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 distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.002 |
| Science and technology studies | 0.000 | 0.002 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".