Retrospective Chart Review of Neurodevelopmental Outcomes in Children with Congenital Cerebellar Malformations (P2.251)
Bibliographic record
Abstract
OBJECTIVE:To assess whether specific features on imaging in patients with congenital cerebellar malformations (CCMs) are correlated with neurodevelopmental outcomes. BACKGROUND: Neurodevelopmental outcomes of children with cerebellar malformations remains poorly defined, consequently limited prognostic information is available. DESIGN/METHODS: Hospital records of patients with CCMs at one pediatric tertiary hospital were reviewed. A single pediatric neuroradiologist systematically reviewed all neuroimaging and grouped patients in subcategories based on specific regions of cerebellar involvement. All patients with ischemic, destructive or progressive cerebellar lesions were excluded. Statistical analysis included chi square and bivariate spearman correlation. RESULTS: The sample contained 72 children (41 males and 31 females, age range at last assessment 0.23-21.33 years, mean age 9.14 years). Subgroups included: isolated vermis hypoplasia (n=20), cerebellar hypoplasia (vermis and hemispheres, n=28), unilateral cerebellar hemisphere malformation (n=2), dandy walker malformation (n=10), mega cisterna magna (n=3), posterior fossa retrocerebellar cyst (n=8), molar tooth malformation (n=5), rhombencephalosynapsis (n=1), pontine hypoplasia (n=10). Overall, 77.5[percnt] had GDD and 60[percnt] had intellectual disability (ID). Increased severity of GDD was associated with the presence of molar tooth malformation (p=0.009), cerebellar hypoplasia (p=0.005) and pontine hypoplasia (p=0.009). Surprisingly, the presence of supratentorial abnormalities was not significantly associated with worse neurodevelopmental outcomes. Isolated vermis hypoplasia was associated with a better motor outcome (p=0.022). Autism spectrum disorder (ASD) was present in 16.7[percnt] of the cohort. Children with isolated vermis hypoplasia had a higher rate of ASD (29[percnt], p=0.125), whereas presence of cerebellar hypoplasia involving the hemispheres was associated with a lower rate of ASD (0[percnt], p=0.008). The presence of chromosomal abnormalities was associated with increased GDD and ID severity (p=0.007 and p=0.033 respectively) and intractable seizures (p=0.033). CONCLUSIONS: Our findings support that children with CCMs have a high prevalence of neurological, developmental, cognitive and social-behavioral deficits. The presence of specific features on imaging can aid in prognostication.
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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 | 0.001 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.002 | 0.003 |
| 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".