Granulomatous meningoencephalitis and blindness associated with Mycobacterium tuberculosis complex infection in a senile female chimpanzee (Pan troglodytes).

A 40-year old female chimpanzee (Pan troglodytes) developed hyporexia, weight loss, followed by progressive and complete blindness. Tomography demonstrated an intracranial mass in the rostroventral brain involving the optic chiasm, with a presumptive diagnosis of neoplasm. However, histopathology revealed a granulomatous meningoencephalitis, and tissue samples tested positive for Mycobacterium tuberculosis.

[Perioperative visual loss : Rare, unknown, relevant?] / Perioperativer Visusverlust : Selten, unbekannt, relevant?

Perioperative visual loss (POVL) is a rare but severe complication following non-ophthalmological surgery under general anesthesia. A POVL can be caused by lesions in any part of the optical system. The predominant causes include corneal injuries and particularly ischemic damage. The symptoms of POVL substantially vary ranging from reduced vision to complete blindness. The risks involve factors related to the surgery as well as patient-specific factors. In general, the prognosis in cases of mechanical damage is better than for ischemic lesions. The treatment measures depend on the underlying pathomechanism and due to the limited evidence only a few treatment options are available. Therefore, preventive measures and meticulous documentation play a crucial role.

Sudden Vision Loss.

A 37-year-old woman was referred for evaluation of a retinal detachment in her left eye. Posterior examination results demonstrated a retinal detachment in the posterior pole with shifting fluid and no identifiable retinal break, and there was a thickened choroid with a hyporeflective band on ultrasound biomicroscopy. What would you do next?

Navigating diagnostic and therapeutic challenges in ocular manifestations of Hansen's disease and trachoma: A narrative review.

Neglected tropical diseases (NTDs) encompass a group of approximately 20 diseases prevalent in tropical and subtropical regions, closely associated with poverty, affecting over a billion people in low-income countries. This manuscript aims to explore the ocular manifestations and burden of two significant NTDs, namely Hansen's disease and trachoma while addressing gaps in understanding and management. Hansen's disease, caused by Mycobacterium leprae , has a long history and presents with diverse neurological and ocular manifestations. Despite the availability of treatment, ocular complications persist, leading to significant visual impairment in some cases. The manuscript emphasizes the importance of early diagnosis, regular ophthalmic examinations, and follow-ups to prevent and control ocular complications, reducing the burden of visual impairment and blindness. Trachoma, caused by Chlamydia trachomatis , remains the leading infectious cause of blindness in underdeveloped and remote areas. The manuscript highlights the clinical diagnosis and implementation of the World Health Organization's (WHO's) SAFE (surgery, antibiotics, facial hygiene, and environmental sanitation) strategy to prevent transmission and associated blindness. However, challenges in health surveillance tools and underreporting of trachoma cases are addressed, emphasizing the need for improved strategies to combat the disease effectively. Through a comprehensive review of the ocular manifestations and management of Hansen's disease and trachoma, this manuscript contributes to the existing knowledge base and enhances a deeper understanding of these NTDs. Addressing gaps in understanding and management emphasizes the importance of implementing WHO's strategies and collaborative efforts to achieve the global goal of reducing the burden of NTDs and improving community health and well-being. The manuscript underscores the significance of early intervention, preventive measures, and technological advancements, providing valuable insights for policymakers, healthcare professionals, and researchers working in the field of NTDs.

Evaluating the accuracy of the Ophthalmologist Robot for multiple blindness-causing eye diseases: a multicentre, prospective study protocol.

INTRODUCTION: Early eye screening and treatment can reduce the incidence of blindness by detecting and addressing eye diseases at an early stage. The Ophthalmologist Robot is an automated device that can simultaneously capture ocular surface and fundus images without the need for ophthalmologists, making it highly suitable for primary application. However, the accuracy of the device's screening capabilities requires further validation. This study aims to evaluate and compare the screening accuracies of ophthalmologists and deep learning models using images captured by the Ophthalmologist Robot, in order to identify a screening method that is both highly accurate and cost-effective. Our findings may provide valuable insights into the potential applications of remote eye screening. METHODS AND ANALYSIS: This is a multicentre, prospective study that will recruit approximately 1578 participants from 3 hospitals. All participants will undergo ocular surface and fundus images taken by the Ophthalmologist Robot. Additionally, 695 participants will have their ocular surface imaged with a slit lamp. Relevant information from outpatient medical records will be collected. The primary objective is to evaluate the accuracy of ophthalmologists' screening for multiple blindness-causing eye diseases using device images through receiver operating characteristic curve analysis. The targeted diseases include keratitis, corneal scar, cataract, diabetic retinopathy, age-related macular degeneration, glaucomatous optic neuropathy and pathological myopia. The secondary objective is to assess the accuracy of deep learning models in disease screening. Furthermore, the study aims to compare the consistency between the Ophthalmologist Robot and the slit lamp in screening for keratitis and corneal scar using the Kappa test. Additionally, the cost-effectiveness of three eye screening methods, based on non-telemedicine screening, ophthalmologist-telemedicine screening and artificial intelligence-telemedicine screening, will be assessed by constructing Markov models. ETHICS AND DISSEMINATION: The study has obtained approval from the ethics committee of the Ophthalmology and Optometry Hospital of Wenzhou Medical University (reference: 2023-026 K-21-01). This work will be disseminated by peer-review publications, abstract presentations at national and international conferences and data sharing with other researchers. TRIAL REGISTRATION NUMBER: ChiCTR2300070082.

Auditory Spatial Bisection of Blind and Normally Sighted Individuals in Free Field and Virtual Acoustics.

Sound localization is an important ability in everyday life. This study investigates the influence of vision and presentation mode on auditory spatial bisection performance. Subjects were asked to identify the smaller perceived distance between three consecutive stimuli that were either presented via loudspeakers (free field) or via headphones after convolution with generic head-related impulse responses (binaural reproduction). Thirteen azimuthal sound incidence angles on a circular arc segment of ±24° at a radius of 3 m were included in three regions of space (front, rear, and laterally left). Twenty normally sighted (measured both sighted and blindfolded) and eight blind persons participated. Results showed no significant differences with respect to visual condition, but strong effects of sound direction and presentation mode. Psychometric functions were steepest in frontal space and indicated median spatial bisection thresholds of 11°-14°. Thresholds increased significantly in rear (11°-17°) and laterally left (20°-28°) space in free field. Individual pinna and torso cues, as available only in free field presentation, improved the performance of all participants compared to binaural reproduction. Especially in rear space, auditory spatial bisection thresholds were three to four times higher (i.e., poorer) using binaural reproduction than in free field. The results underline the importance of individual auditory spatial cues for spatial bisection, irrespective of access to vision, which indicates that vision may not be strictly necessary to calibrate allocentric spatial hearing.

Managing the apparently blind child presenting in the first year of life: A review.

Severe vision impairment and blindness in childhood have a significant health burden on the child, family and society. This review article seeks to provide a structured framework for managing the apparently blind child presenting in the first year of life, starting from a comprehensive history and examination. Different investigation modalities and the increasingly important role of genetics will also be described, in addition to common causes of severe vision impairment. Crucially, a systematic approach to the blind infant is key to correct diagnoses and timely management. Incorrect diagnoses can be costly to all involved, however it is important to note that diagnoses can change with ongoing follow-up and investigations. Furthermore, the modern age of ophthalmology requires a multi-disciplinary approach and close collaboration with specialists including paediatricians, neurologists and geneticists, in addition to rehabilitation and low vision services, to ensure the best care for these vulnerable infants.

Vision loss due to atypical bilateral edema of the optic nerve in a patient with hereditary angioedema: A case report.

PURPOSE: To describe a rare case of vision loss due to bilateral edema of the optic nerve in a patient with Hereditary Angioedema, treated with prophylactic C1-esterase inhibitor. METHODS: A 60-year-old Caucasian male affected by Hereditary Angioedema with unknown genetic defect (HAE- UNK) was admitted to our hospital presenting bilateral vision loss (best corrected visual acuity of 20/32 in the right eye and hand motion in the left eye) during an HAE attack. Intravenous administration of C1- esterase inhibitor (C1-INH, 1500 IU, Berinert, CSL Behring) determined the resolution of facial and periorbital swelling, however visual impairment persisted, in contrast with previous attacks experienced by the patient. Fundus examination revealed a vital optic disc without papilledema in both eyes. Magnetic resonance imaging (MRI) of the head and orbits showed bilateral edema of the optic nerve sheath. Treatment with intravenous and oral steroids was ineffective. Subsequently, a prophylactic treatment strategy with subcutaneous C1-esterase inhibitor was started (7000 IU every four days). RESULTS: Complete regression of edema of the optic nerves was observed by imaging at two months of follow-up after chronic treatment with C1-esterase inhibitor (7000 IU every four days). Complete restoration of visual acuity was achieved (BCVA 20/20 in both eyes) and multimodal imaging of the optic nerves demonstrated the absence of anatomical and functional damage. CONCLUSION: Patients affected by HAE may show atypical presentation with edema of the optic nerves without involvement of the optic nerve head. They may significantly benefit from prophylactic and chronic treatment with C1-esterase inhibitor.

A SYSTEMATIC LITERATURE REVIEW OF DISEASE PROGRESSION REPORTED IN RPGR -ASSOCIATED X-LINKED RETINITIS PIGMENTOSA.

PURPOSE: Retinitis pigmentosa GTPase regulator-associated X-linked retinitis pigmentosa ( RPGR -associated XLRP) is a rare and severe form of retinitis pigmentosa, resulting in progressive visual impairment; however, disease progression data are limited. A systematic literature review was conducted to assess available data on disease progression in RPGR -associated XLRP. METHODS: PubMed, Embase, and select congress abstracts were evaluated through June 2022. Eligible studies included results specific to RPGR -associated XLRP or populations with ≥80% of patients with retinitis pigmentosa carrying disease-causing RPGR variants. End points of interest included visual acuity, visual field, ellipsoid zone width, progression to blindness, and patient-reported outcomes. RESULTS: Fourteen studies met ≥1 end point of interest. Progressive declines in visual acuity, visual field, and ellipsoid zone width were reported across studies. Nearly all publications reported annual declines in visual acuity (3.5%-8.2%). Annual visual field declines ranged from 4.2% to 13.3%. Changes in retinal structure were also observed (ellipsoid zone width changes: -177 to -830 µ m/year). Most studies measured blindness using visual acuity; visual field-based definitions resulted in blindness by age ∼25 years. Patient-reported outcome data were limited. CONCLUSION: Published evidence shows that patients with RPGR -associated XLRP experience progressive decline in visual acuity, visual field, and ellipsoid zone width, eventually resulting in blindness. Additional longitudinal data with standardized end points and expanded collection of patient-reported outcomes are needed to assess visual decline in RPGR -associated XLRP.

Novel mutation in the NDP gene associated with Norrie disease in a Chinese pedigree.

PURPOSE: Norrie disease (ND) is a rare X-linked recessive disorder characteristic of early childhood blindness. While several mutations in the NDP gene have been reported as causative for ND, the genetic etiology remains unknown for many patients. This study aims to describe a novel mutation and explore the clinical manifestations in a Chinese family with two affected males. METHODS: Exome sequencing (ES) was employed to identify the causative gene in a four-generation pedigree. Sanger sequencing was subsequently utilized to validate the mutation detected by ES in additional family members. Ophthalmologic examination and diagnostic imaging relevant to ND were conducted. RESULTS: The proband (IV:2), an 8-month-old male infant, presented with binocular retinal detachment. DNA sequencing revealed a novel heterozygous missense mutation (c.174G>C) within the NDP gene in the proband. This mutation affected highly conserved residues and was predicted to disrupt the normal protein structure. Furthermore, the variant co-segregated with the disease phenotypes within the family. CONCLUSIONS: Our findings identified a novel missense mutation in the NDP gene associated with Norrie disease in China, expanding the mutation spectrum associated with ND. This discovery holds diagnostic, prognostic, and genetic counseling implications for affected individuals.

Case report: GCA like picture-preceding inaugural MOGAD presentation: A patient with a sudden-onset uniocular blindness.

RATIONALE: Myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) represents a demyelinating neurological syndrome characterized by the presence of serum IgG antibodies directed against myelin oligodendrocyte glycoprotein (MOG-IgG). Concurrently, giant cell arteritis (GCA) constitutes a systemic autoimmune vasculitis. PATIENT CONCERNS: In this case, we describe an elderly female patient who presented with the sudden onset of a severe headache, unilateral blindness, and clinical manifestations resembling those of GCA. DIAGNOSIS: Upon conducting a comprehensive analysis of serum antibodies, the diagnosis of MOGAD was established due to the presence of detectable serum MOG-IgG. INTERVENTIONS: Subsequently, the patient was administered intravenous methylprednisolone therapy, commencing 27 days after the initial onset of symptoms. OUTCOMES: It is noteworthy that patients afflicted by MOGAD typically manifest severe visual impairment, which, in many instances, exhibits significant improvement following immunotherapeutic interventions. However, this particular patient did not experience any amelioration in visual function despite glucocorticoid therapy. LESSONS: This unique case illustrates that the clinical presentation resembling GCA may precede the inaugural manifestation of MOGAD. This suggests the possibility of immune-mediated arterial involvement. The significance of glucocorticoid therapy in the context of immune-related diseases warrants further scrutiny, particularly in cases where MOG-IgG screening should be promptly considered.

Acute bilateral blindness due to diffuse outer retinopathy following clear lens exchange: a case report.

BACKGROUND: As the trend of refractive lens exchange for presbyopia continues to grow, our case report shows the first occurrence of an acute bilateral outer retinopathy following uncomplicated sequential clear lens extraction in an otherwise healthy individual. CASE PRESENTATION: A 54-year-old male without significant medical history benefited from a sequential bilateral lens exchange for presbyopia. He then experienced a rapid vision loss in both eyes, accompanied by photopsias and myodesopsias, with symptoms appearing respectively 4 and 3 weeks after the surgeries. Multimodal imaging revealed a fulminant outer retinopathy, leading to a total loss of light perception within a few days. Immediate intravenous corticosteroid therapy was administered, permitting to recover a small area of central visual function in both eyes, enabling shape and color distinction. The primary diagnostic hypothesis is a presumed autoimmune retinopathy, triggered by the cataract extraction, while an alternative diagnosis could be a toxic reaction secondary to the use of intracameral cefuroxime and lidocaine during the surgery. CONCLUSION: In this report, the authors describe the first recorded instance of outer retinopathy following cataract surgery. This occurrence raises the possibility of auto-immunization leading to retinal atrophy and vision loss as a potential outcome after undergoing cataract surgery.

Methods for a population-based Comprehensive Eye care Workload Assessment (CEWA) study in Southern India.

Eye care programs, in developing countries, are often planned using the prevalence of blindness and visual impairment, often estimated from Rapid Assessment of Avoidable Blindness (RAAB) surveys. A limitation of this planning approach is that it ignores the annual overall eye care requirements for a given population. Moreover, targets set are arbitrary, often influenced by capacity rather than need. To address this lacunae, we implemented a novel study design to estimate the annual need for comprehensive eye care in a 1.2 million populations. We conducted a population-based longitudinal study in Theni district, Tamil Nadu, India. All permanent residents of all ages were included. We conducted the study in three phases, (i) household-level enumeration and enrollment, (ii) basic eye examination (BEE) at household one-year post-enrollment, and (iii) assessment of eye care utilization and full eye examination (FEE) at central locations. All people aged 40 years and above were invited to the FEE. Those aged <40 years were invited to the FEE if indicated. In the main study, we enrolled 24,327 subjects (58% aged below 40 years and 42% aged 40 years and above). Of those less than 40 years, 72% completed the BEE, of whom 20% were referred for FEE at central location. Of the people aged ≥40 years, 70% underwent FEE. Our study design provides insights for appropriate long-term public health intervention planning, resource allocation, effective service delivery, and designing of eye care services for resource-limited settings.