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Record W4367303539 · doi:10.1212/wnl.0000000000202017

Incidental Detection of Anti-IgLON5: A Diagnostic and Therapeutic Dilemma (P5-5.021)

2023· article· en· W4367303539 on OpenAlexaff
Samir Alkabie, Brian J. Murray, Adrian Budhram

Bibliographic record

VenueNeurology · 2023
Typearticle
Languageen
FieldMedicine
TopicAutoimmune Neurological Disorders and Treatments
Canadian institutionsHealth Sciences CentreUniversity of TorontoSunnybrook Health Science CentreWestern University
Fundersnot available
KeywordsMedicineEpilepsyPolysomnographyParasomniaTemporal lobeElectroencephalographyPediatricsPsychiatry

Abstract

fetched live from OpenAlex

Objective: To report a patient in whom anti-IgLON5 was incidentally detected as part of work-up for possible autoimmune-associated epilepsy. Background: Anti-IgLON5 disease has recently been characterized. Core manifestations include sleep disturbances, bulbar dysfunction, gait instability, chorea and cognitive decline, while seizures are not typical. Response to immunotherapy is usually suboptimal and prognosis is frequently poor, although early treatment could improve outcomes. Design/Methods: Case report. Results: A 49-year-old woman had an 18-year-history of temporal lobe epilepsy that developed after cervical cancer. Brain MRI was unremarkable but EEG showed right temporal epileptiform discharges and PET showed right temporal hypometabolism, compatible with right temporal seizure origin. Due to seizure refractoriness and history of cancer, comprehensive neural antibody testing for possible autoimmune-associated epilepsy was pursued and revealed anti-IgLON5 in serum and cerebrospinal fluid. On directed history, the patient reported snoring and insomnia that had developed two years prior, which were symptoms she had not initially volunteered because she felt they were mild and stable. Neurologic examination was unremarkable. The patient received a six-week trial of corticosteroids, without improvement in seizure frequency or sleep disturbances. Polysomnography revealed minimal sleep disordered breathing, mild arousals from slow wave sleep, unusually prominent beta activity in REM sleep, and excessive fragmentary myoclonus, which may represent early anti-IgLON5 disease. Conclusions: Diagnostically, we hypothesize that anti-IgLON5 is unrelated to chronic temporal lobe epilepsy in our patient, which we suspect is idiopathic. However, this antibody may explain sleep findings, and the mildness of her symptoms referable to anti-IgLON5 disease creates a unique therapeutic dilemma. In discussion with the patient, we have opted to monitor clinically and with polysomnography, and to consider institution of further immunotherapy if there is demonstrable disease progression. Future study into the natural history of anti-IgLON5 disease is needed to determine the optimal management of patients with mild symptoms. Disclosure: Dr. Alkabie has nothing to disclose. The institution of Dr. Murray has received research support from Wake Up Narcolepsy. Dr. Murray has received publishing royalties from a publication relating to health care. Dr. Budhram 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)0.001
metaresearch head score (Gemma)0.003
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Case report · Consensus signal: Case report
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.006
Threshold uncertainty score0.008

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0010.003
Meta-epidemiology (narrow)0.0020.001
Meta-epidemiology (broad)0.0010.001
Bibliometrics0.0010.001
Science and technology studies0.0020.001
Scholarly communication0.0010.002
Open science0.0010.001
Research integrity0.0060.003
Insufficient payload (model declined to judge)0.0020.001

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 head0.017
GPT teacher head0.262
Teacher spread0.245 · 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 designCase report
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
Published2023
Admission routes1
Has abstractyes

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