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Record W2012578107 · doi:10.1001/archopht.122.5.790

Progressive Pigmentation of Chorioretinal Lesions in Aicardi Syndrome

2004· article· en· W2012578107 on OpenAlexaff
Luis H. Ospina

Bibliographic record

VenueArchives of Ophthalmology · 2004
Typearticle
Languageen
FieldMedicine
TopicNeurological Complications and Syndromes
Canadian institutionsBC Children's Hospital
Fundersnot available
KeywordsMedicinePigmentation disorderOphthalmologyPathology

Abstract

fetched live from OpenAlex

3-MONTH-OLD FEmale infant was referred to the ophthalmology unit as partoftheinvestigationforinfantilespasms.Ocularexaminationshowednormalvisualattentiveness and pupillary function. Theopticnerveswereverticallyoval and there were multiple chorioretinal lacunae adjacent to the discs (Figure 1). Neuroimages revealed absence of the corpus callosum. Aicardisyndromewasdiagnosed.By the age of 6 years devastating neurological deterioration had occurred. Reexamination of the retinas at this time revealed increased pigmentation of the retinal lesions, even within the lacunae (Figure 2). COMMENT In 1965 Aicardi and associates 1 describedasyndromeconsistingofcallosal agenesis, chorioretinal lacunae,andinfantilespasms.Sincethen many cases of this X-linked recessivedisorderhavebeenreportedand the spectrum of ophthalmological and neurological findings is now broad. Becausethechorioretinallacunae are characteristic, their recognition is critical. Aicardi et al 1 first described these lesions as areas of “pseudotoxoplasmic”atrophicchoroiditis. They may vary in size from 0.1toseveraldiscdiametersandtend toclusteraroundtheopticnerve 2 or even involve it. The so-called colobomas of the optic nerves do not resemble the fusion defect that results in true colobomas. Although the lacunae of Aicardi syndrome have been likened to the pigmentary changes associated with infectious chorioretinopathies, 3 they really in no way resemble the harsh

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 score0.199
Threshold uncertainty score0.239

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 head0.032
GPT teacher head0.328
Teacher spread0.295 · 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

Citations4
Published2004
Admission routes1
Has abstractyes

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