Research progress of Charles Bonnet syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

Charles Bonnet syndrome(CBS)refers to hallucinations resulting from visual pathway damage in individuals without neuropsychiatric disorders. CBS patients typically have insight into the unreality of their hallucinations, and the disorder may be episodic, periodic, or chronic. The condition's prevalence varies from 0.4% to 30% due to the absence of agreed diagnostic criteria for CBS and some patients' reluctance to acknowledge hallucinations. Massive studies have demonstrated an association between CBS and ocular diseases, such as age-related macular degeneration and glaucoma. Additionally, there have been reports of CBS occurring after ophthalmic surgery or medication. Therefore, clinicians should be vigilant in diagnosing CBS promptly and reassuring patients. There is a lack of literature on CBS in ophthalmology, and further research is needed to explore its pathogenesis and diagnostic and therapeutic options. This article reviews some of the studies related to CBS in recent years.

Charles Bonnet syndrome in age-related macular degeneration - prevalence and clinical features in a Portuguese population / Síndrome de Charles Bonnet na degeneração macular relacionada à idade - prevalência e características clínicas numa população portuguesa

ABSTRACT Objective: Age-related macular degeneration (AMD) is the most prevalent cause of irreversible visual loss in the developed world. In late stages, it may lead to extremely low visual acuities, especially when associated with geographic atrophy or choroidal neovascularization. According to recent literature, Charles Bonnet syndrome (CBS) may be a rather common feature of late AMD. Methods: One hundred patients with late-stage age-related macular degeneration were actively asked whether they had symptoms of Charles Bonnet syndrome. Those that answered positively underwent a comprehensive questionnaire about the details of the visual hallucinations. Results: The following factors were significantly associated with Charles Bonnet syndrome: older age (+6.3 years; p=0.003), lower visual acuity in the better eye (Charles Bonnet Syndrome Group: 0.11; Non-Charles Bonnet Syndrome Group: 0.42; p=0.005) and female sex (Charles Bonnet Syndrome Group: 88%; Non-Charles Bonnet Syndrome Group: 43%; p=0.02). The visual hallucinations occurred mainly straight ahead (n=5), once per day (n=4), at no particular time (n=6), lasted some minutes (n=5), and disappeared after blinking (n=3) or looking away (n=3). The majority of patients lived alone (n=7), had not told anyone about the hallucinations (n=6), and associated the episodes with severe distress (n=5). Conclusion: Charles Bonnet syndrome was fairly prevalent in this late-stage age-related macular degeneration population. Our sample shows the importance of directly asking subjects about Charles Bonnet syndrome since they are often reluctant to admit to having visual hallucinations. Reassurance about its benignity is crucial to improve their quality of life.

RESUMO Objetivo: A doença macular ligada à idade (DMI) é a causa mais prevalente de perda visual irreversível nos países desenvolvidos. Em estadios avançados, esta doença pode levar a acuidades visuais extremamente baixas. De acordo com literatura recente, a Síndrome de Charles Bonnet (SCB) pode acontecer de forma relativamente comum na DMI tardia. Métodos: Cem doentes com degeneração macular da idade avançada foram interrogados ativamente sobre terem sintomas da síndrome de Charles Bonnet. Os que responderam de forma positiva foram submetidos a um questionário oral detalhado sobre os pormenores das alucinações visuais. Resultados: Os seguintes fatores foram significativamente associados à síndrome de Charles Bonnet: idade avançada (+6,3 anos; p=0,003), menor melhor acuidade visual corrigida no melhor olho (Grupo com Síndrome de Charles Bonnet: 0,11; Grupo sem Síndrome de Charles Bonnet: 0,42; p=0,005) e sexo feminino (Grupo com Síndrome de Charles Bonnet: 88%; Grupo sem Síndrome de Charles Bonnet: 43%; p=0,02). As alucinações visuais ocorriam principalmente em frente (n=5), uma vez por dia (n=4), em qualquer altura do dia (n=6), duravam alguns minutos (n=5) e desapareciam após pestanejo (n=3) ou desvio do olhar (n=3). A maioria dos doentes vivia sozinha (n=7), não tinha partilhado sua condição com ninguém (n=6) e associava os episódios a uma sensação angustiante (n=5). Conclusão: A síndrome de Charles Bonnet teve prevalência relativamente alta nessa população de degeneração macular da idade. Nossa amostra sublinha a importância de questionar diretamente sobre síndrome de Charles Bonnet, uma vez que os doentes se sentem muitas vezes relutantes em admitir alucinações visuais. A reafirmação da benignidade da situação é crucial para aumentar a qualidade de vida desses indivíduos.

Charles Bonnet Syndrome Following Trans-Sphenoidal Adenomectomy without Optic Nerve Atrophy

Charles Bonnet syndrome (CBS) can develop after trans-sphenoidal adenomectomy (TSA); however, the neural mechanisms remain unknown. Sensory deprivation and releasing phenomenon are both hypothetical explanations for this condition; however, there is no definite evidence that strongly supports either supposition. We report the first case of CBS after TSA without optic nerve atrophy. Postoperatively, the patient's vision seemed to be relatively well preserved, apart from the left-side hemianopsia in the right eye. Distinctive visual hallucinations only appeared when his eyes were closed, and these responded to quetiapine in a dose-dependent manner. Dose dependent change in colors and formation of hallucination was reported. Two weeks after quetiapine initiation, the patient's CBS was completely resolved. This unique case suggests that blocking sensory input from the periphery is more critical than neural damage of the bottom-up connection to the visual association cortex. In addition, quetiapine should be considered as a specific treatment for CBS.

Alucinaciones visuales en pacientes con privación sensorial: ¿síndrome de Charles Bonnet? / Visual hallucinations in sensory deprived patients: Charles Bonnet syndrome?

Introducción: Existen pacientes que, después de lesiones cerebrales o periféricas, pierden alguna función sensorial, como la vista o el oído. Paradójicamente, después de perder esta función, presentan alucinaciones complejas relacionadas con la función perdida. Se sabe que este fenómeno puede presentarse ante lesiones en cualquier nivel de la vía visual, especialmente en el nivel de la retina.Objetivo: Revisar la bibliografía existente acerca del síndrome de Charles Bonnet para conocer los últimos avances con respecto a este fenómeno.Método: Se revisaron las bases de datos de PubMed y PsychInfo con las siguientes palabras clave: síndrome de Charles Bonnet; alucinaciones visuales; alucinosis peduncular; Charles Bonnet; privación sensorial. Se incluyeron aquellos artículos que efectivamente trataran del tema. Asimismo, se revisaron los textos clásicos referentes a este síndrome y los artículos mencionados en la bibliografía encontrada.Resultados: En el presente artículo se describe la historia del síndrome, el fenómeno clínico, los factores de riesgo, los criterios diagnósticos, los tratamientos empleados, otros fenómenos similares y las teorías propuestas para explicarlo.Discusión y conclusión: A la fecha continúan siendo controvertidos los criterios diagnósticos del síndrome de Charles Bonnet, en especial en lo referente a la preservación absoluta del insight como condición sine qua non. Descrito desde el siglo XVIII, el síndrome de Charles Bonnet corresponde al prototipo de alucinaciones visuales en pacientes con privación visual, si bien, de acuerdo con la presente revisión, la fenomenología de éste es bastante variada, siendo cuestionable si resulta también el prototipo de las alucinaciones con insight preservado.

Introduction: Some patients, after brain or peripheral injuries, lose a sensory function, such as sight or hearing, but paradoxically experience complex hallucinations related to the function they have lost. It is known that this phenomenon may appear with injuries at any level in the visual pathway, especially in the retina.Objective:To review the existent bibliography on the Charles Bonnet syndrome to establish the state of the art with regards to this phenomenon.Method: The databases PubMed and PsychInfo were searched for articles containing the following keywords: Charles Bonnet syndrome; visual hallucinations; peduncular hallucinosis; Charles Bonnet; sensory deprivation. We included those related to the subject. We also included the classic texts referring to this phenomenon and the articles mentioned in the literature.Results: In the present study, we describe the history of Charles Bonnet syndrome, clinical presentation, risk factors, diagnostic criteria, treatment employed, similar conditions and the theories seeking to explain it.Discussion and conclusion: To date, the diagnostic criteria for Charles Bonnet syndrome remain controversial, especially those concerning the absolute preservation of insight as a sine qua non factor to establish the diagnosis. Conclusion: Described since the 18th century, the Charles Bonnet syndrome corresponds to the prototype of visual hallucinations in patients with visual deprivation, although, according to the present review, its phenomenology is vast, remaining unclear if it corresponds to the prototype of hallucinations with preserved insight.

Charles Bonnet syndrome: characteristics of its visual hallucinations and differential diagnosis / Síndrome de Charles Bonnet: características das alucinações visuais e diagnóstico diferencial

Objective: To present an eight-case serie of patients with Charles Bonnet syndrome (CBS). Method: All patients were initially evaluated by an ophthalmologist and then submitted to a neurologic evaluation with exclusion of alternative psychiatric and neurologic diagnoses. Results: Five patients were male (62.5%) and the mean age was 52.3+16.0 years. Two patients suffered from severe myopia and glaucoma, three had retinitis pigmentosa, one had anterior ischemic optic neuropathy, one had age-related macular degeneration and one had toxoplasmic retinochoroiditis. Mean visual acuity in the right eye was 1,12 logMAR and in the left eye 0.57 logMAR. A mean delay of 41.7 months occurred until diagnosis. All hallucinations were complexes and mostly ocurred on a weekly-basis (62.5%) and lasted for seconds (87.5%). Conclusions: Physicians who care for low vision patients should be aware of CBS and appropriately diagnose its hallucinations after exclusion of psychiatric and neurologic diseases. .

Objetivo: Apresentar uma série de oito casos da síndrome de Charles Bonnet (SCB). Método: Todos os pacientes foram avaliados por um oftalmologista e por um neurologista, tendo sido excluídos diagnósticos alternativos neurológicos e psiquiátricos. Resultados: Cinco pacientes eram homens (62,5%) e a média de idade foi de 52,3±16,0 anos. Do total de pacientes: dois tinham miopia grave e glaucoma, três retinose pigmentar, um neuropatia óptica isquêmica anterior, um degeneração macular relacionada à idade e um retinocoroidite toxoplásmica. A acuidade visual média no olho direito foi de 1,12 logMAR e no olho esquerdo foi de 0.57 logMAR. Houve um atraso de 41,7 meses, em média, para o diagnóstico. Todas as alucinações eram complexas, ocorriam, em sua maioria, semanalmente (62,5%) e duravam segundos (87,5%). Conclusão: Médicos que lidam com pacientes com baixa visão devem saber distinguir as características peculiares da SCB, após exclusão de doenças neurológicas e psiquiátricas. .

Síndrome de Charles Bonnet en cuatro casos con atrofia óptica / Charles Bonnet syndrome in four cases with optical atrophy

El síndrome de Charles Bonnet constituye un trastorno muy poco reportado. En Cuba, que se conozca, no hay referencia de casos publicados hasta este momento. Se caracteriza por la presencia de alucinaciones visuales complejas en individuos con pérdida visual, mentalmente sanos y con conciencia crítica de la naturaleza irreal de sus alucinaciones. Se presentan cuatro casos con este síndrome relacionados con déficit visual secundario a atrofia óptica...

Charles Bonnet syndrome is an underreported disorder. As far as we know, there are no publicized references in Cuba up to the present time. This syndrome involves complex visual hallucinations in visually impaired individuals, who are mentally healthy and have intact cognition of the unreal nature of their hallucinations...

Charles Bonnet Syndrome Following Bilateral Occipital Infarction

No abstract available.

A síndrome de Charles Bonnet e o problema dos transtornos de percepção visual nos idosos / The Charles Bonnet syndrome and the problem of visual perceptual disorders in the elderly / Le syndrome de Charles Bonnet et le problème des troubles de perception visuelle chez les personnes âgées / El síndrome de Charles Bonnet y el problema de los trastornos de percepción visual en personas viejas

A literatura, clássica ou recente, sobre os transtornos de percepção nos idosos é revisada. A utilidade do conceito de Síndrome de Charles Bonnet, um epônimo originalmente proposto para descrever as alucinações visuais nos idosos na ausência de prejuízos cognitivos e de oftalmopatia periférica é contestada, principalmente devido à síndrome ter sido progressivamente ampliada. Descrevemos três casos representativos de pacientes idosos que desenvolveram diferentes tipos de transtornos de percepção visual. Propomos a condução de estudos sistemáticos da frequência de tais transtornos nos idosos e de quais outras patologias, particularmente cognitivas e visuais que possam estar associadas a eles.

A review is presented here of the classical and recent literature on perception disorders in the elderly. The author contests the usefulness of the concept of the Charles Bonnet syndrome, a category first proposed to describe visual hallucinations in elderly persons in the absence of cognitive damage or peripheral ophthalmopathy. This objection is brought up mainly because the concept has been progressively broadened. We describe here three representative cases of elderly patients who developed different types of disorders in visual perception. Systematic studies are now needed on the frequency of such disorders in the elderly and on the question as to what other pathologies, especially of the cognitive and visual type, may be associated with them.

Cet article présente une révision de la littérature classique et récente sur les troubles de la perception des personnes âgées. L'utilité du concept du syndrome de Charles Bonnet, un éponyme proposé à l'origine pour décrire des hallucinations visuelles chez les personnes âgées sans troubles cognitifs, ni d'ophtalmopathie périphérique, est contestée, surtout à partir du fait que ce syndrome a été progressivement élargi. Nous décrivons trois cas représentatifs de patients âgés qui ont développé de différents types de troubles de perception visuelle. Nous suggérons des études systématiques sur la fréquence de ces troubles chez les personnes âgées et sur les autres maladies, en particulier cognitives et visuelles, qui peuvent y être associées.

Se revisa la literatura clásica o reciente sobre los trastornos de percepción en viejos. Se cuestiona la utilidad del concepto de Síndrome de Charles Bonnet, un epónimo, originalmente propuesto para describir las alucinaciones visuales en viejos en ausencia de prejuicios cognitivos y de oftalmopatologia periférica, principalmente por causa de una ampliación progresiva de ese síndrome. Se describen tres casos representativos de pacientes de edad que desarrollaron diferentes tipos de trastornos de la percepción visual. Proponemos la conducción de estudios sistemáticos de la frecuencia de esos trastornos en viejos y de cualquier otras patologias, particularmente cognitivas y visuales que puedan estar asociadas a ese síndrome.

Eye-related visual hallucinations: Consider ‘Charles Bonnet syndrome’.

The Charles Bonnet Syndrome (CBS) is typically characterized by visual hallucinations in elderly people without cognitive defects. This article presents the case of an 80-year-old male patient with a one-year history of visual hallucinations, secondary to glaucoma, in both eyes. Neither a dopamine agonist nor cholinesterase inhibitor therapy improved his symptoms. In this case, the hallucinations were gradually improved after administration of a GABAergic drug, pregabalin, for diabetic polyneuropathy. Placebo-controlled clinical trials would be needed to support this effect of pregabalin, as suggested by this association.

Síndrome de Charles Bonnet / Charles Bonnet syndrome

En la medida que crece la población y aumenta el número de personas de la tercera edad con trastornos de la visión, se necesita cada vez más hacer conciencia del Síndrome de Charles Bonnet entre los oftalmólogos, psiquiatras y neurólogos. El propósito de la presente revisión es ofrecer una visión actualizada sobre sus aspectos clínicos, fisiopatológicos, epidemiológicos junto con las estrategias de manejo más razonables. Para ello se emprendió una búsqueda exhaustiva de la literatura más relevante, con el fin de comprender estos aspectos. El síndrome de Charles Bonnet es una entidad subreportada y subdiagnosticada que aparece en personas de la tercera edad, visualmente comprometidas. Estos pacientes por lo general tienen un estado cognoscitivo intacto aunque algunos autores sostienen que puede ser más frecuente en casos de deterioro cognoscitivo leve. Su fisiopatología aún es tema de debate pero las teorías mas aceptadas, hasta el momento, son la teoría de la denervación y el fenómeno de liberación. Los reportes de casos aislados señalan la posibilidad de intervenciones farmacológicas. Sin embargo, asegurar al paciente y tratar la patología visual son las piezas principales del tratamiento, no obstante, se necesitan más investigaciones que permitan aumentar las opciones terapéuticas eficaces.

As population grows, the number of older people with impaired vision does too.That is why ophthalmologists, psychiatrists and neurologists urgently require familiarization with the most important characteristics of Charles Bonnet syndrome. The purpose of this review is to give an update on its clinical aspects, pathophysiology, epidemiology and more reasonable management strategies of this syndrome. Accordingly, we carried out an exhaustive search of the relevant literature, in order to understand these issues. Charles Bonnet syndrome is a sub reported and sub diagnosed entity in visually impaired older people. These patients usually have an intact mental status although some authors claim that may be more common in cases of mild cognitive impairment. Its pathophysiology is still debated but most accepted theories are “the deafferentation theory” and “the phenomenon of liberation”. Isolated case reports point to the possibility of pharmacological interventions, however reassuring the patient and treating visual diseases are the main parts of treatment, more research is needed to allow the possibility of effective treatment options.

Alucinaciones visuales: síndrome de Charles Bonnet / Visual hallucinations: Charles Bonnet Syndrome

El síndrome de Charles Bonnet se caracteriza por alucinaciones visuales que se presentan en personas con una pérdida de visión severa y, generalmente, enpersonas de edad avanzada, con un estado mental normal. En este artículo se analizan las características de este síndrome, como la etiología, la fisiopatología y el diagnóstico diferencial, y se dan conocer criterios de diagnóstico para tener en cuenta en la práctica diaria, en especial para los optómetras, quedeben estar familiarizados con los signos y síntomas de este síndrome, ya que este grupo de pacientes casi siempre acude primero a la consulta optométrica. Aunque la condición se hizo pública en la comunidad médica hace más de doscientos años atrás, es muy poco diagnosticada por el desconocimiento existente sobre ella. En la práctica optométrica, se debe sospechar del síndrome de Charles Bonnet, especialmente en los grupos de pacientes que presentan alucinaciones visuales y debilidad visual o ceguera, evitando un diagnóstico psiquiátrico equivocado.

The Charles Bonnet Syndrome is characterized by visual hallucinations that occur in people with severe sight loss and usually in elderly people with a normal mental state. This article discusses the characteristics of this syndrome as the etiology, pathophysiology and differential diagnosis, and is known diagnostic criteria for consideration in daily practice. Especially for optometrists be who sould be familiar with the signs and symptoms of this syndrome, as this group of patients almost always come first to consult optometrists. Even though the condition was published in the medical community for over 200 years ago, it has been rarely diagnosed due to the lack of knowledge about it. In optometric practice, it is suspected Charles Bonnet Syndrome especially in this age group with visual hallucinations and visual weakness or blindness, avoiding a psychiatric diagnosis wrong.

Síndrome de Charles Bonnet: a propósito de um caso / Charles Bonnet syndrome: regarding a case

Idosos com déficit visual por vezes apresentam alucinações visuais relacionadas à síndrome de Charles Bonnet. O surgimento destas alucinoses frequentemente causa ansiedade ao paciente e gera dificuldade diagnóstica e de conduta. Neste artigo descrevemos o caso de uma senhora de 82 anos de idade que se queixou de alucinações visuais complexas e revimos a literatura a respeito da síndrome de Charles Bonnet; condição que promove alucinação visual em pessoas mentalmente saudáveis.

Visually handicapped elderly people sometimes experience visual hallucinations associated with Charles Bonnet syndrome. The appearance of these hallucinosis often causes anxiety to the sufferer and can be difficult for the diagnosis and management. In this article, we report an 82-year-old woman that suddenly complained of complex visual hallucinations, and review the literature on the Charles Bonnet syndrome, a condition involving visual hallucinations in a mentally normal person.

Quetiapine Therapy for Visual Hallucinations Caused by Charles Bonnet Syndrome / 대한정신약물학회지

The case of a 77-year-old man with Charles Bonnet syndrome was presented. This patient lost his vision due to glaucoma, and he subsequently developed complex visual hallucinations. No other psychotic symptoms (e.g., delusions, perceptual disturbances) and no evidence of cognitive impairment or neurological diseases were reported. The visual hallucinations disappeared after treatment with quetiapine, an atypical antipsychotic, without any side effects. The visual hallucinations reappeared after quetiapine was discontinued. Treatment with a small dose of quetiapine has been maintained to prevent the exacerbation of symptoms.

Síndrome de Charles Bonnet: Manejo con haloperidol en paciente nonagenaria. Caso clínico / Charle Bonnet syndrome: Report of one case managed with haloperidol

Charles Bonnet syndrome is an underrecognized condition characterized by complex visual hallucinations, ocular problems causing visual deterioration and preserved cognitive status. Its prevalence is 5/1000 in ambulatory ophthalmologic patients. Generally occurs in elderly people in whom it may be confused with delirium or dementia. The first management step is to improve vision, if possible. Hallucinations may be managed pharmacologically. We report a 94 year-old woman with the characteristic clinical picture of the syndrome that improved with haloperidol.

A Case of Charles Bonnet Syndrome After Enucleation

PURPOSE: To report a case of Charles Bonnet syndrome in a patient who developed phantom vision of an enucleated eye. The patient had lost her vision because of a chemical burn 10 years previously. CASE SUMMARY: A 37-year-old woman received enucleation of one eye because ocular pain and phthisis had developed due to a chemical burn that occurred 10 years previously. On the first postoperative day, she complained of phantom vision in the enucleated eye. The image she described was like sparkling bubbles. She had recognized the image was not real, and no psychological problems were found during pre- and postoperative periods. Her wound state was good, so she is currently wearing an artificial eye, though her phantom vision intermittently returns. She has adapted to her phantom vision such that she now rarely feels discomfort. RESULTS: Charles Bonnet syndrome is a visual hallucination that occurs among the blind or those who are visually impaired. Besides ophthalmologic care, sociological and psychological care should be considered.

A Case of Charles Bonnet Syndrome After Resection of a Meningioma

PURPOSE: To report a case of Charles Bonnet syndrome that developed after resection of a meningioma. CASE SUMMARY: The authors reviewed the medical record, brain magnetic resonance image, and Goldmann visual field test of a 56-year-old male patient who was diagnosed with a meningioma in the right parietal and occipital lobe and underwent resection of the tumor. The preoperative Goldmann visual field test showed homonymous left inferior quadrantanopsia. Subtotal resection of the mass in the right parietal and occipital lobe was performed, and postoperative histopathologic examination confirmed the diagnosis of a meningioma. Postoperatively, the patient complained of visual hallucination in an area of the eye with visual field defects. However, his consciousness and orientations were intact, and other cognitive functions were also normal. CONCLUSIONS: Visual hallucination can manifest in an area with visual field defects after resection of abrain tumor. In such a case, a diagnosis of Charles Bonnet syndrome should be considered.

Síndrome de Charles Bonnet: presentación de tres casos y revisión de la literatura / Charles Bonnet syndrome: Report of three cases

Charles Bonnet syndrome is characterized by the presence of visual hallucinations associated with loss of vision. We report three patients aged 74, 84 and 80 years (two women) with a severe loss of vision due to ocular diseases and silent visual hallucinations, that they recognized as unreal. Two patients felt that the hallucinations were ominous. Years later, two suffered a probable Alzheimer's disease (AD). One of them had a mild cognitive impairment when the syndrome appeared, that evolved into an AD with psychosis. This syndrome is not uncommon in older patients with loss of vision and it is probably underdiagnosed. Its pathogenesis is probably a cortical deafferentation. The content of the visual hallucinations (faces, landscaped, and so on) coincides with the activation of different areas of the visual association cortices).

A Case of Charles Bonnet Syndrome Following Cerebral Infarction in the Right Occipital Lobe

Charles Bonnet syndrome is characterized by relatively preserved cognitive functions with the exception of complex visual hallucinations caused by injury to the visual system. We report the case of a 68 year-old woman presenting with colored, dynamic and organized visual hallucinations in the left visual field, which suddenly developed about 3 months ago. Brain MRI showed chronic infarction in the right occipital lobe. Treatment with olanzapine reduced the visual hallucinations. This is a rare case of Charles Bonnet syndrome following an occipital lobe infarction.