Clinical Characteristics of the Charles Bonnet Syndrome in Patients with Neovascular Age-Related Macular Degeneration: The Importance of Early Detection.

PURPOSE: We investigated the characteristics, prognosis, and clinical outcome of the Charles Bonnet syndrome (CBS) in patients with neovascular age-related macular degeneration (AMD). METHODS: Five hundred psychiatrically healthy patients with neovascular AMD were screened for CBS. The individuals that fulfilled the inclusion criteria were systematically interviewed using a structured questionnaire that covered the impact, prognosis, risk factors, phenomenology, symptoms, and knowledge about the syndrome. A control group of 45 patients was used for comparison. Demographic data, current medication, and ocular risk factors were collected in all patients. RESULTS: Forty-five patients with CBS were identified. The majority of patients reported images that consisted of colored (62%) animals (44%) or faces (42%) that lasted for seconds (53%). Most patients reported a self-limited disease with a median duration of symptoms between 9 and 11.5 months, with only 7% knowing about CBS at symptom onset. The degree of visual deficit did not predict the characteristics, complexity, frequency, duration, or impact of visual hallucinations. One-third of patients reported negative outcome, which was associated with shorter duration of CBS (p = 0.023), fear-inducing images (p < 0.001), and impact on daily activities (p = 0.015). CONCLUSION: The prevalence of CBS in neovascular AMD patients is high and clinically relevant. Patients with recent onset of visual hallucinations and describing fear-inducing images are at greater risk for negative outcome. Periodic screening may minimize the negative consequences of this disease.

The sound of music: A rare case of auditory Charles Bonnet syndrome in an elderly male.

BACKGROUND: Auditory Charles Bonnet syndrome describes a rare condition that presents with sensorineural hearing loss, which can result in musical hallucinations. METHODS: A Hispanic man, age 78, with no previous psychiatric history was evaluated at our clinic with a complaint of hearing voices and music. The patient was noted to have received cochlear implantation in his right ear in 2013, due to bilateral sensorineural hearing loss. He had auditometric testing completed in 2013 following the onset of hallucinations. RESULTS: Routine laboratory workup was unremarkable. Computed tomography of the brain revealed mucosal thickening in the left maxillary sinus and mild generalized cerebral atrophy. Over the course of 4 months, treatment with donepezil led to improvement in symptomatology. The Brief Psychiatric Rating Scale score decreased substantially from 15 to 6 over an 8-week period. The Clinical Global Impression-Severity scale score decreased from 4 to 2 and the Clinical Global Impression-Improvement scale score increased from 0 to 1 over the same period. CONCLUSIONS: Auditory Charles Bonnet syndrome should be considered in patients endorsing auditory hallucinations with hearing loss in whom the etiology is not clearly due to a psychiatric condition. The role of acetylcholine requires further elucidation; however, donepezil demonstrated efficacy in the treatment of musical hallucinations in our patient.

Evaluation of the Clinical Features, Management, and Prognoses of Patients With Charles Bonnet Syndrome.

Charles Bonnet syndrome (CBS) is characterized by visual hallucinations with preservation of cognitive abilities. The hallucinations consist mostly of vivid (realistic) objects and tend to reoccur. Here, we evaluate the etiologies, symptoms, treatments, and prognoses of 13 CBS cases. All patients had visual hallucinations but were normal on cognitive and psychiatric assessments. Patient demographic and clinical characteristics, treatment options, and 3-month follow-up data were retrospectively reviewed. The possible causes of CBS and what the patients perceived during their hallucinations were recorded. Antipsychotic agents, such as risperidone and quetiapine, and anticonvulsants, such as levetiracetam, may be effective in some cases.

The elephant in the room: understanding the pathogenesis of Charles Bonnet syndrome.

PURPOSE: Charles Bonnet syndrome (CBS) is a syndrome characterised by complex visual hallucinations in individuals who are cognitively normal, though often elderly and visually impaired. Although first described over 250 years ago, the condition remains poorly understood and difficult to treat. RECENT FINDINGS: Our understanding of CBS pathogenesis has advanced little since it was first described, and much of the recent literature consists of case studies strikingly similar to the first published account of CBS. However, imaging studies have provided some indication as to the cortical areas implicated in the genesis of complex visual hallucinations, and the existence of similar hallucinatory syndromes in other sensory modalities suggests a common underlying mechanism. SUMMARY: This review begins by describing what is currently known about CBS, focusing on epidemiology, clinical presentation and diagnosis. It then explores potential starting points for better understanding the pathogenesis of CBS, namely the existence of similar conditions in other sensory modalities and the reproduction of complex visual hallucinations in sensory deprivation scenarios. Finally, it discusses how CBS should be approached in clinical practice.

Family physician awareness of Charles Bonnet syndrome.

Background: Charles Bonnet syndrome is characterized by formed visual hallucinations in individuals with vision loss. It is reported that one in five older adults with vision loss suffer from Charles Bonnet syndrome and the suspected lack of awareness amongst family physicians may lead to misdiagnosis and inappropriate treatment. Objective: To assess Canadian family physicians' awareness of Charles Bonnet syndrome. Methods: We conducted a national perception and practices survey of family physicians across Canada to assess (i) the level of awareness of Charles Bonnet syndrome amongst family physicians; (ii) the frequency of family physicians' encounters with patients with visual hallucinations and (iii) management strategies and referral patterns for patients with Charles Bonnet syndrome presenting to family physicians. Results: Four hundred and ninety-nine family physicians across Canada answered at least one question on the survey. 54.7% indicated they were not at all aware and 19.7% indicated they were slightly aware of Charles Bonnet syndrome. 72.8% of physicians had patients present with visual hallucinations once a year or less often. The frequency of patients seen in practice with visual hallucinations is significantly associated with awareness by physicians of Charles Bonnet syndrome (ß = 0.501, t(388) = 5.59, P < 0.001). Of those who encountered patients with visual hallucinations, the majority of respondents (77.4%) provided counselling or education to the patient. Conclusion: There is a great lack of knowledge about Charles Bonnet syndrome among family physicians. Awareness of Charles Bonnet syndrome is critical to appropriate diagnosis, assessment and treatment of this condition and to reassure patients that they are not suffering from a mental disorder.

Reversible Charles Bonnet Syndrome After Oculoplastic Procedures.

Individuals with Charles Bonnet syndrome (CBS) typically have severe visual loss and experience visual hallucinations yet have no psychiatric disease. Visual impairment often is due to end-stage glaucoma or macular degeneration. We report 3 cases of CBS in patients who underwent an oculoplastic surgical procedure. One patient experienced binocular visual distortion due to excessive topical ophthalmic ointment, and 2 patients experienced monocular visual impairment from patching. Visual hallucinations resolved once vision returned to baseline. We highlight the possibility of transient CBS in postoperative patients who have temporary iatrogenic vision impairment in one or both eyes.

Hallucinatory experiences in visually impaired individuals: Charles Bonnet syndrome - implications for research and clinical practice.

BACKGROUND: Charles Bonnet syndrome (CBS) refers to visual hallucinations that occur in individuals with preserved cognitive functions associated with visual impairment. METHODS: This article reviews occurence of visual hallucinations in subjects with CBS by journals published in English in the Pubmed database in the period 1992-2018. Criteria for selection of appropriate papers were sufficient information and perspicuous view on pathogenesis, epidemiology, clinical presentation and treatment possibilities of CBS. RESULTS: Most commonly, visual hallucinations in patients with CBS are complex, repetitive and stereotyped. Such individuals have preserved insight that those percepts are not real, and there is an absence of secondary explanatory delusions and hallucinations within other modalities. Seeing as the aforementioned percepts do not share all the characteristics of hallucinations, it remains unresolved how they should be referred to. Terms as release hallucinations, one that is reflecting its underlying pathogenesis, or confabulatory hallucinatory experiences have been proposed. Moreover, CBS has also been referred to as phantom vision syndrome and may occur in any ophthalmological disease. It is not particularly connected with loss of function along any level of the visual pathway. Although this syndrome is mostly associated with age-related macular degeneration, glaucoma and cataract, it could be related to almost any other ophthalmological conditions. The incidence of CBS alongside with mostly other ocular pathology is rising as population is ageing. CONCLUSIONS: Nonetheless, CBS remains commonly underreported, under recognized and/or misrecognized. Albeit the treatment recommendations and guidelines are not yet fully established, it is important to raise awareness of this specific and distinct condition, which inevitably implicates many differential diagnostic deliberations.

Charles Bonnet Syndrome Associated With Recurrent Hypertensive Crisis.

Charles Bonnet syndrome (CBS) is a disorder of visual hallucinations in psychologically normal patients with ocular disease or damage to visual pathways. The etiology of CBS is not fully understood. It is associated with various triggers, with age-related macular degeneration the most common; other triggers are systemic diseases such as stroke, multiple sclerosis, and anemia as well as lighting issues, fatigue, and medical or surgical eye treatments. Visual disturbances such as decreased visual acuity, visual field deficits, or visual hallucinations are common in association with hypertensive encephalopathy. We describe a patient with episodic CBS triggered by recurrent hypertensive crises, which resolved with blood pressure management in the hospital setting.

Charles Bonnet Syndrome in Patients with Open-Angle Glaucoma: Prevalence and Correlation to Visual Field Loss.

PURPOSE: To determine the prevalence and characteristics of Charles Bonnet Syndrome (CBS) and its relation to visual field loss (VFL) in patients with open-angle glaucoma (OAG). DESIGN: Prospective, cross-sectional study. PARTICIPANTS: Adult patients (n = 337) with manifest OAG with verified VFL and without significant macular disease or extraocular conditions known to cause visual hallucinations. METHODS: Patients attending the glaucoma outpatient department of the Skåne University hospital, Malmö, Sweden, between April 1, 2018, and December 31, 2018, were consecutively evaluated for inclusion. Potentially eligible patients admitting to having complex visual hallucinations were interviewed to explore the characteristics of their hallucinatory experiences. Recent automated visual field examinations were available for all participants, and swept-source OCT was performed in participants with CBS to rule out previously undiagnosed macular pathology. The correlation between potential risk factors and CBS was evaluated with logistic regression analysis. MAIN OUTCOME MEASURE: Prevalence of CBS in patients with OAG. RESULTS: Charles Bonnet Syndrome was found in 7.1% (95% confidence interval [CI], 4.7-10.6) of patients with OAG. Participants with CBS were more likely to have at least 1 eye with a visual field index (VFI) of ≤30% compared with those without CBS (71% vs. 34.2%; P = 0.001). Although the best-corrected visual acuity (BCVA) in the worse eye was significantly lower in participants with CBS (decimal equivalent of Snellen BCVA: 0.25 vs. 0.6, P = 0.003), 33% of these participants had a BCVA of ≥0.5 in the worse eye. In multivariable analysis, CBS was correlated to the VFI of the better eye (odds ratio, 0.984; 95% CI, 0.969-0.998, P = 0.030) and the BCVA of the worse-seeing eye (odds ratio, 0.210; 95% CI, 0.046-0.952, P = 0.043). CONCLUSIONS: Charles Bonnet Syndrome was not a rare condition in patients with glaucoma. Patients with a combination of advanced VFL and low BCVA had the highest risk of CBS; however, 1 of 3 patients with CBS had a BCVA of ≥0.5 in both eyes. These findings emphasize the importance of being attentive to symptoms of CBS in patients with glaucomatous VFL even when visual acuity is preserved.

High-Density EEG in a Charles Bonnet Syndrome Patient during and without Visual Hallucinations: A Case-Report Study.

Charles Bonnet syndrome (CBS) is a rare clinical condition characterized by complex visual hallucinations in people with loss of vision. So far, the neurobiological mechanisms underlying the hallucinations remain elusive. This case-report study aims at investigating electrical activity changes in a CBS patient during visual hallucinations, as compared to a resting-state period (without hallucinations). Prior to the EEG, the patient underwent neuropsychological, ophthalmologic, and neurological examinations. Spectral and connectivity, graph analyses and signal diversity were applied to high-density EEG data. Visual hallucinations (as compared to resting-state) were characterized by a significant reduction of power in the frontal areas, paralleled by an increase in the midline posterior regions in delta and theta bands and by an increase of alpha power in the occipital and midline posterior regions. We next observed a reduction of theta connectivity in the frontal and right posterior areas, which at a network level was complemented by a disruption of small-worldness (lower local and global efficiency) and by an increase of network modularity. Finally, we found a higher signal complexity especially when considering the frontal areas in the alpha band. The emergence of hallucinations may stem from these changes in the visual cortex and in core cortical regions encompassing both the default mode and the fronto-parietal attentional networks.

Visual hallucinations and sight loss in children and young adults: a retrospective case series of Charles Bonnet syndrome.

BACKGROUND/AIMS: Charles Bonnet syndrome (CBS) is a complication of sight loss affecting all ages; yet, few childhood cases have been reported. Our aim is to raise awareness of this under-reported association occurring in children and young adults in order to prevent psychological harm in this age group. METHODS: A retrospective case series reviewing medical notes of patients <25 years of age with sight loss and reported CBS at a single centre hospital eye service in London, United Kingdom. Search of electronic patient records identified 13 patients experiencing hallucinatory events over a 9-year period. Outcomes were patient demographics including ocular diagnosis, visual acuity at time of onset, characteristics of hallucinations, clinical management strategies and patient-reported affliction. RESULTS: Eight patients were diagnosed with progressive inherited retinal diseases, primarily Stargardt disease (N=5). Clinical history indicated patients had significantly reduced best-corrected visual acuity (BCVA) in at least one eye at onset; median (IQR) worse eye BCVA was 1.0 (0.86-1.6) logarithm of minimum angle of resolution(LogMAR). CBS significantly affected patients' personal lives including education, diet and sleep. Clinical management was varied, mostly relating to reassurance at the point of contact. CONCLUSION: We describe the clinical features of young patients with CBS, with management strategies and aspects of negative outcomes. High potential caseload and risk of psychological harm merit further research. Increased awareness among healthcare professionals and patient education to forewarn susceptible individuals may reduce the overall impact and improve coping with symptoms.