The incidence and characteristics of Charles Bonnet syndrome in Leber’s hereditary optic neuropathy in a large cohort
Bibliographic record
Abstract
Abstract Purpose To investigate the incidence and characteristics of Charles Bonnet syndrome (CBS) in patients with Leber’s hereditary optic neuropathy (LHON) via an online questionnaire shared with the LHON patient community. Methods A recently validated French‐Canadian CBS screening questionnaire was adapted to an online bilingual (English‐French) format while being tailored to LHON patients. It was distributed to numerous mailing lists and online communities consisting of LHON patients. The 62‐item questionnaire was organized into 3 parts to cover 9 dimensions: Demographics, Screening questions for CBS, Characteristics of hallucinations, Psychological impact, Psychopathological origin, Coping strategies, Context of appearance of hallucinations, Time‐related matters, Psychosocial support. Quantitative and qualitative response data were used for cross‐sectional analysis. Results A total of 63 LHON patients (71% male) completed the questionnaire – with 56% screening positive for CBS. Comparing CBS‐positive and CBS‐negative patients revealed significantly different LHON mutation distributions (p = 0.01137). Among CBS‐positive patients, 74% reported experiencing hallucinations for at least one year. Furthermore, 37% said the images disturb their sleep, while 49% reported they negatively affect their mood. Additionally, only 49% had heard of CBS before and just a single patient (3%) had been diagnosed with CBS by a healthcare professional. Conclusions The results show there is a significant prevalence of CBS among LHON patients, but only a minority of these patients are being assessed and managed for the condition. There is also an indication that CBS tends to be long‐lasting and can be associated with various negative health outcomes. Early diagnosis and counseling of patients are important to help alleviate stress and anxiety related to CBS and improve quality of life.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot 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.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.005 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".