Abstract
Purpose
To perform a systematic review of diagnostic criteria for the Charles Bonnet syndrome (CBS).
Recent findings
Across 33 studies that specified diagnostic criteria for CBS, hallucinations and vision loss were a common requirement, but there was considerable heterogeneity regarding hallucination properties (i.e., formed vs. unformed) and the severity of vision loss. The exclusion of confounding neuropsychiatric disorders was also common, but specific disorders and their method of ascertainment were variable.
Summary
There is considerable diagnostic heterogeneity for CBS in the literature. These differences have important implications for the results of observational and interventional studies of CBS and highlight the need for unified diagnostic criteria.
Similar content being viewed by others
Change history
04 October 2019
Shortly after publication, the authors became aware of several typographical errors in Table 1 which were introduced during the copy-editing process.
References and Recommended Reading
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
Hedges TR. Charles Bonnet, his life, and his syndrome. Surv Ophthalmol. 2007;52(1):111–4. https://doi.org/10.1016/j.survophthal.2006.10.007.
de Morsier G. The Charles Bonnet syndrome: visual hallucinations in the aged without mental deficiency. Ann Med Psychol (Paris). 1967;2(5):678–702.
Cogan DG. Visual hallucinations as release phenomena. Albrecht Von Graefes Arch Klin Exp Ophthalmol Albrecht Von Graefes Arch Clin Exp Ophthalmol. 1973;188(2):139–50.
Burke W. The neural basis of Charles Bonnet hallucinations: a hypothesis. J Neurol Neurosurg Psychiatry. 2002;73(5):535–41.
Schultz G, Melzack R. The Charles Bonnet syndrome: “phantom visual images”. Perception. 1991;20(6):809–25. https://doi.org/10.1068/p200809.
Menon GJ, Rahman I, Menon SJ, Dutton GN. Complex visual hallucinations in the visually impaired: the Charles Bonnet Syndrome. Surv Ophthalmol. 2003;48(1):58–72.
Levine AM. Visual hallucinations and cataracts. Ophthalmic Surg. 1980;11(2):95–8.
Crumbliss KE, Taussig MJ, Jay WM. Vision rehabilitation and Charles Bonnet Syndrome. Semin Ophthalmol. 2008;23(2):121–6. https://doi.org/10.1080/08820530801888170.
Ukai S, Yamamoto M, Tanaka M, Takeda M. Treatment of typical Charles bonnet syndrome with donepezil. Int Clin Psychopharmacol. 2004;19(6):355–7.
Bergman Y, Barak Y. Escitalopram for antipsychotic nonresponsive visual hallucinosis: eight patients suffering from Charles Bonnet syndrome. Int Psychogeriatr. 2013;25(9):1433–6. https://doi.org/10.1017/S1041610213000719.
Hori H, Terao T, Shiraishi Y, Nakamura J. Treatment of Charles bonnet syndrome with valproate. Int Clin Psychopharmacol. 2000;15(2):117–9.
Lepore FE. Spontaneous visual phenomena with visual loss: 104 patients with lesions of retinal and neural afferent pathways. Neurology. 1990;40(3 Pt 1):444–7.
McKeith IG, Boeve BF, Dickson DW, Halliday G, Taylor J-P, Weintraub D, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology. 2017;89(1):88–100. https://doi.org/10.1212/WNL.0000000000004058.
• Kosman KA, Silbersweig DA. Pseudo-Charles Bonnet syndrome with a frontal tumor: visual hallucinations, the brain, and the two-hit hypothesis. J Neuropsychiatr Clin Neurosci. 2018;30(1):84–6. https://doi.org/10.1176/appi.neuropsych.17040076This is a case report of visual hallucinations in a 52 year-old woman with vision loss due to severe bilateral optic atrophy from a large (> 6 cm) planum sphenoidale meningioma. The patient's hallucinations resolved after resection of her meningioma, despite persistence of her vison loss. The authors discuss the hypothesis that a “second hit” besides vision loss—in this case, frontal lobe dysfunction due to mass effect and vasogenic edema from her meningioma—is required to cause release hallucinations.
Moher D, Liberati A, Tetzlaff J, Altman DG, PRISMA Group. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. Ann Intern Med. 2009;151(4):264–9W64.
Abbott EJ, Connor GB, Artes PH, Abadi RV. Visual loss and visual hallucinations in patients with age-related macular degeneration (Charles Bonnet syndrome). Invest Ophthalmol Vis Sci. 2007;48(3):1416–23. https://doi.org/10.1167/iovs.06-0942.
Chen C-C, Liu H-C. Low-dose aripiprazole resolved complex hallucinations in the left visual field after right occipital infarction (Charles Bonnet syndrome). Psychogeriatr Off J Jpn Psychogeriatr Soc. 2011;11(2):116–8. https://doi.org/10.1111/j.1479-8301.2010.00353.x.
Cox TM, Ffytche DH. Negative outcome Charles Bonnet syndrome. Br J Ophthalmol. 2014;98(9):1236–9. https://doi.org/10.1136/bjophthalmol-2014-304920.
Eagan SM, Williams JA. The formed visual hallucinations associated with vision loss. Optom St Louis Mo. 2000;71(8):519–27.
Elflein HM, Rudy M, Lorenz K, Ponto KA, Scheurich A, Pitz S. Charles Bonnet’s syndrome: not only a condition of the elderly. Graefes Arch Clin Exp Ophthalmol Albrecht Von Graefes Arch Klin Exp Ophthalmol. 2016;254(8):1637–42. https://doi.org/10.1007/s00417-016-3387-x.
• ClinicalTrials.gov [Internet]. Bethesda (MD): National Library of Medicine (US). 2017 Feb 1 - . Identifier NCT03148249, A Trial of Patients With a Charles Bonnet Syndrome (CBS).https://clinicaltrials.gov/ct2/show/NCT03148249. Accessed April 3, 2019. This is the only clinical trial for Charles Bonnet syndrome registered on clinicaltrials.gov. Because it is a clinical trial, its inclusion and exclusion criteria form some of the strictest and most detailed diagnostic criteria (including a visual acuity cutoff, which few other studies have) for Charles Bonnet syndrome in the literature. The trial reportedly randomized patients with Charles Bonnet syndrome to consultation and treatment with a psychiatrist, and the primary outcome was quality of life. However, while it is listed as having been conducted between 2014 and 2017, its results have not been published to our knowledge.
Gilmour G, Schreiber C, Ewing C. An examination of the relationship between low vision and Charles Bonnet syndrome. Can J Ophthalmol J Can Ophtalmol. 2009;44(1):49–52. https://doi.org/10.3129/i08-169.
Gold K, Rabins PV. Isolated visual hallucinations and the Charles Bonnet syndrome: a review of the literature and presentation of six cases. Compr Psychiatry. 1989;30(1):90–8.
Holroyd S, Rabins PV, Finkelstein D, Nicholson MC, Chase GA, Wisniewski SC. Visual hallucinations in patients with macular degeneration. Am J Psychiatry. 1992;149(12):1701–6. https://doi.org/10.1176/ajp.149.12.1701.
Hou Y, Zhang Y. The prevalence and clinical characteristics of Charles Bonnet syndrome in Chinese patients. Gen Hosp Psychiatry. 2012;34(5):566–70. https://doi.org/10.1016/j.genhosppsych.2012.05.007.
Jackson ML, Drohan B, Agrawal K, Rhee DJ. Charles Bonnet syndrome and glaucoma. Ophthalmology. 2011;118(5):1005–5.e2. https://doi.org/10.1016/j.ophtha.2011.01.007.
Khan JC, Shahid H, Thurlby DA, Yates JRW, Moore AT. Charles Bonnet syndrome in age-related macular degeneration: the nature and frequency of images in subjects with end-stage disease. Ophthalmic Epidemiol. 2008;15(3):202–8. https://doi.org/10.1080/09286580801939320.
Leandro JE, Beato J, Pedrosa AC, Pinheiro-Costa J, Falcão M, Falcão-Reis F, et al. The Charles Bonnet syndrome in patients with neovascular age-related macular degeneration: association with proton pump inhibitors. Invest Ophthalmol Vis Sci. 2017;58(10):4138–42. https://doi.org/10.1167/iovs.16-21270.
Madill SA, Lascaratos G, Arden GB, Ffytche DH. Perceived color of hallucinations in the Charles Bonnet syndrome is related to residual color contrast sensitivity. J Neuro-Ophthalmol Off J North Am Neuro-Ophthalmol Soc. 2009;29(3):192–6. https://doi.org/10.1097/WNO.0b013e3181b1b2bf.
Miyaoka T, Furuya M, Kristian L, Wake R, Kawakami K, Nagahama M, et al. Yi-gan san for treatment of charles bonnet syndrome (visual hallucination due to vision loss): an open-label study. Clin Neuropharmacol. 2011;34(1):24–7. https://doi.org/10.1097/WNF.0b013e318206785a.
Nalcaci S, İlim O, Oztas Z, Akkin C, Acarer A, Afrashi F, et al. The prevalence and characteristics of Charles Bonnet syndrome in Turkish patients with retinal disease. Ophthalmol J Int Ophtalmol Int J Ophthalmol Z Augenheilkd. 2016;236(1):48–52. https://doi.org/10.1159/000446295.
Nesher R, Nesher G, Epstein E, Assia E. Charles Bonnet syndrome in glaucoma patients with low vision. J Glaucoma. 2001;10(5):396–400.
O’Hare F, Bentley SA, Wu Z, Guymer RH, Luu CD, Ayton LN. Charles Bonnet syndrome in advanced retinitis pigmentosa. Ophthalmology. 2015;122(9):1951–3. https://doi.org/10.1016/j.ophtha.2015.03.006.
Olbrich HM, Engelmeier MP, Pauleikhoff D, Waubke T. Visual hallucinations in ophthalmology. Graefes Arch Clin Exp Ophthalmol Albrecht Von Graefes Arch Klin Exp Ophthalmol. 1987;225(3):217–20.
Pliskin NH, Kiolbasa TA, Towle VL, Pankow L, Ernest JT, Noronha A, et al. Charles Bonnet syndrome: an early marker for dementia? J Am Geriatr Soc. 1996;44(9):1055–61.
• Russell G, Harper R, Allen H, Baldwin R, Burns A. Cognitive impairment and Charles Bonnet syndrome: a prospective study. Int J Geriatr Psychiatry. 2018;33(1):39–46. https://doi.org/10.1002/gps.4665 This is a small prospective cohort study of adults attending a low vision clinic. The authors found that 2 of 12 patients with Charles Bonnet syndrome developed dementia during the course of follow-up period, whereas none of 10 visually impaired adults without hallucinations developed dementia. The authors conclude that Charles Bonnet syndrome is not universally benign, and others have gone further to argue that it may be a forme fruste of Lewy body disease.
Santhouse AM, Howard RJ, Ffytche DH. Visual hallucinatory syndromes and the anatomy of the visual brain. Brain J Neurol. 2000;123(Pt 10):2055–64.
Shiraishi Y, Terao T, Ibi K, Nakamura J, Tawara A. The rarity of Charles bonnet syndrome. J Psychiatr Res. 2004;38(2):207–13.
Singh A, Sørensen TL. Charles bonnet syndrome improves when treatment is effective in age-related macular degeneration. Br J Ophthalmol. 2011;95(2):291–2. https://doi.org/10.1136/bjo.2010.179465.
Singh A, Sørensen TL. The prevalence and clinical characteristics of Charles Bonnet syndrome in Danish patients with neovascular age-related macular degeneration. Acta Ophthalmol. 2012;90(5):476–80. https://doi.org/10.1111/j.1755-3768.2010.02051.x.
Tan CSH, Lim VSY, Ho DYM, Yeo E, Ng BY, Au Eong KG. Charles Bonnet syndrome in Asian patients in a tertiary ophthalmic centre. Br J Ophthalmol. 2004;88(10):1325–9. https://doi.org/10.1136/bjo.2004.041947.
Teunisse RJ, Cruysberg JR, Hoefnagels WH, Verbeek AL, Zitman FG. Visual hallucinations in psychologically normal people: Charles Bonnet’s syndrome. Lancet Lond Engl. 1996;347(9004):794–7.
Teunisse RJ, Cruysberg JR, Verbeek A, Zitman FG. The Charles Bonnet syndrome: a large prospective study in The Netherlands. A study of the prevalence of the Charles Bonnet syndrome and associated factors in 500 patients attending the University Department of Ophthalmology at Nijmegen. Br J Psychiatry J Ment Sci. 1995;166(2):254–7.
Teunisse RJ, Zitman FG, Raes DC. Clinical evaluation of 14 patients with the Charles Bonnet syndrome (isolated visual hallucinations). Compr Psychiatry. 1994;35(1):70–5.
Vale TC, Fernandes LC, Caramelli P. Charles Bonnet syndrome: characteristics of its visual hallucinations and differential diagnosis. Arq Neuropsiquiatr. 2014;72(5):333–6.
Vukicevic M, Fitzmaurice K. Butterflies and black lacy patterns: the prevalence and characteristics of Charles Bonnet hallucinations in an Australian population. Clin Exp Ophthalmol. 2008;36(7):659–65. https://doi.org/10.1111/j.1442-9071.2008.01814.x.
Zhang H, Liu Z-L, Sun P, Gu F. Incidence of Charles Bonnet syndrome after intravitreal bevacizumab injection in neovascular age-related macular degeneration. Acta Ophthalmol. 2012;90(8):e647–8. https://doi.org/10.1111/j.1755-3768.2012.02421.x.
Hanyu H, Takasaki A, Sato T, Akai T, Iwamoto T. Is Charles Bonnet syndrome an early stage of dementia with Lewy bodies? J Am Geriatr Soc. 2008;56(9):1763–4. https://doi.org/10.1111/j.1532-5415.2008.01814.x.
Lapid MI, Burton MC, Chang MT, Rummans TA, Cha SS, Leavitt JA, et al. Clinical phenomenology and mortality in Charles bonnet syndrome. J Geriatr Psychiatry Neurol. 2013;26(1):3–9. https://doi.org/10.1177/0891988712473800.
Issa BA, Yussuf AD. Charles bonnet syndrome, management with simple behavioral technique. J Neurosci Rural Pract. 2013;4(1):63–5. https://doi.org/10.4103/0976-3147.105618.
Bhatia MS, Srivastava S, Jhanjee A. Charles Bonnet syndrome responding to amisulpride. J Neuropsychiatr Clin Neurosci. 2012;24(3):E40. https://doi.org/10.1176/appi.neuropsych.11080178.
Coletti Moja M, Milano E, Gasverde S, Gianelli M, Giordana MT. Olanzapine therapy in hallucinatory visions related to Bonnet syndrome. Neurol Sci Off J Ital Neurol Soc Ital Soc Clin Neurophysiol. 2005;26(3):168–70. https://doi.org/10.1007/s10072-005-0455-0.
Alamri SH. A low dose of risperidone resolved Charles Bonnet syndrome after an unsuccessful trial of quetiapine: a case report. Neuropsychiatr Dis Treat. 2018;14:809–11. https://doi.org/10.2147/NDT.S161124.
Boren RA, Boren CB. Charles Bonnet syndrome treated with pimavanserin. J Neuro-Ophthalmol Off J North Am Neuro-Ophthalmol Soc. 2019:1. https://doi.org/10.1097/WNO.0000000000000800.
Barnes JJ. The Charles Bonnet syndrome: symptomatic relief with atypical neuroleptics: a case series. Int J Psychiatry Clin Pract. 2001;5(2):141–4. https://doi.org/10.1080/136515001300374894.
Hanoglu L, Yildiz S, Polat B, Demirci S, Tavli AM, Yilmaz N, et al. Therapeutic effects of rivastigmine and alfa-lipoic acid combination in the Charles Bonnet syndrome: electroencephalography correlates. Curr Clin Pharmacol. 2016;11(4):270–3.
Takaya M, Matsusaka K, Yanagida M, Kimura R, Matsunaga H. The effects of memantine on a patient having preclinical dementia with Lewy bodies. Gen Hosp Psychiatry. 2013;35(3):327.e1–3. https://doi.org/10.1016/j.genhosppsych.2012.06.017.
Segers K. Charles Bonnet syndrome disappearing with carbamazepine and valproic acid but not with levetiracetam. Acta Neurol Belg. 2009;109(1):42–3.
Finucane TE. Neurontin for Charles Bonnet syndrome. J Am Geriatr Soc. 2006;54(9):1478. https://doi.org/10.1111/j.1532-5415.2006.00877.x.
Paulig M, Mentrup H. Charles Bonnet’s syndrome: complete remission of complex visual hallucinations treated by gabapentin. J Neurol Neurosurg Psychiatry. 2001;70(6):813–4. https://doi.org/10.1136/jnnp.70.6.813.
Grüter T, Ayzenberg I, Gold R, Börnke C. Charles Bonnet syndrome successfully treated with levetiracetam. J Neurol. 2016;263(9):1872–5. https://doi.org/10.1007/s00415-016-8240-y.
Sawant NS, Bokdawala RA. Pregabalin in the treatment of Charles Bonnet syndrome. JPMA J Pak Med Assoc. 2013;63(4):530–1.
Siddiqui Z, Ramaswmay S, Petty F. Mirtazapine for Charles Bonnet syndrome. Can J Psychiatr Rev Can Psychiatr. 2004;49(11):787–8.
Lang UE, Stogowski D, Schulze D, Domula M, Schmidt E, Gallinat J, et al. Charles bonnet syndrome: successful treatment of visual hallucinations due to vision loss with selective serotonin reuptake inhibitors. J Psychopharmacol Oxf Engl. 2007;21(5):553–5. https://doi.org/10.1177/0269881106075275.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of Interest
Ali G. Hamedani declares no potential conflicts of interest. Victoria S. Pelak reports royalties from Up-to-Date, Inc. and Elsevier.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
The original version of this article was revised: Shortly after publication, the authors became aware of several typographical errors in Table 1 which were introduced during the copy-editing process.
This article is part of the Topical Collection on Neurologic Ophthalmology and Otology
Rights and permissions
About this article
Cite this article
Hamedani, A.G., Pelak, V.S. The Charles Bonnet Syndrome: a Systematic Review of Diagnostic Criteria. Curr Treat Options Neurol 21, 41 (2019). https://doi.org/10.1007/s11940-019-0582-1
Published:
DOI: https://doi.org/10.1007/s11940-019-0582-1