[Charles Bonnet syndrome. A 45-case series]. / Sindrome de Charles Bonnet. Serie de 45 casos.

INTRODUCTION: Charles Bonnet Syndrome (CBS) is a condition characterised by the presence of visual hallucinations, mainly complex, in patients with significant vision loss and without cognitive impairment. The rise in CBS cases is due to an increased life expectancy and to the development of age-related pathologies such as age-related macular degeneration (AMD). PATIENTS AND METHODS: We herein analyse the main characteristics present in 45 patients diagnosed with CBS at the Neuro-ophthalmology Unit in Hospital Clinico San Carlos. The patients were referred from the macular pathology, glaucoma and ocular surface units, as well as from AE, where they were diagnosed with CBS and later confirmed at the Multidisciplinary Unit formed by the ophthalmology, neurology and psychiatry services of the hospital. RESULTS: Women (66.66%) over 80 constituted 68.88% of the patients and mainly had AMD (37.77%). The most prevalent hallucinations described by the patients were of people and faces (35.55%), in colour (66.66%), in movement (80%), had developed over a period of 6 to 12 months (26.66%), had a frequency of 3 episodes per day (35.55%) and lasted between 3 to 5 minutes (35.55%). CONCLUSIONS: CBS is a complex disorder that requires a multidisciplinary approach from neurologists, psychiatrists, general practitioners and ophthalmologists. New studies are needed in order to understand its clinical presentation and behaviour, and thus improve its management.

TITLE: Sindrome de Charles Bonnet. Serie de 45 casos.Introduccion. El sindrome de Charles Bonnet (SCB) es un cuadro clinico que se caracteriza por la presencia de alucinaciones visuales, principalmente complejas, en pacientes con estado cognitivo conservado e importante deterioro de la vision. El incremento del SCB se debe al aumento de la esperanza de vida y al desarrollo de patologias asociadas al envejecimiento, como la degeneracion macular asociada a la edad. Pacientes y metodos. Se estudian las caracteristicas de una serie de 45 pacientes diagnosticados de SCB en la unidad de neurooftalmologia del Hospital Clinico San Carlos. Los pacientes procedian de las unidades de patologia macular, glaucoma, superficie ocular y urgencias, en las que fueron diagnosticados de SCB, que se confirmo en la unidad multidisciplinar formada por oftalmologia, neurologia y psiquiatria del mismo hospital. Resultados. El 66,66% eran mujeres, de mas de 80 anos (68,88%), principalmente con degeneracion macular asociada a la edad (37,77%). Las alucinaciones que los pacientes presentaban con mas frecuencia eran personas y caras (35,55%), en color (66,66%), en movimiento (80%), con un tiempo de evolucion de 6-12 meses (26,66%), frecuencia de tres episodios al dia (35,55%) y de 3-5 minutos de duracion (35,55%). Conclusiones. El SCB es un complejo sindrome cuya incidencia se esta incrementando en nuestras consultas y que precisa un abordaje multidisciplinar entre oftalmologos, neurologos y psiquiatras para evitar diagnosticos erroneos y proporcionar un tratamiento adecuado. Son necesarios nuevos estudios para un conocimiento mas profundo y adecuado del SCB.

Síndrome de Charles Bonnet. Serie de 45 casos / Charles Bonnet syndrome. A 45-case series

Introducción. El síndrome de Charles Bonnet (SCB) es un cuadro clínico que se caracteriza por la presencia de alucinaciones visuales, principalmente complejas, en pacientes con estado cognitivo conservado e importante deterioro de la visión. El incremento del SCB se debe al aumento de la esperanza de vida y al desarrollo de patologías asociadas al envejecimiento, como la degeneración macular asociada a la edad. Pacientes y métodos. Se estudian las características de una serie de 45 pacientes diagnosticados de SCB en la unidad de neurooftalmología del Hospital Clínico San Carlos. Los pacientes procedían de las unidades de patología macular, glaucoma, superficie ocular y urgencias, en las que fueron diagnosticados de SCB, que se confirmó en la unidad multidisciplinar formada por oftalmología, neurología y psiquiatría del mismo hospital. Resultados. El 66,66% eran mujeres, de más de 80 años (68,88%), principalmente con degeneración macular asociada a la edad (37,77%). Las alucinaciones que los pacientes presentaban con más frecuencia eran personas y caras (35,55%), en color (66,66%), en movimiento (80%), con un tiempo de evolución de 6-12 meses (26,66%), frecuencia de tres episodios al día (35,55%) y de 3-5 minutos de duración (35,55%). Conclusiones. El SCB es un complejo síndrome cuya incidencia se está incrementando en nuestras consultas y que precisa un abordaje multidisciplinar entre oftalmólogos, neurólogos y psiquiatras para evitar diagnósticos erróneos y proporcionar un tratamiento adecuado. Son necesarios nuevos estudios para un conocimiento más profundo y adecuado del SCB (AU)

Introduction. Charles Bonnet Syndrome (CBS) is a condition characterised by the presence of visual hallucinations, mainly complex, in patients with significant vision loss and without cognitive impairment. The rise in CBS cases is due to an increased life expectancy and to the development of age-related pathologies such as age-related macular degeneration. Patients and methods. We herein analyse the main characteristics present in 45 patients diagnosed with CBS at the Neuroophthalmology Unit in Hospital Clínico San Carlos. The patients were referred from the macular pathology, glaucoma and ocular surface units, as well as from A&E, where they were diagnosed with CBS and later confirmed at the Multidisciplinary Unit formed by the ophthalmology, neurology and psychiatry services of the hospital. Results. Women (66.66%) over 80 constituted 68.88% of the patients and mainly had age-related macular degeneration (37.77%). The most prevalent hallucinations described by the patients were of people and faces (35.55%), in colour (66.66%), in movement (80%), had developed over a period of 6 to 12 months (26.66%), had a frequency of three episodes per day (35.55%) and lasted between 3 to 5 minutes (35.55%). Conclusions. CBS is a complex disorder that requires a multidisciplinary approach from neurologists, psychiatrists, general practitioners and ophthalmologists. New studies are needed in order to understand its clinical presentation and behaviour, and thus improve its management (AU)

Síndrome de Charles Bonnet plus / Charles Bonnet plus syndrome

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Prevalence and clinical characteristics of Charles Bonnet syndrome in Madrid, Spain.

PURPOSE: Charles Bonnet syndrome (CBS) is a condition characterized by development of visual hallucinations in patients with no cognitive impairment and significant loss of vision mainly caused by age-related macular degeneration (AMD) or glaucoma. METHODS: This was a study of prevalence and characteristics of CBS diagnosed at the Neuroophthalmic Unit within the Ophthalmology Department of Hospital Clínico San Carlos (HCSC), Madrid, Spain. RESULTS: The CBS prevalence in patients from HCSC Madrid is 0.47%, rising to 15% in patients with low vision. Women over 80 years of age comprised 58.3% of the patients, who mainly had AMD (58.3%). Main characteristics of hallucinations included animals (50%), color (58.3%), moving (75%), 6- to 12-month evolution (50%), three times a day frequency (75%), and 3- to 5-minute duration (50%). CONCLUSIONS: Charles Bonnet syndrome is a complex process that must be treated jointly by ophthalmologists, neurologists, and psychiatrists in order to ensure accurate diagnosis and adequate management. New studies are needed in order to improve awareness of clinical manifestation of this condition, the incidence of which is underestimated due to patients' fear of being branded mentally ill, as well as physicians' lack of knowledge about CBS.

Clinical relevance of foveal location on retinal nerve fiber layer thickness using the new FoDi software in spectralis optical coherence tomography.

PURPOSE: To evaluate the effect of improper foveal location on retinal nerve fiber layer (RNFL) thickness measurements by using the new FoDi software in spectral-domain optical coherence tomography (Spectralis SDOCT). METHODS: Cross-sectional study with 126 subjects: 66 healthy, 30 early, and 30 moderate glaucomatous eyes. Fast RNFL scans were performed by using the new FoDi technology. The position of the fovea was manually displaced inferiorly after acquisition (producing clockwise torsion of scan circle) and then superiorly (counterclockwise) to generate study sets of images. Differences in RNFL thickness between foveal-guided and alternative scans were analyzed and color changes in sector charts were evaluated. RESULTS: In healthy eyes, placing the fovea inferiorly led to significant RNFL thickness changes in all sectors. Locating the fovea superiorly seemed to have less impact. Early glaucomatous eyes were more susceptible to quantitative changes, but moderate glaucomatous eyes were more susceptible to qualitative changes. CONCLUSIONS: Improper fovea disc alignment when using the FoDi software in Spectralis OCT significantly affected sectoral RNFL thickness measurements and color chart representation. As final report of FoDi analysis does not show the foveal position used, careful acquisition is encouraged, so that results are reliable. Otherwise, this technique can easily be misinterpreted and patients could be misdiagnosed.

Simultaneous optic and vestibulocochlear syphilitic neuropathy in a patient with HIV infection.

BACKGROUND: The purpose of this report is to present a case of optic and vestibulocochlear neuropathy as a manifestation of concurrent HIV and syphilis coinfection. This is an interventional case report of a 37-year-old man who complained of blurry central vision in his left eye and hearing loss in his left ear over the past 2 weeks. FINDINGS: Visual acuity was 20/20 in both eyes, and the anterior segment was normal in both eyes without relative afferent pupillary defect. Fundoscopy revealed swelling of the left optic disc. Optic coherence tomography and the Heidelberg retina tomograph confirmed and quantified the oedema of the left optic disc. An audiometry showed a left sensorineural deafness. Serological examinations disclosed confirmed HIV and syphilis infection. Magnetic resonance imaging of the brain showed no abnormalities. Properly treated with intravenous penicillin, the lesions resolved. CONCLUSIONS: Simultaneous optic and auditive involvement can be the first manifestation of syphilitic and HIV coinfection. To our knowledge, this report is the first to describe the rare occurrence of syphilitic optic neuritis and ipsilateral affectation of the vestibulocochlear nerve.

Charles Bonnet plus syndrome: apropos of a case.

PURPOSE: Charles Bonnet syndrome plus is an exceedingly rare variant of this disorder. The variant has been described in patients with sight impairment and severe hypoacusis, and is usually characterized by complex visual and auditory--musical--hallucinations that the patients recognize as unreal. METHOD: Case report. RESULTS: A 75-year-old woman diagnosed with Usher syndrome presented with visual acuity of light perception in both eyes, which did not improve with the use of a pinhole occluder. She also had coptosis in right ear and severe hypoacusis in left ear, confirmed through audiometry. Audiometric tests were normal once the implant and the hearing aid were connected. The patient was referred to the Neuro-Ophthalmology Unit after recounting experiencing complex visual hallucinations, as well as auditory (musical) ones at night after disconnecting the hearing aid. She described the latter as a nightly occurrence of hearing "cabaret music." Nevertheless, she was aware of reality and of her sensory impairments. The patient was diagnosed at the interdisciplinary Neuro-Ophthalmology Unit, and began pharmacologic treatment with clear improvement. CONCLUSIONS: Knowledge of Charles Bonnet syndrome and in particular of Charles Bonnet syndrome plus--due to its infrequency--on the part of ophthalmologists is fundamental to adequately diagnose and treat this rare disorder.