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.

Three cases of Charles Bonnet Syndrome in patients with advanced glaucomatous visual field loss but preserved visual acuity.

PURPOSE: To describe three cases of Charles Bonnet syndrome (CBS) in glaucoma patients with preserved visual acuity. METHODS: Three glaucoma patients who had taken part in a recent CBS study were interviewed about their hallucinations. The patients underwent macular optical coherence tomography (OCT) of both eyes. The visual function was evaluated with visual field measurement (Humphrey visual field analyser) and visual acuity testing (Snellen scale). RESULTS: All three patients had preserved visual acuity (≥0.5 in both eyes) and at least one eye with advanced visual field defect (Mean Deviation worse than -12.00 decibel). They all reported vivid visual hallucinations with insight into the unreal nature of the hallucinations. CONCLUSION: Charles Bonnet syndrome can occur in glaucoma despite preserved visual acuity. Awareness of this relation is desirable among clinicians, as it will improve communication with patients.

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.

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.

[Giant Cell Arteritis and Charles Bonnet Syndrome. The Case of Saint Martin of Leon] / Arteritis de Células Gigantes y Síndrome de Charles Bonnet/Giant Cell Arteritis and Charles Bonnet Syndrome. El caso de Santo Martino de León

Saint Martin of Leon was a monk who lived in the 12th century. The details of his life are known because they were described by his contemporary, Lucas de Tuy, in the Liber de Miraculis Sancti Isidori. Saint Martin of Leon was a venerable old man who suffered from severe headaches, considerable difficulties in raising or maintaining his arms raised, asthenia, and anorexia. In addition, he is represented in his codex Concordia with an unusual hyperpigmentation of the temples suggesting a therapeutic intervention on temporal arteries. These data lead us to think that this could be the first described case of giant cell arteritis with some clinical information. Moreover, he experienced a singular and curious appearance of Saint Isidore of Seville, who made him swallow a booklet, which might correspond to a complex visual hallucination or associated Charles Bonnet syndrome. Historical data on the disease are reviewed and discussed, as well as its evolution and treatment before giving steroids.

Santo Martino de León fue un clérigo leonés del siglo XII. Se conocen algunos detalles de su vida porque fueron descritos por su coetáneo Lucas de Tuy en el Liber de Miraculis Sancti Isidori. Santo Martino era un anciano venerable que padecía fuertes dolores de cabeza, notables dificultades para elevar o mantener elevados los brazos, cansancio y anorexia. Además, aparece representado en su obra Concordia con una inusual hiperpigmentación de las sienes que sugiere alguna intervención terapéutica sobre las arterias temporales. Estos datos nos llevan a pensar que podría ser el primer caso descrito de arteritis de células gigantes con alguna información clínica. Por otra parte, sufrió una singular y curiosa aparición de San Isidoro de Sevilla que le hizo tragar un librillo, que pudiera corresponder a una alucinación visual compleja o síndrome de Charles Bonnet asociado. Se comentan los datos históricos de la enfermedad, así como su evolución y tratamiento antes de los corticoides.

Screening for Charles Bonnet syndrome: Should the definition be reconsidered?

Purpose: Charles Bonnet syndrome (CBS) is a condition in which individuals with visual impairment (VI) and with no cognitive deficits experience visual hallucinations, typically with no other sensory hallucinations. Although few isolated case reports of CBS from India have been published, the prevalence for CBS in India is largely unknown. The primary aim of this study was to estimate CBS prevalence in patients with vision impairment visiting a tertiary eye care center. Methods: The study was conducted in two phases. In phase 1, patients with VI, age ≥40 years with presenting visual acuity worse than 20/63 were enrolled. In phase 2, patients with presenting visual acuity worse than 20/63 and/or with binocular visual field loss, age ≥18 years were recruited. A CBS survey was administered only to those who passed a screening test for cognition impairment. Results: A total of 218 patients were screened (phase 1 = 113 and phase 2 = 105). Two-hundred ten patients (mean age ± standard deviation = 49.2 ± 17.3 years, males = 139) were found eligible to complete the CBS survey. Fourteen patients were found to have visual hallucinations. In addition, three other patients had visual hallucinations with associated auditory input to the visual imagery. All patients had complete insight about their hallucinations. Conclusion: Depending on the inclusion criteria, we found the prevalence for CBS in patients with VI to vary between 6.7% to 8.1% (if including patients with auditory input). More investigation is needed to assess the associated role of other sensory inputs (e.g. auditory) with the visual imagery experienced in CBS.