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The White Matter Rounds Experience: The Importance of a Multidisciplinary International Network to Accelerate the Diagnostic Process for Adult Patients with Rare White Matter Disorders (P1-1.Virtual)

2022· article· en· W4389404645 on OpenAlexaff
Yutong Huang, Paul S. Giacomini, Rami Massie, Sunita Venkateswaran, Roberta La Piana, Anne‐Marie Trudelle, Giulia Fadda, Maryam Sharifian, Hayet Boudjani, Laurence Poliquin‐Lasnier, Laura Airas, Alexander Saveriano, Myriam Levesque‐Roy, Matthias Georg Ziller, Elka Miller, Claudia Martinez‐Rios, Jorge Dávila, Nagwa Wilson, Erin E. Longbrake, Jack P. Antel, Bernard Brais, Geneviève Bernard, Carolina Rush

Bibliographic record

VenueNeurology · 2022
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicRNA regulation and disease
Canadian institutionsMcGill University Health CentreUniversity of OttawaUniversité LavalUniversité de MontréalHôpital Maisonneuve-RosemontClinique Neuro-OutaouaisMcGill UniversityMontreal Neurological Institute and Hospital
Fundersnot available
KeywordsWhite matterWhite (mutation)Multidisciplinary approachMillerPsychoanalysisMedicinePsychologyPediatricsPolitical scienceMagnetic resonance imagingLawChemistry

Abstract

fetched live from OpenAlex

Objective: To present the experience of the White Matter (WM) Rounds Network and demonstrate the usefulness of collaborative multidisciplinary international case discussion meetings in improving the diagnostic rate in patients with rare white matter disorders. Background: Adult patients with genetic leukoencephalopathies or atypical white matter abnormalities often experience considerable delays before receiving a final diagnosis, due to the rarity of their conditions and the clinical and radiological overlap between hereditary and acquired white matter disorders, especially multiple sclerosis (MS). In this context, a strong collaborative network between same-field experts is beneficial for shortening the diagnostic odyssey of these patients and for preventing misdiagnosis. The WM Rounds is a multidisciplinary network of physicians and scientists from North America, Europe, and Middle East, that gather every month to discuss patients with atypical white matter disorders and that form a strong collaborative network. Design/Methods: We retrospectively reviewed the demographic, clinical and radiological data of all the subjects presented at the WM Rounds since its creation in 2013. Results: Eighty-one patients (mean age 43.9) have been referred and discussed at the WM Rounds since 2013. In most cases, the referring physician was a geneticist (36/81; 44.4%) or an MS specialist (34/81; 41.9%). Thanks to the WM Rounds recommendations, a final diagnosis was made in 36/81 (44.4%) patients. Of these, 25 (69.4%) were diagnosed with a genetic neurological disease, six of whom (6/26, 23.1%) had been initially assessed in MS clinics. Conclusions: The experience of the WM Rounds Network demonstrates the benefit of joining efforts through collective discussions on challenging patient cases to increase the diagnostic rate and prevent misdiagnosis. Networks of this nature allow physicians and scientists to compare and share information on rare cases from across the world and provide a basis for future multicenter research studies. Disclosure: Miss Huang has nothing to disclose. Dr. Giacomini has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Innodem Neurosciences. Dr. Giacomini has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis. Dr. Giacomini has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for F. Hoffmann- La Roche. Dr. Giacomini has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Giacomini has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Giacomini has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squibb. Dr. Giacomini has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Giacomini has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for F. Hoffmann-La Roche. Dr. Giacomini has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Giacomini has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. Dr. Giacomini has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen. Dr. Giacomini has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for F. Hoffman-La Roche. Dr. Giacomini has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Novartis. Dr. Giacomini has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for EMD Serono. Dr. Giacomini has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Sanofi. Dr. Massie has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kye pharmaceutical. Dr. Massie has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alnylam Pharmaceutical. Dr. Massie has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Akcea Therapeutics. The institution of Dr. Massie has received research support from Alnylam Pharmaceuticals. Dr. Venkateswaran has nothing to disclose. Roberta La Piana has nothing to disclose. Dr. Trudelle has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squibb. Dr. Trudelle has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Trudelle has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Trudelle has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Trudelle has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi Genzyme. Dr. Trudelle has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Bristol Myers Squibb. Dr. Trudelle has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Trudelle has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Trudelle has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Dr. Trudelle has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi Genzyme. Dr. Fadda has nothing to disclose. Dr. Sharifiandorche has nothing to disclose. Dr. Boudjani has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen . Dr. Boudjani has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Poliquin-Lasnier has nothing to disclose. Dr. Airas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genzyme. Dr. Airas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Airas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Dr. Airas has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Saveriano has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Saveriano has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Saveriano has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi-Genzyme. Dr. Saveriano has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Saveriano has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD-Serono. Dr. Saveriano has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Levesque-Roy has nothing to disclose. Dr. Ziller has nothing to disclose. Elka Miller has nothing to disclose. Dr. Martinez-Rios has nothing to disclose. Dr. Davila has nothing to disclose. Dr. Longbrake has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Longbrake has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Longbrake has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Longbrake has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genzyme. Dr. Longbrake has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Dr. Longbrake has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Longbrake has received personal compensation in the range of $0-$499 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Annals of Neurology. The institution of Dr. Longbrake has received research support from Genentech. The institution of Dr. Longbrake has received research support from Race to Erase MS. The institution of Dr. Longbrake has received research support from NINDS K23. Dr. Longbrake has received personal compensation in the range of $500-$4,999 for serving as a Study Section Member with Department of Defense (CDMRP). Dr. Antel has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for sanofi, roche, biogen, wave,nervgen. The institution of Dr. Antel has received research support from Novartis canada bristol myers squib. Dr. Antel has a non-compensated relationship as a past president with ACTRIMS that is relevant to AAN interests or activities. Dr. Brais has nothing to disclose. The institution of Dr. Bernard has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Passage Bio. Dr. Bernard has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ionis. The institution of Dr. Bernard has received research support from Takeda. The institution of Dr. Bernard has received research support from Canadian Institutes of Health Research. The institution of Dr. Bernard has received research support from Montreal Children's Foundation. The institution of Dr. Bernard has received research support from Yaya Foundation for 4H Leukodystrophy. The institution of Dr. Bernard has received research support from Fondation Les Amis d'Elliot. The institution of Dr. Bernard has received research support from Pelizaeus-Merzbacher Disease Foundation. Dr. Bernard has received research support from Canadian Institutes of Health Research. Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pendopharm . Dr. Rush has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi . Roberta La Piana has nothing to disclose.

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 machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.005
metaresearch head score (Gemma)0.016
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Not applicable · Consensus signal: Not applicable
GenreCandidate signal: Other · Consensus signal: none
Teacher disagreement score0.120
Threshold uncertainty score0.400

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0050.016
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0010.000
Science and technology studies0.0020.001
Scholarly communication0.0040.003
Open science0.0010.003
Research integrity0.0060.006
Insufficient payload (model declined to judge)0.1200.054

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 head0.005
GPT teacher head0.244
Teacher spread0.239 · 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 source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designNot applicable
Domainnot available
GenreOther

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".

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Citations0
Published2022
Admission routes1
Has abstractyes

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