A Feasibility Study on a Portable Vision Device for Patients with Stroke and Brain Tumours.

This prospective, single-centre cohort study aimed to evaluate the impact of a portable vision reading device, OrCam Read, on vision-related quality-of-life and independent functional status in patients with low vision due to stroke or brain tumours. Six patients with poor visual acuity or visual field defects due to a stroke or a brain tumour were enrolled at a U.S. Ophthalmology Department. Participants were trained to use OrCam Read and given a loaner device for the 1 month duration of the study. Various assessments, including daily function tests, the National Eye Institute Visual Function Questionnaire-25, and the 10-item neuro-ophthalmic supplement, were administered at the first and last visits. Patients' experience with the device was evaluated with weekly telephone and end-of-study satisfaction surveys. The main outcome measures were the patient satisfaction with OrCam and the mean assessment scores between enrolment and final visits. The intervention with OrCam significantly improved patients' ability to complete daily tasks and participants reported good satisfaction with the device. The results also show non-significant improvement with distant activities, dependency, and role difficulties. Our findings demonstrate the feasibility of studying vision-related quality-of-life using a portable vision device in this patient population and pave the way for a larger study to validate the results of this study.

Telemedicine for neuro-ophthalmology: challenges and opportunities.

PURPOSE OF REVIEW: Telemedicine for neuro-ophthalmology (tele-neuro-ophthalmology) has the potential to increase access to neuro-ophthalmic care by improving efficiency and decreasing the need for long-distance travel for patients. Requirements for decreased person-to-person contacts during the COVID-19 pandemic accelerated adoption of tele-neuro-ophthalmology. This review highlights the challenges and opportunities with tele-neuro-ophthalmology. RECENT FINDINGS: Tele-neuro-ophthalmology programs can be used for triage, diagnostic consultation, and long-term treatment monitoring. Formats include telephone appointments, interprofessional collaborations, remote data interpretation, online asynchronous patient communication, and video visits. Barriers to long-term implementation of tele-neuro-ophthalmology arise from data quality, patient engagement, workflow integration, state and federal regulations, and reimbursement. General neurologists may collaborate with local eye care providers for ophthalmic examination, imaging, and testing to facilitate efficient and effective tele-neuro-ophthalmology consultation. SUMMARY: Tele-neuro-ophthalmology has tremendous potential to improve patient access to high-quality cost-effective neuro-ophthalmic care. However, many factors may impact its long-term sustainability.

Racial, Ethnic, and Gender Diversity in United States Ophthalmology Clinical Trials.

Purpose: To investigate the representation of various gender, racial, and ethnic groups in ophthalmology clinical trials conducted in the United States (US) between 1997 and 2022. Design: Retrospective cross-sectional study. Participants: We included all participants in completed phase II/III, III, and IV ophthalmology clinical trials reported on the ClincialTrials.gov database. Methods: The proportional enrollment of each racial/ethnic and gender group in the clinical trials was calculated and compared with the US population. We also investigated the impact of various clinical trial features on the rate of reporting demographic information and enrollment of minorities. Main Outcome Measures: Proportional enrollment of each gender and race/ethnicity group compared with the US Census. Results: Of the total clinical trials included in the study, less than half (43.6%) provided information on the racial or ethnic backgrounds of their participants. The majority of the enrollees in trials were female (median: 57.5%, interquartile range [IQR]: 47.2%-65.8%). Among the trials that reported race and/or ethnicity data, White populations were overrepresented (median: 76.6%, IQR: 69.0%-84.0%, P = 0.001), and minorities, including Asian, Hispanic, and "other" groups, were underrepresented compared with the 2010 US Census (P < 0.001). Enrollment of Black individuals was found to be comparable to the US population estimates (median: 12.4%, IQR: 6.2%-20.8%, P = 0.44). The trial phase, the number of study participants, the primary clinical condition, and the year the trial started all affected demographic reporting and minority enrollments. Conclusions: Our findings highlight the need for increased efforts to promote diversity and inclusivity in ophthalmology clinical trials. Ensuring equitable inclusion of different gender, racial, and ethnic groups in the trials is essential for minimizing disparities and producing unbiased scientific findings generalizable to the entire population. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

A Novel Predictive Model Utilizing Retinal Microstructural Features for Estimating Survival Outcome in Patients with Glioblastoma.

Glioblastoma is a highly aggressive brain tumor with poor prognosis despite surgery and chemoradiation. The visual sequelae of glioblastoma have not been well characterized. This study assessed visual outcomes in glioblastoma patients through neuro-ophthalmic exams, imaging of the retinal microstructures/microvasculature, and perimetry. A total of 19 patients (9 male, 10 female, average age at diagnosis 69 years) were enrolled. Best-corrected visual acuity ranged from 20/20-20/50. Occipital tumors showed worse visual fields than frontal tumors (mean deviation - 14.9 and - 0.23, respectively, p < 0.0001). Those with overall survival (OS) < 15 months demonstrated thinner retinal nerve fiber layer and ganglion cell complex (p < 0.0001) and enlarged foveal avascular zone starting from 4 months post-diagnosis (p = 0.006). There was no significant difference between eyes ipsilateral and contralateral to radiation fields (average doses were 1370 cGy and 1180 cGy, respectively, p = 0.42). A machine learning algorithm using retinal microstructure and visual fields predicted patients with long (≥ 15 months) progression free and overall survival with 78% accuracy. Glioblastoma patients frequently present with visual field defects despite normal visual acuity. Patients with poor survival duration demonstrated significant retinal thinning and decreased microvascular density. A machine learning algorithm predicted survival; further validation is warranted.

Combined central retinal artery and medial posterior ciliary artery occlusion: Localizing the lesion.

Purpose: To report a rare case of a combined central retinal artery (CRA) and medial posterior ciliary artery (MPCA) occlusion due to an atherosclerotic lesion in the common trunk supplying both arteries. Observations: A 75-year-old man presented with acute vision loss associated with elevated intraocular pressure in the right eye. Multi-modal imaging revealed a combined retinal and choroidal infarction in the distribution of the CRA and MPCA, localizing the lesion to the common trunk of the ophthalmic artery supplying both the CRA and MPCA. Neurovascular imaging provided supportive evidence for the diagnosis. Conclusions and importance: A simultaneous retinal and choroidal vascular occlusion is an uncommon presentation. Familiarity with the anatomy of the ophthalmic arteries and its branches facilitates localizing the lesion.

Neuro-ophthalmic features of patients with spontaneous cerebrospinal fluid leaks.

Background: Increased intracranial pressure is a potential cause of spontaneous cerebrospinal fluid (sCSF) leak. Associated neuro-ophthalmic features have not been well studied, particularly relationships with idiopathic intracranial hypertension (IIH). We hypothesized that neuro-ophthalmic features routinely used in evaluations for IIH can be useful in the investigation of a causal relationship between IIH and sCSF leak. We reviewed the neuro-ophthalmic examination and office-based ophthalmic imaging data of all consecutive patients with sCSF leaks and at least one repair to investigate the clinical and neuro-ophthalmic features of increased intracranial pressure. Methods: We conducted a retrospective longitudinal study at a single institution by querying the electronic medical record system for CSF leak Current Procedural Terminology (CPT) codes (G96.00 and G96.01) from June 1, 2019, to July 31, 2022. For patients with a confirmed diagnosis of sCSF leak, demographic information, eye examination results, and ophthalmic imaging details for both eyes were collected. Results: A total of 189 patients with CSF leaks were identified through CPT coding; 159 had iatrogenic or traumatic CSF leaks, and 30 individuals (3 male, 27 female) had confirmed sCSF leaks. The mean age of patients with sCSF leaks was 46 years (range: 29 - 81), with a mean body mass index of 35.2 kg/m2 (range: 18.2 - 54.1). Only 11 of 30 underwent eye examinations (8 before surgical repair and 10 after). The mean pre-repair and post-repair best-corrected visual acuity were 20/30 (range: 20/20 - 20/55) and 20/25 (range: 20/20 - 20/40), respectively (P = 0.188). The mean retinal nerve fiber layer thickness was 99 µm (range: 96 - 104) pre-repair and 97 µm (range: 84 - 103) post-repair (P = 0.195). The mean ganglion cell complex thickness was 84 µm (range: 72 - 94) pre-repair and 82 µm (range: 71 - 94) post-repair (P = 0.500). Humphrey visual field average mean deviation was -5.1 (range: -12.4 - -1.8) pre-repair and -1.0 (range: -10.1 - 2.1) post-repair (P = 0.063). Conclusions: Serial neuro-ophthalmic examinations are recommended for patients with sCSF leaks to screen for signs of current or prior increased intracranial pressure. Larger studies are required to clarify the longitudinal changes in neuro-ophthalmic features, to investigate the incidence of IIH in cases of sCSF leak development or recurrence after surgical repair, and to explore potential causal relationships to guide post-repair management and prevent recurrent leaks. A multicenter consortium is also suggested to develop a standard clinical protocol for comprehensive management of sCSF leaks.

Application of Machine Learning to Metabolomic Profile Characterization in Glioblastoma Patients Undergoing Concurrent Chemoradiation.

We here characterize changes in metabolite patterns in glioblastoma patients undergoing surgery and concurrent chemoradiation using machine learning (ML) algorithms to characterize metabolic changes during different stages of the treatment protocol. We examined 105 plasma specimens (before surgery, 2 days after surgical resection, before starting concurrent chemoradiation, and immediately after chemoradiation) from 36 patients with isocitrate dehydrogenase (IDH) wildtype glioblastoma. Untargeted GC-TOF mass spectrometry-based metabolomics was used given its superiority in identifying and quantitating small metabolites; this yielded 157 structurally identified metabolites. Using Multinomial Logistic Regression (MLR) and GradientBoostingClassifier (GB Classifier), ML models classified specimens based on metabolic changes. The classification performance of these models was evaluated using performance metrics and area under the curve (AUC) scores. Comparing post-radiation to pre-radiation showed increased levels of 15 metabolites: glycine, serine, threonine, oxoproline, 6-deoxyglucose, gluconic acid, glycerol-alpha-phosphate, ethanolamine, propyleneglycol, triethanolamine, xylitol, succinic acid, arachidonic acid, linoleic acid, and fumaric acid. After chemoradiation, a significant decrease was detected in 3-aminopiperidine 2,6-dione. An MLR classification of the treatment phases was performed with 78% accuracy and 75% precision (AUC = 0.89). The alternative GB Classifier algorithm achieved 75% accuracy and 77% precision (AUC = 0.91). Finally, we investigated specific patterns for metabolite changes in highly correlated metabolites. We identified metabolites with characteristic changing patterns between pre-surgery and post-surgery and post-radiation samples. To the best of our knowledge, this is the first study to describe blood metabolic signatures using ML algorithms during different treatment phases in patients with glioblastoma. A larger study is needed to validate the results and the potential application of this algorithm for the characterization of treatment responses.

Ways to Improve Workflow and Morale in an Ophthalmology Clinic: Survey Advice from Clinic Staff.

Objective: We aim to improve job workflow and satisfaction amongst clinic staff at an academic ophthalmology department. Methods: We analyzed survey data given over a 2-week period in July 2021. The participants were support staff (N = 18) from an academic ophthalmology department. Paper surveys were distributed to participants and returned anonymously for analysis. Results: The survey contained 9 Likert-style categorical questions, 2 of which were free response options. A total of 22 participants attempted the survey, 18 of these (82%) were complete and included in analysis. About half of the staff were satisfied with the current workflow 10/18 (56%). Staff who were clinical care coordinators had the lowest average satisfaction (2/5 on a 5-point scale) and the nursing team had the highest average (4.75/5). The most common staff suggestion for improving workflow efficiency was to train residents on forwarding and answering messages more effectively. Conclusion: This survey suggests that assigning patient message processing to the nursing staff can improve job satisfaction and workflow. Staff told us that the most exciting part of the job was appreciation from coworkers 9/30 (30%) and from physicians 8/30 (27%). The findings provide advice to physicians for optimizing communication, and staff experience, within their own ophthalmology clinics.

The basic science of optic nerve regeneration.

Diverse insults to the optic nerve result in partial to total vision loss as the axons of retinal ganglion cells are destroyed. In glaucoma, axons are injured at the optic nerve head; in other optic neuropathies, axons can be damaged along the entire visual pathway. In all cases, as mammals cannot regenerate injured central nervous system cells, once the axons are lost, vision loss is irreversible. However, much has been learned about how retinal ganglion cells respond to axon injuries, and many of these crucial discoveries offer hope for future regenerative therapies. Here we review the current understanding regarding the temporal progression of axonal degeneration. We summarize known survival and regenerative mechanisms in mammals, including specific signaling pathways, key transcription factors, and reprogramming genes. We cover mechanisms intrinsic to retinal ganglion cells as well as their interactions with myeloid and glial cell populations in the retina and optic nerve that affect survival and regeneration. Finally, we highlight some non-mammalian species that are able to regenerate their retinal ganglion cell axons after injury, as understanding these successful regenerative responses may be essential to the rational design of future clinical interventions to regrow the optic nerve. In the end, a combination of many different molecular and cellular interventions will likely be the only way to achieve functional recovery of vision and restore quality of life to millions of patients around the world.

Aggressive FUS-Mutant Motor Neuron Disease Without Profound Spinal Cord Pathology.

A 29-year-old man presented with rapidly progressive severe neck weakness, asymmetrical bilateral upper extremity weakness, bulbar dysfunction, profound muscle wasting, and weight loss. Within 1 year, his speech became unintelligible, he became gastrostomy- and tracheostomy/ventilator-dependent, and wheelchair bound. Electrophysiology suggested motor neuron disease. Whole exome sequencing revealed a heterozygous pathogenic variant in the fused in sarcoma gene (FUS), c.1574C>T,p. R525L, consistent with autosomal dominant amyotrophic lateral sclerosis. Autopsy revealed extensive denervation atrophy of skeletal musculature. Surprisingly, there was only minimal patchy depletion of motor neurons within the cervico-thoracic spinal cord anterior horn cells, and the tracts were largely preserved. TDP-43 inclusions were absent. Abnormal expression of FUS mutation product (cytoplasmic inclusions) was demonstrated by immunohistochemistry within anterior horn motor neurons. The most prominent finding was a disparity between profound neck weakness and relatively low-grade anterior horn cell loss or tract degeneration in the cervico-thoracic cord.