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Outstretched Index Finger - A Pointer is Peculiar Behaviour Characteristic of Angelman Syndrome in Adults – A Case Series Study

2019· article· en· W2990673862 on OpenAlexvenueno aff
Peter R. Martin

Bibliographic record

VenueJournal of Intellectual Disability - Diagnosis and Treatment · 2019
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicGenetic Syndromes and Imprinting
Canadian institutionsnot available
Fundersnot available
KeywordsAngelman syndromePointer (user interface)Index (typography)PsychologySeries (stratigraphy)Index fingerAudiologyComputer scienceMedicineArtificial intelligenceGeologyAnatomyWorld Wide Web

Abstract

fetched live from OpenAlex

Objective: To describe a behavioural feature, the outstretched pointing (and shaking) index finger, as a clue to the clinical diagnosis of Angelman syndrome (AS) in adults. Methods: A case series, consisting of 43 adult individuals with the clinical diagnosis of AS, approved by genetic testing is presented. In 16/43 patients the clinical diagnosis was made by the author, mostly due to behavioural signs, including the pointing gesture, which he had increasingly noticed as a behavioural key feature in his adult patients with AS. Conclusion: Pointing with the outstretched index finger is a communicative gesture frequently seen in adult persons with AS. Its absence is most likely due to more limited motor and/or intellectual functions, in phenotypes caused by deletions of 15q11-13 respectively. It can be seen as a clinical sign that significantly contributes to the diagnosis in adulthood.

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 score0.019
Threshold uncertainty score0.693

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 head0.014
GPT teacher head0.260
Teacher spread0.247 · 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
Published2019
Admission routes1
Has abstractyes

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