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Record W2607180485 · doi:10.1017/cjn.2015.164

Seropositive PERM associated with leucocytoclastic vasculitis

2015· article· en· W2607180485 on OpenAlexvenueaboutno aff
BK Steven

Bibliographic record

VenueCanadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · 2015
Typearticle
Languageen
FieldMedicine
TopicGlycogen Storage Diseases and Myoclonus
Canadian institutionsnot available
Fundersnot available
KeywordsMedicineVasculitisMyoclonusDysautonomiaUrinary urgencyPathologyAnesthesiaOveractive bladder

Abstract

fetched live from OpenAlex

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.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.003
metaresearch head score (Gemma)0.004
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesScience and technology studies
Consensus categoriesScience and technology studies
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.488
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0030.004
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0010.001
Science and technology studies0.0020.006
Scholarly communication0.0000.001
Open science0.0010.000
Research integrity0.0000.001
Insufficient payload (model declined to judge)0.0000.000

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.

Opus teacher head0.042
GPT teacher head0.271
Teacher spread0.228 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; both teacher heads agree on what is shown here.

Study designObservational
Domainnot available
GenreEmpirical

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".

Quick stats

Citations0
Published2015
Admission routes2
Has abstractyes

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