P.078 Children with Trisomy 21 and Lennox-Gastaut Syndrome with predominant myoclonic seizures
Bibliographic record
Abstract
Background: Background: Lennox-Gastaut syndrome (LGS) is a severe form of pediatric epilepsy that is classically defined by a triad of drug-resistant seizures, characteristic EEG patterns, and intellectual disability. Long-term prognosis is generally poor with progressive intellectual deterioration and persistent seizures. At present, there are few reported cases of LGS and Trisomy 21 (T21) in the literature. To further delineate the spectrum of epilepsy in T21, we reviewed children with T21 and LGS at one center over 28 years. Methods: Methods: This is a retrospective case series. At our institution, all EEG results are entered into a database, which was queried for patients with T21 from 1992-2019. Pertinent electro-clinical data was obtained from medical records. Results: Results: 63 patients with T21 and epilepsy, 6 (10%) had LGS and were included in the study. Four of the six patients were male and 5/6, had neuro-imaging, which was normal. Follow-up ranged from 3-20 years. Notably, 5/6 had predominant myoclonic seizures throughout the course of their epilepsy, associated with generalized spike-wave discharges. Conclusions: Conclusions: Myoclonic seizures appear to be a predominant seizure type in patients with T21, suggestive that T21 patients may have a unique pattern of LGS.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot 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.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.007 | 0.001 |
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.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
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".