhite matterLumbar punctureSagittal planeProtocol (science)NeuroradiologyMagnetic resonance imaging

Abstract

fetched live from OpenAlex

BACKGROUND: Adult-onset genetic leukoencephalopathies are frequently misdiagnosed due to their overlap with more common acquired white matter diseases. The White Matter (WM) Rounds Network includes clinicians and scientists from more than 15 centers around the world who meet monthly to discuss undiagnosed white matter diseases. A key barrier to optimizing diagnosis that arose during these monthly discussions was the lack of MRI protocol standardization between institutions. We aimed to: 1) assess the state of practice of current MR protocols for investigating suspected genetic leukoencephalopathies and 2) propose a core standard protocol. METHODS: We used a Delphi method to facilitate group judgements. We submitted a survey for feedback to a panel of neuroimaging experts whose final version was circulated to the entire WM Rounds Network. The results were analyzed, and specific recommendations were put forward during Delphi rounds, for which the stop criterion was 75% agreement. KEY MESSAGE: The MR protocols had an average magnet time of 40 minutes (range 25-60) and included: 3-dimensional (3D) T1 MPRAGE and 3D FLAIR obtained in the sagittal plane, axial T2 and T2-FLAIR, axial DWI/ADC, and axial SWI. Cervical and thoracic spine imaging were also frequently performed. A standardized core imaging protocol inclusive of the above-listed sequences would help harmonize sequence acquisition across institutions and promote cost-effective optimization of the imaging work-up of adult-onset leukoencephalopathies. Four additional recommendations were proposed: 1) contrast-enhanced T1 sequences should be performed for patients with strong clinical and/or radiologic suspicion of adult genetic leukoencephalopathy; 2) cervical and thoracic spine MRI may provide diagnostic value; 3) head CT is of little added value and should not be included routinely; and 4) MR spectroscopy, diffusion tensor imaging, and other advanced imaging techniques are not yet ready to be implemented in the protocol but may be helpful in supporting a working diagnosis.

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.

How this classification was reachedexpand

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 score0.276
Threshold uncertainty score0.301

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 studies0.0000.001
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 head0.011
GPT teacher head0.267
Teacher spread0.257 · 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

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations0
Published2025
Admission routes2
Has abstractyes

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