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Record W1846940247 · doi:10.1017/cbo9780511545900.016

Neurofibromatosis

2008· book-chapter· en· W1846940247 on OpenAlexaff
Bartlett D. Moore, John M. Slopis

Bibliographic record

VenueCambridge University Press eBooks · 2008
Typebook-chapter
Languageen
FieldMedicine
TopicNeurofibromatosis and Schwannoma Cases
Canadian institutionsUniversity of Toronto
Fundersnot available
KeywordsNeurofibromatosisMedicinePathology

Abstract

fetched live from OpenAlex

Introduction History Neurofibromatosis (NF) is a common neurocutaneous disorder that has an incidence of approximately 1 in 4000 (Mulvihill et al ., 1990). Although NF has been postulated to have as many as eight different forms (Riccardi & Eichner, 1986), this classification system has not been widely adopted. Neurofibromatosis is a group of genetic disorders including NF type I (NF-I), NF type II (NF-II), and multiple schwannomatosis, each with distinctly different genetic mutations and pathologic bases. The NF-I gene is nearly ubiquitous in human tissues and so impacts virtually all organ systems. NF-I is particularly interesting to neurocognitive scientists because of its characteristic phenotypical abnormalities in development of form and function in brain. NF-II and multiple schwannomatosis are essentially disorders of cranial nerves, peripheral nerves, and meningeal tissues with no associated cognitive abnormalities and so these disorders will be excluded from this discussion. The original term neurofibromatosis was derived at the turn of the last century but the disorder is also called von Recklinghausen's disease because the condition was described in the late 1800s clinically and scientifically by Friedrich Daniel von Recklinghausen (Cawthon et al ., 1990; Crump, 1981; Viskochil et al ., 1990). The molecular genetic basis of distinguishing clinical features of NF-I was localized to chromosome 17 in 1990 by two teams of investigators (Viskochil et al ., 1990; Wallace et al ., 1990).

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)0.000
metaresearch head score (Gemma)0.000
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: none
GenreCandidate signal: Review · Consensus signal: none
Teacher disagreement score0.080
Threshold uncertainty score0.267

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.001
Science and technology studies0.0010.000
Scholarly communication0.0010.001
Open science0.0010.001
Research integrity0.0010.001
Insufficient payload (model declined to judge)0.0800.046

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 head0.031
GPT teacher head0.209
Teacher spread0.179 · 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
GenreReview

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

Citations2
Published2008
Admission routes1
Has abstractyes

Explore more

Same venueCambridge University Press eBooksSame topicNeurofibromatosis and Schwannoma CasesFrench-language works237,207