Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids Presenting With Predominantly Neuro-Ophthalmic Features
Bibliographic record
Abstract
BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare and poorly understood inflammatory disorder of the central nervous system centered on the pons. It has a characteristic imaging appearance with enhancing and T2-hyperintense punctate and curvilinear lesions in the pons. The lesions lack restricted diffusion and have relatively little perilesional edema. Although patients typically present with gait ataxia and other sequelae of brainstem inflammation including diplopia, there is scant literature focusing on patients who present with primarily neuro-ophthalmic manifestations. METHODS: Case series of 3 patients presenting with diplopia who had a final diagnosis of CLIPPERS. RESULTS: Case descriptions of a 71-year-old man, 61-year-old woman, and 38-year-old man are reported. Diplopia was the chief presenting complaint, owing to internuclear ophthalmoplegia, sixth nerve palsy, or skew deviation. All patients had nystagmus and gait ataxia. Brain MRI displayed punctate or curvilinear enhancement of pontine/middle cerebellar peduncle lesions without restricted diffusion. All patients achieved rapid improvement after corticosteroid treatment. CONCLUSIONS: In 3 patients with CLIPPERS, the main presenting complaint was diplopia. The distinctive imaging signs led to a strong presumption of CLIPPERS, permitting a truncated evaluation and early corticosteroid treatment, which provided rapid reversal of clinical and imaging manifestations.
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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 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 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".