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BACKGROUND: The novel coronavirus 2019 (COVID-19) pandemic has transformed health care. With the need to limit COVID-19 exposures, telemedicine has become an increasingly important format for clinical care. Compared with other fields, neuro-ophthalmology faces unique challenges, given its dependence on physical examination signs that are difficult to elicit outside the office setting. As such, it is imperative to understand both patient and provider experiences to continue to adapt the technology and tailor its application. The purpose of this study is to analyze both neuro-ophthalmology physician and patient satisfaction with virtual health visits during the time of the COVID-19 pandemic. METHODS: Across three institutions (NYU Langone Health, Indiana University Health, and Columbia University Medical Center), telemedicine surveys were administered to 159 patients. Neuro-ophthalmologists completed 157 surveys; each of these were linked to a single patient visit. Patient surveys consisted of 5 questions regarding visit preparation, satisfaction, challenges, and comfort. The physician survey included 4 questions that focused on ability to gather specific clinical information by history and examination. RESULTS: Among 159 patients, 104 (65.4%) reported that they were satisfied with the visit, and 149 (93.7%) indicated that they were comfortable asking questions. Sixty-eight (73.9%) patients found the instructions provided before the visit easy to understand. Potential areas for improvement noted by patients included more detailed preparation instructions and better technology (phone positioning, Internet connection, and software). More than 87% (137/157) of neuro-ophthalmologists surveyed reported having performed an examination that provided enough information for medical decision-making. Some areas of the neuro-ophthalmologic examination were reported to be easy to conduct (range of eye movements, visual acuity, Amsler grids, Ishihara color plates, and pupillary examination). Other components were more difficult (saccades, red desaturation, visual fields, convergence, oscillations, ocular alignment, and smooth pursuit); some were especially challenging (vestibulo-ocular reflex [VOR], VOR suppression, and optokinetic nystagmus). Clinicians noted that virtual health visits were limited by patient preparation, inability to perform certain parts of the examination (funduscopy and pupils), and technological issues. CONCLUSIONS: Among virtual neuro-ophthalmology visits evaluated, most offer patients with appointments that satisfy their needs. Most physicians in this cohort obtained adequate clinical information for decision-making. Even better technology and instructions may help improve aspects of virtual health visits.

PURPOSE: To provide a summary of the neuro-ophthalmic manifestations of coronavirus disease 19 (COVID-19) documented in the literature thus far. METHODS: The PubMed and Google Scholar databases were searched using the keywords: Neuro-Ophthalmology, COVID-19, SARS-CoV-2, and coronavirus. A manual search through reference lists of relevant articles was also performed. RESULTS/CONCLUSIONS: The literature on COVID-associated neuro-ophthalmic disease continues to grow. Afferent neuro-ophthalmic complications associated with COVID-19 include optic neuritis, papillophlebitis, papilledema, visual disturbance associated with posterior reversible encephalopathy syndrome, and vision loss caused by stroke. Efferent neuro-ophthalmic complications associated with COVID-19 include cranial neuropathies, Miller Fisher syndrome, Adie's pupils, ocular myasthenia gravis, nystagmus and eye movement disorders. Proposed mechanisms of neurologic disease include immunologic upregulation, vasodilation and vascular permeability, endothelial dysfunction, coagulopathy, and direct viral neurotropism. When patients present to medical centers with new onset neuro-ophthalmic conditions during the pandemic, COVID-19 infection should be kept on the differential.

The role of systemic steroids in the treatment of ophthalmoplegia in the setting of herpes zoster ophthalmicus (HZO) is controversial. We conducted a case report-based meta-analysis to investigate the role of systemic steroid in the recovery of efferent dysfunctions in HZO.Case-report based meta-analysis.We report a case of herpes zoster ophthalmicus-related ophthalmoplegia (HZORO) in which systemic steroid led to complete resolution of external ophthalmoplegia. We further identified subjects from published cases of HZO-related ophthalmoplegia by searching PubMed and Google Scholar, which elicited 42 articles (49 cases) after excluding those younger than 18 years or with incomplete follow-up data. With the present case, a total of 50 cases are included in the analysis. Main outcome measure is the recovery of efferent dysfunction at the last known follow-up, coded as 1 for complete recovery or 0 for noncomplete recovery. We performed multivariable linear regression and Cox proportional hazards analysis to determine the contribution of steroid duration on the status of complete recovery.Multivariable linear regression showed significant association between duration of steroid treatment and status of complete recovery (P.001). Cox proportional hazards analysis showed a hazard ratio of 1.1 (P = .04), indicating that longer treatment duration increased chance of complete recovery. Age, gender, and initial steroid dose did not contribute significantly to recovery status.Our meta-analysis suggests that extended steroid taper may aid the recovery of ophthalmoplegia in the setting of HZO and should be investigated further in the future.

Background Invasive fungal sinusitis carries high morbidity and mortality and often poses a diagnostic challenge. Orbital apex syndrome (OAS) is not an uncommon presentation in the setting of invasive fungal sinusitis. Delays in diagnosis and appropriate treatment can result in permanent visual dysfunction and, potentially, death. We present 2 cases of OAS secondary to invasive sinus aspergillosis, detailing the diagnostic process, treatment, and outcome for both patients. Subsequently, we present a review of the literature and combined analysis of our 2 patients plus 71 cases from previously published reports. Methods Literature review was performed to identify demographic, diagnostic, clinical, and treatment data of patients with OAS caused by Aspergillus species. Results The review resulted in 52 included articles with 71 patients, plus our 2 reported patients, leading to a total of 73 subjects included in the analysis. The average age of patients at presentation was 59.9 years. A combination of visual disturbance and pain (headache and/or periocular pain) was the most common presentation reported (46 cases; 63%). Diabetes mellitus was reported in 15 cases (21%), with more than half specifically noted to have poorly controlled diabetes. After diabetes, the second most common cause of immunocompromise was chronic steroid use (n = 13; 18%). Empiric antifungal treatment was started in 10 patients (14%), while 25 patients (34%) were first treated with systemic steroids due to a concern for an inflammatory etiology. Time to diagnosis from initial presentation was on average 7.4 weeks (range of 0.3-40 weeks). Approximately 78% of the cases (57 of 73) had biopsies with histology that confirmed Aspergillus fungal morphology, and 30/73 (41%) had diagnostic fungal cultures. The majority of the cases received monotherapy with intravenous (IV) amphotericin B (36 patients; 49%) and IV voriconazole (19 patients; 26%), with a combination of the 2 or more antifungal agents being used in 11 patients (15%). Forty patients (55%) showed signs of clinical improvement with treatment, while 33 (45%) patients did not experience any improvement or continued to deteriorate, and 23 (32%) died in the course of their reported follow-up. Conclusions The present cases illustrate well the challenge in the diagnosis and treatment of OAS due to invasive sinus aspergillosis. Our review and analysis of 73 cases support the notion that a high index of suspicion leading to early biopsy with histology and fungal culture is paramount for diagnosis. Early empiric antifungal treatment and debridement can potentially reduce morbidity and mortality.

BackgroundIdiopathic intracranial hypertension is a disease of raised intra-cranial pressure of unknown etiology. Lateral cerebral venous sinus stenosis (VSS) has been increasingly reported in these patients, and stenting has emerged as an alternative treatment for medically refractory symptoms. Treatment efficacy on meta-analysis appears promising, but identifying which patients are likely to benefit most, and which are likely to require repeat procedures, is currently unclear.MethodsWe retrospectively reviewed a prospectively collected database of 79 patients treated with venous sinus stenting at a single academic center with minimum follow-up of 18 months. We extracted baseline clinical data, as well as manometry at lumbar puncture and during angiography, and used logistic regression to identify parameters that could predict stent failure.ResultsRetreatment rate after successful VSS was 13.9%. Lumbar puncture opening pressure (OP) was shown to significantly predict treatment failure (ß=0.06; OR=1.064 (1.003–1.135); P=0.039). This effect remained significant when age, sex and body mass index were added to the model (ß=0.06; OR=1.066 (1.002–1.140); P=0.043). OP was correlated with venous sinus manometry readings in the superior sagittal and transverse sinus pre-stent placement, as well trans-stenotic gradient (PConclusionsHigher lumbar puncture OP was associated with an increased risk of stent failure in transverse sinus stenting for idiopathic intracranial hypertension, although the performance of this model as a linear discriminator was poor. Further studies are required to better assess which patients are at greatest risk of treatment failure.

Visual complaints are commonly encountered by the practicing neurologist. We review assessment of vision loss, diplopia, and positive visual phenomena, all of which require a thoughtful evaluation to localize disease and refine management. While many causative entities are unlikely to cause poor visual outcomes, including dry eyes, migraine, and congenital strabismus, others may threaten vision, life, or both, such as posterior communicating artery aneurysms, pituitary apoplexy, or temporal arteritis. A systematic approach to vision loss and diplopia is reviewed along with focused differential diagnoses.

Background: Due to the COVID-19 pandemic, there was a surge in synchronous ophthalmic telehealth visits. The purpose of this study is to analyze the utilization and patient satisfaction of synchronous ophthalmic video visits over the course of the COVID-19 pandemic. Methods: In this retrospective, single-center cross-sectional study, 1,756 patients seen through synchronous video visits between March 1, 2020, and March 31, 2021, were identified using billing codes. E-mails containing a validated, 11-item, telehealth satisfaction scale were sent to patients who had at least one video visit within the study period. Questions were scored on a 1-4 scale, corresponding to poor, fair, good, and excellent. Main outcome measures included patient satisfaction scores, frequency of repeat video visits, and primary visit diagnoses. Results: The top 3 subspecialties by virtual visit volume were oculoplastic surgery (999 visits, 42.9%), neuro-ophthalmology (331 visits, 17.0%), and cornea (254 visits, 14.2%). The top 3 diagnoses seen were chalazion/hordeolum, dry eye, and meibomian gland dysfunction. The overall survey response rate was 14.3% (252 participants). The mean patient satisfaction score was 3.67 ± 0.63, with no significant difference in scores between specialties. A total of 380 (21%) patients had repeat virtual visits. Mean survey response scores were significantly higher for patients with repeat visits than those without (3.82 ± 0.42 vs. 3.62 ± 0.68, p = 0.03). Patients undergoing oculoplastic services were more likely to have repeat visits (odds ratio 2.58, 95% confidence interval 2.18-3.06, p < 0.001). Multivariate regression analysis found that provider thoroughness/skillfulness was the most predictive feature of the patient returning to a telehealth encounter (p = 0.01). Conclusions: Our study suggests that synchronous videoconferencing for ophthalmology is a highly satisfactory delivery method and will likely find continued success in select subspecialties as the pandemic fades.

Neurologic complications of COVID-19 are not well described. We report 2 patients who were diagnosed with COVID-19 after presenting with diplopia and ophthalmoparesis.

Purpose of Review Giant cell arteritis (GCA), a medium and large vessel vasculitis occurring in the aged, remains a formidable disease, capable of taking both vision and life, through a multitude of vascular complications. Our understanding of the spectrum of its manifestations has grown over the years, to include limb claudication, aortitis, and cardiac disease, in addition to the more classic visual complications resulting from of ischemia to branches of the external and internal carotid arteries. While a clinical presentation of headache, jaw claudication, scalp tenderness, fever and other systemic symptoms and serum markers are together highly suggestive of the disease, diagnosis can be challenging in those cases in which classic symptoms are lacking. The purpose of this review is to update the reader on advances in the diagnosis and treatment of giant cell arteritis and to review our evolving understanding of the immunological mechanism underlying the disease, which have helped guide our search for novel therapies. Recent Findings There is increasing evidence supporting the use of Doppler ultrasound, dedicated post-contrast T1-weighted spin echo MRI of the scalp arteries and PET scan, which can together improve our diagnostic accuracy in cases in which temporal artery biopsy is either inconclusive or not feasible. Advances in our understanding of the immunological cascades underlying the disease have helped guide our search for steroid-sparing treatments for the GCA, the most important of which has been the IL-6 receptor antibody inhibitor tocilizumab, which has been shown to reduce cumulative steroid dose in a large multicenter, placebo-controlled prospective study. Other biologic agents, such as abatacept and ustekinumab have shown promise in smaller studies. Summary GCA is no longer a disease whose diagnosis is based exclusively on temporal artery biopsy and whose complications are prevented solely with the use of corticosteroids. Modern vascular imaging techniques and targeted immunologic therapies are heralding a new era for the disease, in which practitioners will hopefully be able to diagnosis it with greater accuracy and treat it with less ischemic complications and iatrogenic side effects.

Background Venous sinus stenting (VSS) is an accepted and minimally invasive treatment for adult idiopathic intracranial hypertension (IIH) associated with lateral sinus stenosis (LSS). The efficacy and safety of venous sinus stenting (VSS) in children with IIH has not been established. Methods This is a retrospective analysis of IIH patients 18 years of age or younger with LSS treated with VSS at our institution. Included patients have fulminant disease course or are refractory or intolerant to medical management. Results Eight patients were identified; 4 males and 4 females. Mean age is 13.4 years (range 4–18). All patients had severe headaches, 5 had blurred vision, 3 had diplopia and 3 had pulsatile tinnitus. Papilledema was present in 4 patients. Three patients had prior surgical procedures. Four patients were intolerant to medical management, 3 were refractory and 1 had fulminant course. Cerebral venography demonstrated severe stenosis of the dominant sinus in 6 patients and of bilateral co-dominant sinuses in 2 patients. Six patients had intrinsic stenosis and 2 had extrinsic stenosis. Venous sinus stenting (VSS) resulted in improvement of symptoms, papilledema and normalization of CSF opening pressure in 7 patients. No immediate complications were observed. Mean follow-up period is 21 months (range 6–42). Two patients required re-stenting; one responded well and the other had persistent symptoms and underwent subsequent surgical procedures of CSF diversion, suboccipital decompression and duraplasty which were also ineffective. Conclusion VSS may provide a viable option for pediatric IIH patients who are intolerant to medication, have failed conservative management or prior surgical interventions, or present with fulminant disease.

Background Oncogenic driver alterations in FGFR are present in a subset of pediatric gliomas. Debio1347 is an orally available, highly selective FGFR 1–3 inhibitor that had a favorable safety profile and encouraging preliminary clinical activity in an adult phase 1 study. Methods Five children with progressive/refractory CNS tumors harboring an FGFR gene alteration following prior therapy were treated with Debio1347 at Memorial Sloan Kettering Cancer Center on single patient use protocols. Patients were treated using the 20 mg tablet formulation at the adult recommended phase 2 dose (80 mg/1.73 m2 * BSA once daily). Toxicities were graded using CTCAEv5.0 and imaging response assessments were performed every 8–12 weeks. RESULTS All AEs were grade 1–2. Most common treatment-related adverse events were hyperphosphatemia, ALT increased and hypoalbuminemia (4 patients). Two patients met criteria for partial response and two patients had stable disease. A 13 month-old patient with a spinal cord high-grade glioma harboring two FGFR1 mutations (V592M, K687E) had tumor reduction of 91.7% maintained for 12 months. A 26-month-old patient with a pilomyxoid astrocytoma harboring an FGFR1-TACC1 fusion had a tumor reduction of 74.5% maintained for 9 months. Molecular characterization of recurrent tumor from this patient demonstrated an NF1 deletion as a novel molecular mechanism of acquired resistance to FGFR inhibition. Prolonged disease stabilization was noted in an eight year-old patient with metastatic suprasellar pilomyxoid astrocytoma harboring an FGFR1 mutation (9 months) and in a 14 year-old patient with posterior fossa glioneuronal tumor harboring an FGFR3-TACC3 fusion (24 months and ongoing). Conclusions Debio1347 demonstrated tolerable toxicity and promising anti-tumor efficacy in pediatric patients with refractory FGFR altered gliomas. Specific attention to growth velocity and clinical symptoms with incorporation of imaging assessment of bone growth is warranted. Candidate biomarkers (FGFR1 V592M and K687E SNVs, FGFR-TACC fusions) may guide patient selection. Further studies in this population are warranted.

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PurposeThe impact of venous sinus stenting (VSS) on vein of Labbé (VOL) drainage is poorly understood. The purpose of the study is to examine the incidence and potential high risk factors of impaired VOL drainage among idiopathic intracranial hypertension (IIH) patients following VSS.Materials and methodsInstitutional review board approved prospective evaluation of all IIH patients who underwent VSS over a 5 year period (January 2012 to December 2017) at Weill Cornell Medical Center constituted the study population. Patient demographics, procedural details (laterality of stenting, balloon angioplasty, number of stents, and stent diameters), morphology of the VOL and changes in the flow in the VOL, type of sinus stenosis, and transverse sinus symmetry were evaluated. We used χ2 analysis to evaluate impaired VOL drainage against other variables. Statistical significance was set at 0.05.Results70 consecutive patients (67 women, 3 men) with a mean age of 31±9.8 years underwent VSS. Stenosis was extrinsic in 63% (n=44) and intrinsic in 37% (n=26) of patients. Impaired drainage of the VOL was detected in 9/70 (13%) patients. Ipsilateral VOL was recognized as dominant in 20% (n=14), co-dominant in 51% (n=36), and non-dominant in 29% (n=20) of patients. Impaired VOL drainage was significantly associated with ipsilateral VOL dominance (P=0.001) and stent diameter of ≥9 mm (P=0.042). All patients demonstrated widely patent VOL on follow-up contrast enhanced MR venography at 3 months and 24 months.ConclusionImpaired drainage of the ipsilateral VOL is a potential consequence of VSS with 13% incidence, and has significant association with ipsilateral superficial cortical venous drainage via dominant VOL and stent diameter of ≥9 mm.Clinical trial registrationNCT01407809.

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disease primarily affecting obese women of child-bearing age, typically presenting with positional headaches, diplopia from abducens nerve palsies, pulse-synchronous tinnitus, and visual disturbances related to papilledema, including transient visual obscurations, blurry vision, and visual field defects. Controversy remains regarding the pathophysiology of IIH. Elevated intracranial pressure in IIH is thought to be the result of a dysregulation of cerebrospinal fluid (CSF) dynamics, through excessive secretion, reduced drainage, or both. Passive CSF drainage through the arachnoid granulations depends on a pressure gradient between the subarachnoid space and the cerebral venous system, such that elevations in cerebral venous pressure lead to impaired CSF drainage. Dural venous sinus stenosis (DVSS) at the junction of the transverse and sigmoid sinuses, leading to local venous hypertension and a secondary reduction in CSF drainage through the arachnoid granulations, is increasingly recognized as an important component of the pathophysiology of IIH. A growing number of studies have demonstrated a role for stenting of DVSS as an alternative to CSF shunting and optic nerve sheath fenestration in medically refractory IIH patients with vision loss or debilitating symptoms.

Purpose of review This article discusses the varied types of paraneoplastic syndromes that commonly have neuro-ophthalmologic manifestations. Diagnostic considerations and therapeutic options for individual diseases are also discussed. Recent findings Paraneoplastic syndromes can affect the afferent and efferent visual systems. Paraneoplastic syndromes may result in reduced visual acuity from retinal degeneration, alterations in melanocyte proliferation and uveal thickening, or acquired nystagmus. Ocular motor abnormalities related to paraneoplastic syndromes may present with symptoms from opsoclonus or from neuromuscular junction disease. Diagnosis remains challenging, but serologic identification of some specific antibodies may be helpful or confirmatory. Treatment, in addition to directed therapies against the underlying cancer, often requires systemic corticosteroids, plasma exchange, or immunosuppression, but some specific syndromes improve with use of targeted pharmacologic therapy. Summary Diagnosis and therapy of paraneoplastic syndromes presenting with neuro-ophthalmic symptoms remain a challenge, but strategies are evolving and new approaches are on the horizon.

AimsWe report the cerebrospinal fluid opening pressure (CSF-OP) measurements obtained before and after venous sinus stenting (VSS) in 50 patients with idiopathic intracranial hypertension.MethodsThe CSF-OP was measured with a spinal tap 3 months before and 3 months after treatment. All data were prospectively collected and included patient demographics, weight (kg), body mass index (BMI), acetazolamide daily dosage (mg), procedural details, complications, venous sinus pressures (mm Hg), trans-stenotic pressure gradient (mm Hg), transverse sinus symmetry, and type of venous sinus stenosis.ResultsThe average pretreatment CSF-OP was 37 cm H2O (range 25–77) and the average post-treatment CSF-OP was 20.2 cm H2O (range 10–36), with an average reduction of 16.8 cm H2O (P2O in 40/50 patients. The average acetazolamide daily dose decreased from 950 mg to 300 mg at the time of 3-month follow-up (PConclusionsWe provide evidence that there is a significant decrease in CSF-OP in patients with idiopathic intracranial hypertension 3 months after VSS, independent of acetazolamide usage or weight loss.

Idiopathic intracranial hypertension causes headache, papilledema and visual field loss, typically in obese women of childbearing age. Its anatomical underpinnings remain unclear, but a stenosis at the junction of the transverse and sigmoid sinuses has been recognized in the majority of patients through venography. The stenosis may result from intrinsic dural sinus anatomy or extrinsic compression by increased intracranial pressure, but in either case, its stenting has been shown to lead to an improvement in symptoms of intracranial hypertension and papilledema in multiple retrospective, non-controlled studies. Prospective, controlled trials are needed to confirm its efficacy and safety.

A 54-year-old woman presented with a 30-year history of worsening left blepharoptosis. Neuro-ophthalmic examination was significant for blepharoptosis, limited levator function, and supraduction in the left eye with left hypotropia, and normal pupils without diplopia. MRI brain/orbits revealed subtle atrophy of the left superior rectus (figure 1, A and B), without any orbital pathology. Superior compression of the left oculomotor nerve by the posterior cerebral artery (PCA) was observed (figure 1, C–E). Given the microanatomy of oculomotor nerve in the cistern space,1,2 this is a rare selective compression of the superolateral oculomotor nerve by the PCA vessel (figure 2), sparing the superomedial parasympathetic limb.

Patients with visual loss from macroadenomas compressing their optic apparatus may also have concomitant age-related visual pathology such as cataracts. How these two pathologies interact with each other is not well documented. The interaction between these two pathologies in elderly patients is the subject of this study. We identified a series of non-functioning macroadenoma patients over age 50 years with tumors compressing the chiasm who underwent transsphenoidal surgery at our institution between 2004 and 2018. Pre- and post-operative visual complaints, tumor size and extent of resection were analyzed. Prevalence of the diagnosis of cataract and prevalence of cataract surgery in each decade were compared with national averages. We identified 200 patients who met selection criteria. 18% of these patients had a diagnosis of cataract and 12.5% had cataract surgery. Compared with the Eye Diseases Prevalence Research Group (EDPRG) study, the prevalence of cataract surgery was 2.5 times the national average of 5.1%. 32% of these patients had no improvement in their vision after cataract surgery but 76% improved after transsphenoidal surgery. We reported a high prevalence of cataract surgery in patients over age 50 in patients with pituitary macroadenomas compressing the optic pathway compared with national averages in patients without adenomas. While visual loss from adenoma likely precipitated more cataract surgeries in this group of patients, some who may not have required it, those patients with cataracts who did not have their cataracts extracted were less likely to recover vision after transsphenoidal surgery. Addressing both pathologies is beneficial.

Idiopathic intracranial hypertension is a syndrome affecting mostly obese women of childbearing age leading to symptoms and signs of elevated intracranial pressure including papilledema. Management includes weight loss and medications that reduce intracranial pressure, but when these conservative therapies fail, surgical intervention may be warranted to prevent potentially irreversible vision loss. Based upon an observed association between stenosis at the junction of the transverse and sigmoid venous sinuses, endovascular stenting of the stenotic region has emerged as a potential therapeutic option, alongside more established surgical interventions such as CSF shunt procedures and optic nerve sheath fenestration. In this chapter, we review the theoretical basis explaining the relationship between venous stenosis and idiopathic intracranial hypertension, and summarize the outcomes associated with this innovative treatment to date.

To describe a case of optic neuropathy associated with intravascular lymphoma (IVL).Case report and review of the literature.A case of asymmetric binocular vision loss is described, preceded by transient vision loss. Associated optic perineural enhancement and enhancing and diffusion-positive cortical lesions were observed on magnetic resonance imaging. Biopsy of the cerebellum revealed exclusively intraluminal neoplastic B-cells consistent with IVL.Patients with IVL may rarely present with optic nerve involvement, presumably due to small vessel occlusion. The presentation may mimic features of anterior ischemic optic neuropathy including an acute onset and disc edema. Although optic nerve enhancement and associated white matter lesions may suggest optic neuritis, enhancement of the optic nerve sheath, as in this case, has a wide differential diagnosis, which includes giant cell arteritis. IVL should be considered in atypical cases of optic neuropathy accompanied by enhancing, diffusion-positive brain lesions that are not within a specific vascular territory.

Rhinocerebral mucormycosis is a life-threatening fungal disease due to Rhizopus or Mucor species. Infection typically occurs in patients who are immunocompromised or have poorly controlled diabetes.1 The classical syndrome consists of a rapidly progressive sinusitis, periorbital edema and cellulitis, ophthalmoplegia, and unilateral visual loss.1–3 We report rapidly progressive bilateral visual loss from mucormycosis due to bilateral optic nerve and retinal infarction in a patient with diabetes.1

BACKGROUND The goal of our study was to look for the presence of homonymous ganglion cell layer-inner plexiform layer complex (GCL-IPL) thinning using spectral-domain optical coherence tomography (SD-OCT) in patients with a history of adult-onset injury to the postgeniculate pathways with rigorous radiological exclusion of geniculate and pregeniculate pathology. METHODS We performed a retrospective review of twenty-two patients (ages 24-75 y, 6 men, 16 women) with homonymous visual field (VF) defects secondary to postgeniculate injury examining the GCL-IPL with SD-OCT. An additional fifteen patients (ages 28-85 y, 5 men, 10 women) with no visual pathway pathology served as controls. Using segmentation analysis software applied to the macular scan, a normalized asymmetry score was calculated for each eye comparing GCL-IPL thickness ipsilateral vs contralateral to the patient's brain lesions. RESULTS We found that 15 of the twenty-two subjects had a relative thinning of the GCL-IPL ipsilateral to the postgeniculate lesion in both eyes (represented by a positive normalized asymmetry score in both eyes), whereas a similar pattern of right/left asymmetry was found in 4 controls (P = 0.0498). The magnitude of asymmetry was much greater in subjects compared with controls (P = 0.0004). There was no association between the degree of GCL-IPL thinning and the mean deviation on automated VF testing. A moderate correlation (R = 0.782, P = 0.004) between the magnitude of thinning and latency from onset of retrogeniculate injury was observed only after excluding patients beyond a cutoff point of 150 months. CONCLUSIONS This data provides compelling new evidence of retrograde transsynaptic degeneration causing retinal ganglion cell loss after postgeniculate visual pathway injury.

We appreciate Dr. Machado's insightful comments regarding our case series1 describing cranial neuropathies in 2 patients with COVID-19. The triad of progressive ophthalmoplegia, ataxia, and areflexia in our first case suggested Miller Fisher syndrome (MFS), despite the negative ganglioside panel, although we were surprised by the short interval of 4 days between respiratory and neurologic symptoms. However, in 2 cases of COVID-19-associated MFS, latencies were similar (5 and 3 days),2 suggesting that the postinfectious interval is shorter for COVID-19 than for other infections. In fact, in a recent case of Guillain-Barre syndrome associated with COVID-19, neurologic symptoms preceded the respiratory symptoms by 1 week.3 The authors noted that lymphopenia was present on admission, indicating the presence of COVID-19 long before systemic symptoms began. This is in line with recent observations that 10/13 nursing home residents only developed symptoms 7 days after testing positive.4 Our second case may reflect direct viral invasion because there were no signs of MFS. Transfer of the virus through the olfactory bulbs is supported by recent evidence of olfactory bulb edema5 and hyperintensity of the adjacent gyrus rectus6 in patients with COVID-19-associated anosmia, although our patient never developed anosmia. The rapid recovery of anosmia observed in most COVID-19 cases argues for olfactory epithelial dysfunction as opposed to olfactory nerve damage, and we agree with Dr. Machado that hematogenous spread of virus may also play a role in CNS disease.

Objective We report the CSF opening pressure measurements obtained before and after venous sinus stenting in 50 patients with Idiopathic Intracranial Hypertension. Methods The CSF opening pressure was measured with a spinal tap 3 months before and 3 months after treatment. All data was prospectively collected and included patient demographics, weight (kg), body-mass index (BMI), acetazolamide daily dosage (mg), procedural details, complications, venous sinus pressures (mm Hg), trans-stenotic pressure gradient (mm Hg), transverse sinus symmetry, and type of venous sinus stenosis. Results The average pre-treatment CSF-OP was 37 cm water (range: 25–77) and the average post-treatment CSF-OP pressure was 20.2 cm water (range: 10–36), with an average reduction of 16.8 cm water (p Conclusions In summary, we provided evidence that there is significant decrease in CSF-OP pressure in IIH patients 3 months after VSS, independent of acetazolamide usage or weight loss. Disclosures A. Patsalides: None. C. Oliveira: None. M. Dinkin: None.

Purpose Role of contrast-enhanced magnetic resonance venography (CE-MRV) in the follow-up of venous sinus stenting (VSS) among the idiopathic intracranial hypertension (IIH) patients. Materials and methods Prospective evaluation of VSS patients with CE-MRV, DRCV and DSA for follow-up of clinically suspected recurrent stenosis. CE-MRV was evaluated against DRCV and DSA. Results Ten patients with twelve episodes of recurrent symptoms. Sensitivity, specificity, PPV, NPV and accuracy of the CE-MRV for the detection of recurrent stenosis were: 100%, 33.33%, 81.82%, 100% and 83.3% respectively. Conclusion CE-MRV was a reliable first-line investigation for the detection of recurrent stenosis following VSS.

Purpose Magnetic resonance venography (MRV) has not been validated in pre-operative planning of the dural venous sinus stenting (VSS) among idiopathic intracranial hypertension (IIH) patients. We aim to prospectively evaluate dural venous sinus measurement in IIH patient population on two-dimensional time-of-flight (2D-TOF) MRV and Three-dimensional contrast-enhanced (3D-CE) MRV acquisitions and compare them against real-time endoluminal measurements with intravascular ultrasound (IVUS), served as the reference. Materials and methods The study has been approved by the Weill Cornell Medicine institutional review board. All patients signed written informed consent approved by IRB. Prospective evaluation of forty-five consecutive IIH patients treated with VSS at our institution were evaluated. Patients with pre-stent magnetic resonance venography (MRV) ≤ 6-months of VSS and intravascular ultrasound (IVUS) during VSS constituted the study population. Maximum diameter (in mm), Area (in cm2) and Perimeter (in cm) were measured at posterior 1/3rd of superior sagittal sinus (SSS), proximal transverse sinus (PTS), proximal sigmoid sinus (PSS) and mid sigmoid sinus (MSS) on 2D-TOF-MRV, 3D-CE-MRV and IVUS. Statistical analysis performed using box and whisker plots, Bland-Altman analysis and paired sample t-test. Results Twenty (n = 20) patients constituted our study population. The mean age was 30±11 years (7–59 years) and 18 out of 20 were female patients. Mean weight and BMI (range) were 86.3±18.3 kilograms (30.8–107.5 kgs) and 32.9±6.8 kg/M2 (16.4–48.3kg/M2) respectively. The CE-MRV significantly oversized the cerebral venous sinuses compared to TOF-MRV (Dmax: +2.0±1.35 mm, p

This is a case of a 52-year-old man with a past medical history of 2 episodes of coital thunderclap headaches as well as recent cocaine, marijuana, and pseudoephedrine use, who presented with sudden, sharp, posterior headache associated with photophobia and phonophobia. His initial magnetic resonance imaging (MRI) of the brain, magnetic resonance angiography (MRA) of the head, and magnetic resonance venography (MRV) of the head were all normal as well as a normal lumbar puncture. Given the multiple risk factors for reversible cerebral vasoconstriction syndrome (RCVS), the patient was treated for suspected RCVS, despite the normal imaging. Repeat MRI brain 3 days after hospital admission demonstrated confluent white matter T2 hyperintensities most prominent in the occipital lobes, typical of posterior reversible encephalopathy syndrome (PRES). Repeat MRA of the head 1 day after discharge and 4 days after the abnormal MRI brain showed multisegment narrowing of multiple arteries. This case demonstrates that RCVS may present with PRES on MRI brain and also exemplifies the need to treat suspected RCVS even if imaging is normal, as abnormalities in both the MRI and the MRA may be delayed.

Cryptococcus spp is a common fungal infection and frequent cause of meningitis in immunocompromised patients; however, immunocompetent patients are also at risk of infection. Visual loss often occurs via elevated intracranial hypertension but can rarely occur through direct optic nerve, chiasm, or tract invasion. We report a case of a 38-year-old woman who presented with decreased acuity in both eyes. She had generalized visual field constriction in the right eye and temporal hemianopsia in the left eye. Magnetic resonance imaging of the brain and orbits showed multiple areas of ill-defined enhancement in the optic chiasm and tracts as well as in the diaphragmatic sella, prepontine and interpeduncular cisterns, and along cranial nerves VI, VII, and VIII bilaterally. Initial cerebrospinal fluid (CSF) showed 34 white blood cells, hypoglycorrhachia, and negative cryptococcal antigen and bacterial and fungal cultures. A transphenoidal biopsy of the dura and pituitary gland was unremarkable. Empiric steroids resulted in marked improvement in visual acuity in both eyes, but while tapering steroids, she developed rapid visual loss bilaterally. Repeat CSF performed 6 weeks later demonstrated a cryptococcal antigen titer of 1:512. Retroactive staining of the pituitary biopsy was positive for mucicarmine, a component of the polysaccharide capsule of Cryptococcus spp. After induction therapy with amphotericin B and flucytosine and 1 year of fluconazole, her visual acuity was 20/20 in both eyes. In summary, Cryptococcus can affect immunocompetent patients and often presents with insidious, chronic meningitis. Visual loss is common in cryptococcal meningitis but usually results from fulminant papilledema related to elevated intracranial pressure. In rare cases, direct nerve or chiasm infiltration by the fungus results in vision loss.

Degeneration of neuron and axons following injury to cells with which they synapse is termed trans-synaptic degeneration. This phenomenon may be seen in postsynaptic neurons (anterograde) or in presynaptic neurons (retrograde). Retrograde trans-synaptic degeneration (RTSD) of the retinal ganglion cells and retinal nerve fiber layer following injury to the occipital lobe has been well documented histologically in animal studies, but its occurrence in the human retina was, for many years, felt to be limited to cases of neonatal injury during a critical period of neuronal development. Over the last decade, imaging techniques such as MRI and optical coherence tomography have allowed us to visualize and quantify RTSD and analyze its time course and relationship to degree of vision loss and age of cortical injury. A deeper understanding of RTSD in the human visual system may allow us to interfere with its occurrence, potentially allowing for greater recovery following visual cortex injury.

This article offers a pragmatic roadmap to the practicing neurologist on how to approach the patient with double vision. Strategies of history taking and examination techniques are reviewed, followed by a broad overview of the causes of diplopia organized by neuroanatomic localization. Diplopia may be the first symptom of serious vision or life-threatening neurologic disease, and its correct localization and diagnosis are therefore essential. The systems responsible for ocular movement and alignment in the vertical and horizontal plane include complex supranuclear circuitry, brainstem nuclei, cranial nerves III, IV, and VI, and their respective neuromuscular junctions and target muscles. Disruption at any point within this system or within the vestibular afferents that govern eye movement in response to head movements may therefore produce diplopia, leading to a broad differential diagnosis for the patient with diplopia. With a careful history and examination, the neurologist should be able to observe the patterns of diplopia that reveal the site of dysfunction, thus generating a shorter localization-specific list of possible etiologies. Examination of ocular motility including smooth pursuit and saccadic function, followed, if necessary, by testing designed to uncover misalignments of the eyes, including cover and Maddox rod testing, are primary components of the efferent neurologic examination. Further testing designed to detect myasthenia (eg, lid testing and fatigable upgaze) and orbital disease (eg, measuring proptosis, testing for resistance to retropulsion) may be necessary.Recent advances in the diagnosis of diplopia include the observation that vertical diplopia from skew deviation is more likely to improve with supine positioning than that caused by trochlear nerve palsies. Advances in the field of ocular myasthenia include the observation of decreased conversion to the generalized form with treatment with either steroids or thymectomy, although these conclusions need to be confirmed by prospective, randomized trials. Rarely, pure ocular myasthenia may be associated with the muscle-specific tyrosine kinase (MuSK) antibody.With proper skills, the neurologist can elucidate the localization of diplopia, even in cases of complex ocular misalignment, and generate a management plan that can address the underlying disease, and, in many cases, ameliorate or cure the diplopia.

Cavernous sinus cavernous hemangiomas in pregnancy are extremely rare lesions. The precise management of these lesions remains unknown. The authors present a case of a cavernous hemangioma in pregnancy, centered within the cavernous sinus that underwent postpartum involution without surgical intervention. A 34-year-old pregnant patient (gravida 1, para 0) presented to an otolaryngologist with persistent headache and left-sided facial pain and numbness in the V1 distribution. While being treated for sinusitis, her symptoms progressed to include a left-sided oculomotor palsy and abducens palsy. Magnetic resonance imaging without contrast revealed an expansile mass within the left cavernous sinus consistent with a cavernous hemangioma. The patient was evaluated by a neurosurgeon who recommended close follow-up and postpartum imaging without surgical intervention. Although the lesion enlarged during pregnancy, the patient was able to undergo an uncomplicated cesarean section at 37 weeks. All facial and ocular symptoms resolved by 9 months postpartum, and MRI showed a decrease in lesion size and reduced mass effect. The authors conclude that nonsurgical management may be a viable approach in patients who have an onset or exacerbation of symptoms associated with cavernous sinus cavernous hemangiomas during pregnancy because postpartum involution may negate the need for surgical intervention.

A 24-year-old woman presented with a 1-week history of fever, vomiting, and gradual onset bilateral frontal headache. CSF was notable for lymphocytosis, high protein, and low glucose and lactate dehydrogenase, but acid-fast bacilli were only noted 1 month after initial sampling. The patient subsequently developed vision loss with no light perception bilaterally despite prompt antituberculous and steroid therapy. MRI revealed optochiasmatic arachnoiditis and tuberculomas (figure). Tuberculous meningitis has a propensity for the skull base, and is associated with high morbidity and mortality, causing visual impairment in 25%–27% of patients.1,2 Prompt recognition and initiation of antituberculous and steroid therapy is crucial.

The neuro-ophthalmic examination is an invaluable tool that opens a window on diseases of the brain. In the pediatric population in particular, patients may be too young to communicate effectively, or may be completely nonverbal. As a result, children will often not be able to explain symptoms or neurologic difficulties that they are experiencing. Therefore, the objective signs found through analysis of eye movements, visual fields, and visualization of the optic nerve head may be the only evidence of pathology in the central nervous system. With a careful screening of neuro-ophthalmic function, a determination of which patients require neuroimaging and ultimately, referral to the neurosurgeon is more easily made. This chapter reviews the most salient neuro-ophthalmic symptoms and signs and the appropriate management of each.