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Record W4415484294 · doi:10.1212/nxg.0000000000200313

Developing a National Network for Leukodystrophy Research and Care in Canada

2025· article· en· W4415484294 on OpenAlex
Alexandra Chapleau, Adam Le, Justin Simo, S. Venkateswaran, Thierry Lacaze‐Masmonteil, Valerio E. C. Piscopo, Samuel Gauthier, Felipe Villa Tobón, Sabrina Alam, Laura Lentini, Bernard Brais, Carl Ernst, John J. Mitchell, Donald C. Vinh, Timothy E. Kennedy, Naomi Goloff, Badawy Riham, Ron Chapleau, Valerie Greger, Josée Della Rocca, Lynda-Marie Louis, Ashley Dike, L McIntyre, David F. McIntyre, Jérome Tardif, Émilie Lapointe, Valérie Loignon, Ghalib Bardai, Sophie Contant, Thomas M. Durcan, Roberta La Piana, Geneviève Bernard

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.
aboutThe title or abstract carries a Canadian signal from the geographic lexicon.

Bibliographic record

VenueNeurology Genetics · 2025
Typearticle
Languageen
FieldImmunology and Microbiology
TopicNeutrophil, Myeloperoxidase and Oxidative Mechanisms
Canadian institutionsMontreal Children's HospitalOakville-Trafalgar Memorial HospitalChild and Family Research InstituteLondon Health Sciences CentreWestern UniversityMcGill UniversityMcGill University Health CentreChildren's Hospital of Western OntarioMontreal Neurological Institute and Hospital
Fundersnot available
KeywordsExcellenceLeukodystrophyWhite paperMultidisciplinary approachCenter of excellenceDiseaseTranslational research

Abstract

fetched live from OpenAlex

Leukodystrophies (LDs) are a group of rare, genetic disorders unified by their hallmark involvement of the cerebral white matter. They are typically characterized as progressive disorders, resulting in severe neurologic decline and premature death within months to years after onset. Managing LDs therefore requires lifelong, multidisciplinary care, a challenge compounded by their rarity and phenotypic heterogeneity, for which detailed clinical and scientific information is sometimes lacking. Research networks have proven useful in the rare disease community to unite efforts, increase awareness, and accelerate progress toward understanding and treating these often understudied conditions. Therefore, we established the Canadian Association for Research Excellence in Leukodystrophy (CARELeuko), a national network dedicated to improving LD care, research, and treatment within Canada. To better understand and address the most pressing needs for LDs in Canada, we engaged a diverse group of stakeholders including researchers, clinicians, and patient advocates to highlight and prioritize gaps in LD care and research. In this review, we discuss the key gaps identified in the Canadian LD landscape and outline strategies to address these challenges. This effort will inform the development of targeted initiatives aimed at improving outcomes for Canadian families affected by these debilitating disorders.

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: Not applicable · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.534
Threshold uncertainty score0.989

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 head0.044
GPT teacher head0.305
Teacher spread0.262 · 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