In response: Lennox‐Gastaut syndrome may be a curable, reversible epileptic encephalopathy
Bibliographic record
Abstract
Dear Editor: We appreciate the comments of Drs. Ng, Pati, and Fesler regarding our recent Epilepsia supplement, “Current Strategies for the Management of Lennox-Gastaut Syndrome,” and highlighting of their previously reported findings. Our working group recommends that the complete evaluation of patients with epilepsy is mandatory, particularly in children with early refractory seizures and encephalopathy. This requires expert neuroimaging along with extended history, neurophysiology, and supporting studies. As emphasized in references cited in the Epilepsia supplement, semiology such as gelastic seizures may well suggest specific underlying causes, but may not always be present. Similarly, the occurrence of different seizure types may evolve as children age. A variety of underlying etiologies, including hypothalamic hamartoma and cortical malformations, may present with features of Lennox-Gastaut syndrome (LGS). Despite being uncommon, discovering these cortical lesions/malformations and underlying metabolic abnormalities may lead to specific therapies. We also agree that early appropriate therapy, surgical or medical, may certainly alter the course of the progressive epileptic encephalopathies. The goal of the supplement was to emphasize the need for individualizing care of these children and adults with refractory and most frequently intractable epilepsy. Your comments add emphasis to our recommendations. Dr. Pellock is a consultant for Eisai, Jazz, King Pharmaceuticals, KV Pharmaceuticals, Marinus Pharmaceuticals, NeuroPace, Ortho McNeil/Johnson & Johnson, Lundbeck, Pfizer, Questcor, UCB Pharmaceuticals, and Valeant. He has participated in advisory boards for Eisai, Lundbeck, Questcor, UCB Pharmaceuticals, Valeant, Acorda, and Upsher-Smith. Dr. Pellock has received research grants from Eisai, Ortho McNeil/Johnson & Johnson, Lundbeck, Questcor, UCB Pharmaceuticals, and Valeant. Dr. Wheless has received grants from the National Institutes of Health (NIH), the National Institute of Neurological Disorders and Stroke, the Shainberg Foundation, GlaxoSmithKline, Cyberonics, Eisai, and Novartis; is a consultant for Lundbeck, Cyberonics, Pfizer, Eisai, Sunovion, Supernus, and Upsher-Smith; and is a member of the speaker's bureau for Lundbeck, Questcor, Cyberonics, and Eisai. Dr. Douglass received unrestricted educational grants from Eisai to conduct continuing medical education programs and to submit manuscripts from the proceedings of these meetings. Dr. Douglass also has received research support from Questcor Pharmaceuticals to study quality care indicators for infantile spasms. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.
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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 | 0.002 | 0.016 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.002 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.004 |
| Open science | 0.003 | 0.001 |
| Research integrity | 0.023 | 0.027 |
| Insufficient payload (model declined to judge) | 0.008 | 0.006 |
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".