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Charles Bonnet syndrome in patients with Stargardt disease: prevalence and risk factors
  1. Patty P A Dhooge1,2,
  2. Rob J Teunisse3,
  3. Bart Liefers4,
  4. Stanley Lambertus1,
  5. Nathalie M Bax1,
  6. Carel B Hoyng1,
  7. Johannes R M Cruysberg1,
  8. B Jeroen Klevering1
  1. 1 Department of Ophthalmology, Radboudumc, Nijmegen, The Netherlands
  2. 2 Donders Insitute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands
  3. 3 Department of Old Age Psychiatry, GGNet Network for Mental Health Care in East Gelderland and Zutphen, Warnsveld, The Netherlands
  4. 4 Diagnostic Image Analysis Group, Department of Radiology, Radboudumc, Nijmegen, The Netherlands
  1. Correspondence to B Jeroen Klevering, Department of Ophthalmology, Radboudumc, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands; jeroen.klevering{at}


Aims To describe the prevalence of the Charles Bonnet syndrome (CBS) and search for potential CBS risk factors in a Dutch Stargardt disease (STGD1) cohort.

Methods Eighty-three patients with STGD1 were screened for CBS. They underwent a full eye examination. All patients completed the social functioning domain of the 36-Item Short Form Health Survey questionnaire. Participants suspected of CBS were interviewed to further evaluate their visual hallucinations.

Results CBS prevalence was 8.4%. Six out of seven patients with CBS were women. CBS was not associated with age (p=0.279, Mann-Whitney). Patients with CBS had a significant lower social functioning score (p<0.05, Mann-Whitney). All seven patients with CBS were in the category of vision impairment (visual acuity <6/12, but ≥3/60). Moreover, first hallucinations manifested after a drop in visual acuity. The retinal atrophic area of the worst eye tended to be lower in the CBS group (range 0.11–9.86 mm2) as compared with controls (range 0–180 mm2). There was no relation between the position of the scotoma and the location of the visual hallucinations.

Conclusion The relative high CBS prevalence in STGD1 suggests that CBS may be more prevalent in younger ophthalmic patients than currently presumed. In this specific group of patients, we established social isolation and acquired vision impairment as risk factors for CBS. There was a female preponderance among patients with CBS. Age and retinal pigment epithelium atrophy were not identified as significant risk factors. We should actively diagnose CBS in patients of any age who fulfil the criteria for the category vision impairment, especially in cases where social isolation is suspected.

  • retina
  • dystrophy
  • visual perception
  • macula

Data availability statement

Data are available upon reasonable request. Please contact the corresponding author.

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Data availability statement

Data are available upon reasonable request. Please contact the corresponding author.

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  • Contributors PD, RJT, JRMC and JK designed the study, PD, SL and NMB collected the data. All authors contributed to the interpretation of the results. PD and JK took the lead in writing the manuscript with input from all authors.

  • Funding This study was supported by Stichting AF Deutman Oogheelkunde Researchfonds Nijmegen (grant number 18-5-2018).

  • Disclaimer The sponsor or funding organisation had no role in the design or conduct of this research.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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