Atypical Presentations of Extraparenchymal Neurocysticercosis.

BACKGROUND: Neurocysticercosis (NCC) is the most common parasitic infection of the central nervous system and is typically diagnosed through visualization of the cysts in the cerebral parenchyma by neuro-imaging. However, neuro-imaging may not detect extraparenchymal neurocysticercosis (EPNCC), which is a rare manifestation of the disease involving the subarachnoid, meningeal, and intraventricular spaces. We report 2 cases of extraparenchymal neurocysticercosis, and discuss the diagnostic challenges and management of this entity. METHODS: Two cases were identified through clinical records. RESULTS: Both patients had an insidious onset with slow progression of disease, and presented with papilledema and cerebrospinal fluid (CSF) eosinophilia. One case was diagnosed with spinal cord biopsy. The other was diagnosed with CSF serology and next-generation sequencing-based pathogen analysis. Both patients were treated with ventriculoperitoneal shunt, systemic antiparasitic agents, and immunosuppression. CONCLUSIONS: EPNCC is less common than parenchymal NCC. A high level of clinical suspicion is required given its rarity, long incubation period, and slow progression. Diagnosis and treatment can be challenging and requires a multidisciplinary approach.

Tele-Neuro-Ophthalmology Utilization, Availability, and Attitudes: Update 1 Year Into the COVID-19 Public Health Emergency.

BACKGROUND: Telehealth was rapidly adopted early in the COVID-19 pandemic as a way to provide medical care while reducing risk of SARS-CoV2 transmission. Since then, telehealth utilization has evolved differentially according to subspecialty. This study assessed changes in neuro-ophthalmology during the first year of the COVID-19 pandemic. METHODS: Telehealth utilization and opinions pre-COVID-19, early pandemic (spring 2020), and 1 year later (spring 2021) were surveyed among practicing neuro-ophthalmologists in and outside the United States using an online platform. Demographics, self-reported utilization, perceived benefits, barriers, and examination suitability were collected over a 2-week period in May 2021. RESULTS: A total of 135 practicing neuro-ophthalmologists (81.5% United States, 47.4% females, median age 45-54 years) completed the survey. The proportion of participants using video visits remained elevated during COVID + 1 year (50.8%) compared with pre-COVID (6%, P < 0.0005, McNemar), although decreased compared with early COVID (67%, P < 0.0005). Video visits were the most commonly used methodology. The proportion of participants using remote testing (42.2% vs 46.2%), virtual second opinions (14.5% vs 11.9%, P = 0.45), and eConsults (13.5% vs 16.2%, P = 0.38) remained similar between early and COVID + 1 year ( P = 0.25). The majority selected increased access to care, better continuity of care, and enhanced patient appointment efficiency as benefits, whereas reimbursement, liability, disruption of in-person clinic flow, limitations of video examinations, and patient technology use were barriers. Many participants deemed many neuro-ophthalmic examination elements unsuitable when collected during a live video session, although participants believed some examination components could be evaluated adequately through a review of ancillary testing or outside records. CONCLUSIONS: One year into the COVID-19 pandemic, neuro-ophthalmologists maintained telemedicine utilization at rates higher than prepandemic levels. Tele-neuro-ophthalmology remains a valuable tool in augmenting patient care.

Pituitary macroadenoma causing vision loss in Wyburn-Mason syndrome: illustrative case.

BACKGROUND: Wyburn-Mason syndrome (WMS) is a neurocutaneous disorder consisting of vascular malformations of the brain, eye, and skin. These include characteristically high-flow intracranial and intraorbital arteriovenous malformations (AVMs) that present commonly with visual deterioration, headache, and hemiplegia. Complete removal of these lesions is challenging. Most patients are followed closely, and intervention occurs only in the setting of worsening symptoms secondary to AVM growth or hemorrhage. Here the authors present the first known case of a patient with WMS and a pituitary macroadenoma. OBSERVATIONS: A 62-year-old man with a 30-year history of WMS with right basal ganglia and orbital AVMs and right eye blindness presented for new-onset left-sided vision loss. A pituitary adenoma was identified compressing the optic chiasm and left optic nerve. Magnetic resonance imaging and digital subtraction angiography studies were obtained for surgical planning, and the patient underwent an endoscopic transnasal transsphenoidal resection, with significant postoperative vision improvement. LESSONS: Given the variable presentation and poor characterization of this rare syndrome, patients with WMS presenting with new symptoms must undergo evaluation for growth and hemorrhage of known AVMs, as well as new lesions. Further, in patients undergoing intracranial surgery, extensive preoperative imaging and planning are crucial for safe and successful procedures.

Perioperative Care of the Patient with Eye Pathologies Undergoing Nonocular Surgery.

The authors reviewed perioperative ocular complications and implications of ocular diseases during nonocular surgeries. Exposure keratopathy, the most common perioperative eye injury, is preventable. Ischemic optic neuropathy, the leading cause of perioperative blindness, has well-defined risk factors. The incidence of ischemic optic neuropathy after spine fusion, but not cardiac surgery, has been decreasing. Central retinal artery occlusion during spine fusion surgery can be prevented by protecting eyes from compression. Perioperative acute angle closure glaucoma is a vision-threatening emergency that can be successfully treated by rapid reduction of elevated intraocular pressure. Differential diagnoses of visual dysfunction in the perioperative period and treatments are detailed. Although glaucoma is increasingly prevalent and often questions arise concerning perioperative anesthetic management, evidence-based recommendations to guide safe anesthesia care in patients with glaucoma are currently lacking. Patients with low vision present challenges to the anesthesia provider that are becoming more common as the population ages.

Perioperative Ischemic Optic Neuropathy after Cardiac Surgery: Development and Validation of a Preoperative Risk Prediction Model.

OBJECTIVE: Previous studies identified risk factors for ischemic optic neuropathy (ION) after cardiac surgery; however, there is no easy-to-use risk calculator for the physician to identify high-risk patients for ION before cardiac surgery. The authors sought to develop and validate a simple-to-use predictive model and calculator to assist with preoperative identification of risk and informed consent for this rare but serious complication. DESIGN: Retrospective case-control study. SETTING: Hospital discharge records. PATIENTS: A total of 5,561,177 discharges in the National Inpatient Sample >18 years of age, with procedure codes for coronary artery bypass grafting, heart valve repair/replacement, or left ventricular assist device insertion. INTERVENTIONS: All patients had undergone cardiac surgery. MEASUREMENTS AND MAIN RESULTS: Known preoperative risk factors for ION after cardiac surgery were assessed to develop a risk score and prediction model. This model was validated internally using the split-sample method. There were 771 cases of ION among 5,561,177 patients in the National Inpatient Sample. The risk factors for ION used in the model were carotid artery stenosis, cataract, diabetic retinopathy, macular degeneration, glaucoma, male sex, and prior stroke; whereas uncomplicated diabetes decreased risk. With the internal validation, the predictive model had an area under the receiver operating characteristic curve of 0.66. A risk score cutoff ≥3 had 98.4% specificity. CONCLUSIONS: This predictive model, based on previously identified preoperative factors, predicted risk of perioperative ION with a fair area under the receiver operating characteristic curve. This predictive model could enable screening to provide a more accurate risk assessment for ION, and consent process for cardiac surgery.

Predictive Value of International Classification of Diseases Codes for Idiopathic Intracranial Hypertension in a University Health System.

BACKGROUND: Misclassification bias is introduced into medical claims-based research because of reliance on diagnostic coding rather than full medical record review. We sought to characterize this bias for idiopathic intracranial hypertension (IIH) and evaluate strategies to reduce it. METHODS: A retrospective review of medical records was conducted using a clinical data warehouse containing medical records and administrative data from an academic medical center. Patients with 1 or more instances of International Classification of Diseases (ICD)-9 or -10 codes for IIH (348.2 or G93.2) between 1989 and 2017 and original results of neuroimaging (head CT or MRI), lumbar puncture, and optic nerve examination were included in the study. Diagnosis of IIH was classified as definite, probable, possible, or inaccurate based on review of medical records. The positive predictive value (PPV) for IIH ICD codes was calculated for all subjects, subjects with an IIH code after all testing was completed, subjects with high numbers of IIH ICD codes and codes spanning longer periods, subjects with IIH ICD codes associated with expert encounters (ophthalmology, neurology, or neurosurgery), and subjects with acetazolamide treatment. RESULTS: Of 1,005 patients with ICD codes for IIH, 103 patients had complete testing results and were included in the study. PPV of ICD-9/-10 codes for IIH was 0.63. PPV in restricted samples was 0.82 (code by an ophthalmologist n = 57), 0.70 (acetazolamide treatment n = 87), and 0.72 (code after all testing, n = 78). High numbers of code instances and longer duration between the first and last code instance also increased the PPV. CONCLUSIONS: An ICD-9 or -10 code for IIH had a PPV of 63% for probable or definite IIH in patients with necessary diagnostic testing performed at a single institution. Coding accuracy was improved in patients with an IIH ICD code assigned by an ophthalmologist. Use of coding algorithms considering treatment providers, number of codes, and treatment is a potential strategy to reduce misclassification bias in medical claims-based research on IIH. However, these are associated with a reduced sample size.

Patient Personality and Illness Perceptions in Relation to Follow-Up Appointment Adherence in Neuro-Ophthalmology.

BACKGROUND: Improving patient attendance at medical follow-up visits may have a notable impact on disease and overall health outcomes. Understanding factors contributing to poor attendance is important for identifying at-risk patients and designing interventions to improve clinical outcomes. Our objective was to identify personality and illness perception factors associated with attendance at recommended follow-up visits in a neuro-ophthalmology practice. METHODS: New or established patients (≥18 years) with scheduled neuro-ophthalmology (study) or glaucoma (comparison) appointments at a tertiary care academic medical center completed the Brief Illness Perception Questionnaire and Ten-Item Personality Inventory. Physician recommendations made during the visit were recorded (medications, referrals, follow-up, testing, and procedures). A chart review was performed 18 months after enrollment to assess attendance at follow-up appointment and adherence with other physician recommendations. Multiple variable logistic regression models studied associations between follow-up appointment attendance and demographic factors, appointment factors, and survey responses. RESULTS: Among 152 respondents (97% response rate (152 of 157), aged 19-97 years, 58% female, 34% new, 80 neuro-ophthalmology, 72 glaucoma), neuro-ophthalmology subjects were younger, more likely to be White, non-Hispanic, female and new to the practice than subjects with glaucoma. They reported higher emotional impact, identity, and consequences related to their illness (P = 0.001-0.03). Neuro-ophthalmology physician recommendations included more referrals to other services (17.5% vs 1.4%, P = 0.001, chi-square) and more radiology studies (15% vs 0%, P = 0.001, chi-square), but fewer follow-up visits (75% vs 97%, P < 0.0005, chi-square). Among those with recommended follow-up visits, neuro-ophthalmology subjects had lower rates of on-time appointment attendance (55% vs 77%, P = 0.009, chi-square). In a multiple variable model, on-time follow-up attendance was associated with shorter recommended follow-up interval (≤90 days, P < 0.0005), established (vs new) patient status at enrollment visit (P = 0.04), and glaucoma (P = 0.08), but not subject demographics, illness perception, or personality factors. CONCLUSIONS: Patient demographics, illness perception, and personality traits were not associated with follow-up appointment attendance and therefore unlikely to be useful for identifying patients at risk of being lost to follow-up. New neuro-ophthalmology patients with a follow-up recommended ≥90 days in advance may benefit from targeted interventions to improve follow-up appointment adherence.

Availability of the HPV Vaccine in Regional Pharmacies and Provider Perceptions Regarding HPV Vaccination in the Pharmacy Setting.

There is increasing support for HPV vaccination in the pharmacy setting, but the availability of the HPV vaccine is not well known. Additionally, little is known about perceptions of medical providers regarding referring patients to community pharmacies for HPV vaccination. The purpose of this study was to determine HPV vaccine availability in community pharmacies and to understand, among family medicine and obstetrics-gynecology providers, the willingness of and perceived barriers to referring patients for HPV vaccination in a pharmacy setting. HPV vaccine availability data were collected from pharmacies in a southern region of the United States. Family medicine and obstetrics-gynecology providers were surveyed regarding vaccine referral practices and perceived barriers to HPV vaccination in a community pharmacy. Results indicated the HPV vaccine was available in most pharmacies. Providers were willing to refer patients to a community pharmacy for HPV vaccination, despite this not being a common practice, likely due to numerous barriers reported. Pharmacist-administered HPV vaccination continues to be a commonly reported strategy for increasing HPV vaccination coverage. However, coordinated efforts to increase collaboration among vaccinators in different settings and to overcome systematic and legislative barriers to increasing HPV vaccination rates are still needed.

Neuro-ophthalmology and Pregnancy.

PURPOSE OF REVIEW: This article summarizes the impact of pregnancy on neuro-ophthalmic pathways and presents an approach to the evaluation of pregnant women who have neuro-ophthalmic symptoms or signs. RECENT FINDINGS: Advances in noninvasive ophthalmic imaging have increased knowledge of the impact of pregnancy on ocular blood flow, which may have relevance for understanding the impact of preeclampsia and eclampsia on the eye. SUMMARY: The framework for approaching neuro-ophthalmic symptoms and signs in pregnant women is similar to the general approach for people who are not pregnant. Visual symptoms are common in preeclampsia and eclampsia. Some diseases that impact the neuro-ophthalmic pathways are more common in pregnant women. Pregnancy should be considered when recommending the workup and treatment for neuro-ophthalmic symptoms and signs.

Diagnostic performance of neuroimaging in suspected idiopathic intracranial hypertension.

The diagnostic utility of neuroradiologic signs associated with idiopathic intracranial hypertension (IIH) for the evaluation of patients presenting with papilloedema remains yet to be elucidated. This multicentre retrospective cohort study assessed consecutive patients presenting with suspected papilloedema to Auckland District Health Board (NZ) and Stanford University Medical Centre (US), between 2005 and 2019, undergoing magnetic resonance imaging and venography (MRI/MRV) or computed tomography and venography (CT/CTV) prior to lumbar puncture assessment for diagnostic suspicion of IIH. Data were collected regarding demographic, clinical, radiologic, and lumbar puncture parameters, and the diagnosis of IIH was determined according to the Friedman criteria for primary pseudotumor cerebri syndrome. A total of 204 participants (174 females; mean ± SD age 29.9 ± 12.2 years) were included, and 156 (76.5%) participants fulfilled the diagnostic criteria for IIH. The presence of any IIH-associated radiologic sign on MRI/MRV demonstrated a sensitivity (95% CI) of 74.8% (65.8%-82.0%) and specificity (95% CI) of 94.7% (82.7%-98.5%), while radiologic signs on CT/CTV exhibited a sensitivity (95% CI) of 61.0% (49.9%-71.2%) and specificity (95% CI) of 100.0% (83.2%-100.0%). In summary, the modest sensitivities of radiologic signs of IIH would support the routine use of lumbar puncture assessment following neuroimaging to secure the diagnosis. However, the high specificities might lend limited support for the judicious deferment of lumbar puncture assessment among typical IIH demographic patients who consent to the inherent small risk of missed pathology, which has been proposed by some clinicians.

Functional vision disorders in adults: a paradigm and nomenclature shift for ophthalmology.

Vision loss with clinical findings that are incompatible with the symptoms and recognized neurological or ophthalmic conditions is a common presentation of patients to neurologists, ophthalmologists, and neuro-ophthalmologists. The accepted terminology to describe such patients has evolved over time, including functional visual disorder (FVD), non-organic vision loss, non-physiologic vision loss, functional vision loss, psychogenic, psychosomatic, and medically unexplained visual loss. Likewise, attitudes and recommended management options have changed over the years in the fields of psychiatry and neurology. FVD is a diagnosis of inclusion, and it is critical that the diagnosis be made and delivered efficiently and effectively to reduce patient and physician duress. We review the current Diagnostic and Statistical Manual (DSM V) terminology and the prior literature on FVD and describe how the approaches to diagnosis and management have changed. We provide recommendations on the appropriate techniques and diagnostic approach for patients with FVD. We also propose a protocol for consistent and standardized discussion with the patient of the diagnosis of FVD. We believe that the adoption of FVD as both a paradigm and nomenclature shift in ophthalmology will improve patient care.

Emerging concepts in the treatment of optic neuritis: mesenchymal stem cell-derived extracellular vesicles.

BACKGROUND: Optic neuritis (ON) is frequently encountered in multiple sclerosis, neuromyelitis optica spectrum disorder, anti-myelin oligodendrocyte glycoprotein associated disease, and other systemic autoimmune disorders. The hallmarks are an abnormal optic nerve and inflammatory demyelination; episodes of optic neuritis tend to be recurrent, and particularly for neuromyelitis optica spectrum disorder, may result in permanent vision loss. MAIN BODY: Mesenchymal stem cell (MSC) therapy is a promising approach that results in remyelination, neuroprotection of axons, and has demonstrated success in clinical studies in other neuro-degenerative diseases and in animal models of ON. However, cell transplantation has significant disadvantages and complications. Cell-free approaches utilizing extracellular vesicles (EVs) produced by MSCs exhibit anti-inflammatory and neuroprotective effects in multiple animal models of neuro-degenerative diseases and in rodent models of multiple sclerosis (MS). EVs have potential to be an effective cell-free therapy in optic neuritis because of their anti-inflammatory and remyelination stimulating properties, ability to cross the blood brain barrier, and ability to be safely administered without immunosuppression. CONCLUSION: We review the potential application of MSC EVs as an emerging treatment strategy for optic neuritis by reviewing studies in multiple sclerosis and related disorders, and in neurodegeneration, and discuss the challenges and potential rewards of clinical translation of EVs including cell targeting, carrying of therapeutic microRNAs, and prolonging delivery for treatment of optic neuritis.

Predictive Value of International Classification of Diseases Codes for Idiopathic Intracranial Hypertension in a University Health System.

BACKGROUND: Misclassification bias is introduced into medical claims-based research because of reliance on diagnostic coding rather than full medical record review. We sought to characterize this bias for idiopathic intracranial hypertension (IIH) and evaluate strategies to reduce it. METHODS: A retrospective review of medical records was conducted using a clinical data warehouse containing medical records and administrative data from an academic medical center. Patients with 1 or more instances of International Classification of Diseases (ICD)-9 or -10 codes for IIH (348.2 or G93.2) between 1989 and 2017 and original results of neuroimaging (head CT or MRI), lumbar puncture, and optic nerve examination were included in the study. Diagnosis of IIH was classified as definite, probable, possible, or inaccurate based on review of medical records. The positive predictive value (PPV) for IIH ICD codes was calculated for all subjects, subjects with an IIH code after all testing was completed, subjects with high numbers of IIH ICD codes and codes spanning longer periods, subjects with IIH ICD codes associated with expert encounters (ophthalmology, neurology, or neurosurgery), and subjects with acetazolamide treatment. RESULTS: Of 1,005 patients with ICD codes for IIH, 103 patients had complete testing results and were included in the study. PPV of ICD-9/-10 codes for IIH was 0.63. PPV in restricted samples was 0.82 (code by an ophthalmologist n = 57), 0.70 (acetazolamide treatment n = 87), and 0.72 (code after all testing, n = 78). High numbers of code instances and longer duration between the first and last code instance also increased the PPV. CONCLUSIONS: An ICD-9 or -10 code for IIH had a PPV of 63% for probable or definite IIH in patients with necessary diagnostic testing performed at a single institution. Coding accuracy was improved in patients with an IIH ICD code assigned by an ophthalmologist. Use of coding algorithms considering treatment providers, number of codes, and treatment is a potential strategy to reduce misclassification bias in medical claims-based research on IIH. However, these are associated with a reduced sample size.

The Impact of COVID-19 on Neuro-Ophthalmology Office Visits and Adoption of Telemedicine Services.

BACKGROUND: The COVID-19 public health emergency (PHE) has significantly changed medical practice in the United States, including an increase in the utilization of telemedicine. Here, we characterize change in neuro-ophthalmic care delivery during the early COVID-19 PHE, including a comparison of care delivered via telemedicine and in office. METHODS: Neuro-ophthalmology outpatient encounters from 3 practices in the United States (4 providers) were studied during the early COVID-19 PHE (March 15, 2020-June 15, 2020) and during the same dates 1 year prior. For unique patient visits, patient demographics, visit types, visit format, and diagnosis were compared between years and between synchronous telehealth and in-office formats for 2020. RESULTS: There were 1,276 encounters for 1,167 patients. There were 30% fewer unique patient visits in 2020 vs 2019 (477 vs 670) and 55% fewer in-office visits (299 vs 670). Compared with 2019, encounters in 2020 were more likely to be established, to occur via telemedicine and to relate to an efferent diagnosis. In 2020, synchronous telehealth visits were more likely to be established compared with in-office encounters. CONCLUSIONS: In the practices studied, a lower volume of neuro-ophthalmic care was delivered during the early COVID-19 public health emergency than in the same period in 2019. The type of care shifted toward established patients with efferent diagnoses and the modality of care shifted toward telemedicine.

Neuro-Ophthalmic Emergencies.

Neuro-ophthalmic emergencies include optic nerve, central visual pathway, and ocular motility disorders that, if not identified and treated promptly, may lead to permanent vision loss, other significant morbidity, or mortality. This article provides a framework for approaching patients with neuro-ophthalmic symptoms and reviews the presentation, evaluation, and treatment of select emergent conditions that can cause them. Emergent causes of blurry vision, transient vision loss, papilledema, and diplopia, including giant cell arteritis, cardioembolic disease, and aggressive infection, are discussed.

Predictive model of ischemic optic neuropathy in spinal fusion surgery using a longitudinal medical claims database.

BACKGROUND CONTEXT: Perioperative ischemic optic neuropathy (ION) is a devastating complication of spinal fusion surgery. PURPOSE: To develop predictive models of this blinding condition using a longitudinal medical administrative claims database, which provides temporal sequence of perioperative ischemic optic neuropathy and potential risk factors. DESIGN: Nested case control study. PATIENT SAMPLE: Participants in Cliniformatics Data Mart medical claims database (2007-2017) with hospitalization involving lumbar or thoracic spinal fusion surgery and no history of ION. OUTCOME MEASURES: Perioperative ION (or not) during hospitalization for lumbar or thoracic spinal fusion surgery. METHODS: Sixty-five ION cases and 106,871 controls were identified. Matched controls (n=211) were selected based on year of surgery and zip code. Chronic and perioperative variables were assigned based on medical claims codes. Least absolute shrinkage and selection (LASSO) penalized conditional logistic regression with 10-fold cross validation was used to select variables for the optimal predictive model from the subset of variables with p<.15 between cases and matched controls (unadjusted conditional logistic regression). Receiver operating characteristic (ROC) curves were generated for the strata-independent matched and full sample. RESULTS: The predictive model included age 57-65 years, male gender, diabetes with and without complications, chronic anemia, hypertension, heart failure, carotid stenosis, perioperative hemorrhage and perioperative organ damage. Area under ROC curve was 0.75 (95% confidence interval [CI]: 0.68, 0.82) for the matched sample and 0.72 (95% CI: 0.66, 0.78) for the full sample. CONCLUSIONS: This predictive model for ION in spine fusion considering chronic conditions and perioperative conditions is unique to date in its use of longitudinal medical claims data, inclusion of International Classification of Disease-10 codes and study of ophthalmic conditions as risk factors. Similar to other studies of this condition the multivariable model included age, male gender, perioperative organ damage and perioperative hemorrhage. Hypertension, chronic anemia and carotid artery stenosis were new predictive factors identified by 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.