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.

Surgical approach for traumatic chiasmal syndrome and complete third nerve palsy following severe head trauma.

Traumatic chiasmal syndrome and traumatic third nerve palsies are rare entities usually caused by severe, high-speed, closed-head road accidents that require a thorough diagnostic work-up and complex medical and surgical management. This report presents the case of a young adult who was involved in a motorcycle accident and sustained blunt frontal head trauma, resulting in optical chiasmal syndrome and complete unilateral third nerve palsy. Ophthalmological examination demonstrated a right complete ptosis, a downward and outward position of the right eye with a fixed and dilated pupil, and bitemporal hemianopsia. In addition, funduscopy revealed bilateral optical nerve atrophy. After stabilisation and during follow-up, strabismus surgery was performed with improvement of ocular alignment in the primary position. Subsequently, eyelid surgery was carried out with good amplification of the visual field, particularly on the left side. Although challenging, surgical intervention in these cases should be considered for both functional and cosmetic reasons.

Radiotherapy as Salvage Treatment in Intraocular Lymphoma: A Case Report.

A 67-year-old previously healthy woman presented with progressive visual impairment including bitemporal hemianopsia. A brain magnetic resonance imaging revealed a contrast-enhancing mass in the optic chiasm, spreading along the left optic tract. The patient underwent a transcranial biopsy of the left optical tract that yielded a diagnosis of diffuse large B-cell lymphoma. CT scans of the chest, abdomen, and pelvis, PET-CT, and bone marrow biopsy revealed no evidence of systemic lymphoma. Thus, the final diagnosis was of primary central nervous system lymphoma of the optic chiasm. Systemic treatment was initiated with full response. Six months after the end of the treatment, recurrence at cerebellum parenchyma and left tentorium was recorded. A new systemic treatment achieved full response. A second recurrence was noted in an optical coherence tomography of the right eye, 2 years after the initial diagnosis. The patient was treated with intravitreal methotrexate with initial success, but eventual failure after 10 months. Intravitreal rituximab was used with no effect. The patient was then referred to radiotherapy and underwent external beam radiotherapy with VMAT. There were no acute toxicities to report. After the radiotherapy treatment, at 1-year follow-up, the patient has no evidence of disease. Long-term toxicities were recorded and are considered manageable. The present case emphasizes the role of ocular irradiation as an option in the management of intraocular lymphoma patients, including in the salvage setting, with an acceptable ocular toxicity profile.