Hemimegalencephaly: Adult Evolution (P3.234)
Bibliographic record
Abstract
OBJECTIVE/BACKGROUND: Provide improved characterization of the evolution of Hemimegalencephaly (HME) in adult patients. HME is a rare neurological migration disorder resulting in the enlargement of one hemisphere. Depending on the severity of the enlargement, clinical manifestations may include macrocephaly, colpocephaly, epilepsy, and impaired psychomotor development. HME may also be associated with various neurocutaneous syndromes. One neurocutaneous syndrome is Hypomelanosis of Ito (HI). The main characteristics of HI are hypopigmented skin areas along the lines of Blaschko, which appear as streaks and patches. DESIGN/METHODS: We retrospectively examined adult patients with hemimegalencephaly by evaluating their current cognitive development, seizure control, documentation of therapies for seizure management and their outcomes. RESULTS: Five patients were included, 3 males, mean age ±23.8. Four patients had hemimegalencephaly associated with neurocutaneous syndromes, while one patient had isolated hemimegalencephaly. Patients with a seizure onset age past seven years old had better seizure control and psychomotor development in adulthood, in comparison to patients with seizure onset within the first year of life. CONCLUSIONS: In our small sample of adult hemimegalencephaly patients, age of seizure onset, cognitive disability, and seizure control were associated. Additionally, the isolated hemimegalencephaly had a better outcome compared to syndromic cases.
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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 | 0.000 |
| 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) | 0.002 | 0.000 |
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.
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".