Stroke in children with moyamoya and down syndrome; 3 cases and review of the literature
Bibliographic record
Abstract
Background and Aims: There are few retrospective studies describing clinical-radiological features and outcomes of children with Down Syndrome (DS) and Moyamoya (MM). Our aims are to present three cases of DS/MM from our institution, perform a review of the literature to compile similar case reports, determine if there is a clinical-radiological syndrome specific to DS/MM, and determine if this association is related to different outcomes. Methods: Our clinical database was reviewed between 2017-2019 for patients with DS/MM and cross-referenced with our radiology PACS database. We then reviewed the clinical data, imaging, treatment, and outcomes of previously 38-reported cases in English world literature with DS/MM from 1977 to 2018 using PubMed, Ovid, Medline, and Embase. Results: Case 1: Six year-old girl with focal status epilepticus, left MCA infarction and bilateral MM. Case 2: Three year-old boy with right hemiparesis, left MCA infarction and bilateral MM. Case 3: Thirteen year-old boy with right leg monoparesis, MRI-A: left ACA infarction and left ICA-MCA incipient MM. Case 1 & 2: managed with multiple burr holes (MBH). Case 1: surgery was performed 5 days after initial stroke due to rapid progression. Case 3: treated conservatively. On follow-up, all children had minimal deficit and continued on ASA. We reviewed 38 DS/MM cases found in the literature: 30 children presented with hemiparesis and 12 were treated surgically. Conclusions: Our findings are suggestive that DS/MM presentation and severity is variable with relatively good outcomes post-surgery. To our knowledge, Case 1 is the first reported child with DS/MM treated with urgent surgical MBH revascularization.
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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) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.010 | 0.008 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 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".