Ocular Myasthenia: It's Worth a Second Look
Bibliographic record
Abstract
Background/Purpose: Ocular myasthenia is a common presentation of myasthenia gravis (MG) in children. However, confirmation of diagnosis and successful treatment can be challenging. We report on an unusual case, requiring specialized laboratory tests and high doses of pyridostigmine. Methods: A 6-year-old girl presented with a history of right-sided ptosis and diplopia for three weeks. Symptoms slightly worsened over the day and showed rapid alteration (on-off-phenomenon). Past medical history and family history were unremarkable. Physical examination revealed ptosis and impaired elevation of the right eye, causing diplopia. Laboratory tests including ACh-receptor antibodies were negative, CSF, MRI and electrophysiology were normal. We started pyridostigmine 4 mg/kg/day and the patient was discharged without any remaining symptoms. After one week, the family reported a left-sided ptosis and diplopia. Again ACh-R antibodies were negative (ELISA) and the patient was put on prednisone 2 mg/kg/day, leaving the symptoms unchanged. Over the next 8 weeks, ptosis increased on both sides. No signs of generalized MG were noted at any time. Results: We decided to try a pyridostigmine dose of 8 mg/kg/day, which rapidly improved both ptosis and diplopia. However, ELISA AChR-antibodies remained negative. We next turned to a live cell-based assay (CBA) for ACh-R antibodies. Recent publications suggest higher sensitivity for live CBA compared with ELISA and fixed CBA, possibly conserving tertiary protein structure. Live CBA was positive for AChR-antibodies in our patient, confirming the diagnosis of ocular MG. The patient remained symptom-free for 12-month follow-up. Conclusion: In patients with a history suggestive of ocular MG and negative AChR antibodies in fixed essays, live cell based assays can help to confirm the diagnosis. Pyridostigmine at doses higher than usually recommended might be required for successful treatment. Publication History Article published online: 13 November 2023 © 2023. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany
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 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 | 0.001 | 0.005 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.002 | 0.002 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.002 | 0.002 |
| Scholarly communication | 0.003 | 0.006 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.011 | 0.009 |
| Insufficient payload (model declined to judge) | 0.008 | 0.003 |
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".