P.021 Childhood small vessel primary angiitis of the CNS: a potentially treatable cause of super-refractory status epilepticus
Bibliographic record
Abstract
Background: Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory disease of brain vessels. The small vessel subtype is diagnosed on brain biopsy and often presents with cognitive and behavioural changes, headaches and seizures. However, there are few reported cases of super-refractory status epilepticus. Methods: We present a case of small vessel cPACNS complicated by super-refractory status epilepticus and review the literature. Results: Our patient is a previously healthy 11-year-old boy who presented with new-onset seizures and encephalopathy in the context of fever. He developed super-refractory status epilepticus, requiring burst suppression for four weeks with various IV infusions. During this time, he was on the ketogenic diet and tried eight anti-seizure medications. Extensive investigations included brain biopsy confirming small vessel cPACNS. He was treated with IV methylprednisolone, oral steroids, IVIG, and cyclophosphamide. After prolonged rehabilitation, he recovered almost completely and has a normal neurological examination with no epileptiform activity on EEG. Conclusions: Small vessel cPACNS should be considered in the differential diagnosis of super-refractory status epilepticus. Despite being in SE for four weeks, symptomatic management of seizures and immunosuppression to treat the underlying pathology resulted in favourable neurological outcomes. This is one of the longest cases of SE in small vessel cPACNS in the literature.
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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 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.027 | 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".