Bibliographic record
Abstract
Background: Progressive encephalomyelitis, rigidity, and myoclonus (PERM) is a variant stiff-person plus syndrome consisting of brainstem and pyrimidal dysfunction, muscular rigidity, stimulus-evoked spasms, and dysautonomia. Continuous motor-unit activity from ectopic anterior horn cell discharges underlies the myotomal hyperactivity. Case Report: A 51-year-old man with an 8-year history of “spasmodic” mid and low back pain presented with increasing stiffness, hyperekplexia-induced opisthotonus-like posturing, urinary retention, long-tract motor signs, and diplopia. He had a recent history of biopsy-confirmed leucocytoclastic vasculitis-associated diffuse maculopapular rash. He responded well to IVIg treatment manifested by improved 1) gait fluidity, 2) bowel and bladder function 3) tolerance to startle, and 4) vision. Results: Serological testing revealed positive anti-glycine receptor antibodies. Anti-glutamic acid decarboxylase and voltage-gated K+-channel antibodies were absent. A chest CT was unremarkable. Conclusions: This is the second case of seropositive PERM in Canada and the first associated with leucocytoclastic vasculitis. Pathologic findings in PERM reveal perivascular lymphocytic cuffing in the rhombencephalon and spinal cord. Glycine receptor localization correlates with neurologic dysfunction. Mid-brain involvement may underlie the autonomic dysfunction but evidence of direct glycinergic inhibition of the external urethral sphincter in Onuf’s nucleus may be responsible for urinary retention.
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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) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.003 | 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".