A Case of CLIPPERS Syndrome with Atypical Distribution Associated with Multiple Myeloma (P6.111)
Bibliographic record
Abstract
Background: CLIPPERS syndrome (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) is a rare neurological disorder first described by Pittock et al. in 2010. Its distinguishing features include a highly characteristic MRI image of punctuate and/or curvilinear gadolinium enhancing lesions ("peppering") visible on T2 and FLAIR affecting predominantly the brainstem, as well as a rapid and significant response to corticosteroids. The core clinical features include gait ataxia, dysarthria and diplopia. Case Report: We report the case of a 52-year-old man who presented with subacute gait ataxia, diplopia, dysarthria, psychomotor impairment and weight loss. The MRI showed the typical "peppering" pattern, however, the imaging abnormalities were predominantly located in the supratentorial region. A thorough workup was done, including a CSF cytology and flow cytometry, a chest CT, a PET Scan and paraneoplastic antibodies amongst others. Unfortunately, the CNS biopsy was declined by the patient and his family and could not be performed. Furthermore, the CSF cell count showed a non-homogenous lymphocytosis (104 cells/µL). A protein immunoelectrophoresis revealed a IgG monoclonal gammapathy and a bone marrow biopsy revealed a cellularity of 20[percnt] clonal plasma cells compatible with multiple myeloma. The patient was started on corticosteroids and had a very favourable clinical and radiological evolution. Conclusions: In conclusion, the presented patient had clinical features and an MRI image compatible with CLIPPERS syndrome and an exhaustive laboratory and radiological evaluation to eliminate alternative diagnoses. However, certain atypical features were noted including, the pattern distribution, the plasma cell dyscrasia, the high CSF lymphocytes count and the weight loss. To our knowledge it is only the second reported case of CLIPPERS associated with Multiple Myeloma and only one of the few cases of supratentorial CLIPPERS.
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.003 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.002 | 0.002 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.004 | 0.002 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
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".