Guidelines of the International Headache Society for Controlled Clinical Trials in Idiopathic Intracranial Hypertension.

The quality of clinical trials is essential to advance treatment, inform regulatory decisions and meta-analysis. With the increased incidence of idiopathic intracranial hypertension and the emergence of clinical trials for novel therapies in this condition, the International Headache Society Guidelines for Controlled Clinical Trials in Idiopathic Intracranial Hypertension aims to establish guidelines for designing state-of-the-art controlled clinical trials for idiopathic intracranial hypertension.

Idiopathic Intracranial Venous Hypertension: Toward a Better Understanding of Venous Stenosis and the Role of Stenting in Idiopathic Intracranial Hypertension.

BACKGROUND: Venous sinus stenosis, typically at the junction of the transverse and sigmoid sinus, is increasingly recognized as a contributor to the pathophysiology of idiopathic intracranial hypertension (IIH), whether it be the intrinsic type that does not reverse with normalization of intracranial pressure or the extrinsic type, which does. Efforts to treat the stenosis and reduce the associated transstenotic gradient through placement of a stent at the site of stenosis have been studied over the past 2 decades, primarily through retrospective studies, with variable emphasis on formal visual testing and direct assessment of poststent opening pressure. Most studies have presented evidence for utilization of stenting as an alternative to cerebrospinal fluid shunting or optic nerve sheath fenestration in patients with IIH who harbor the stenosis and are refractory to or intolerant of intracranial pressure-lowering medications, but an assessment of the current data is needed to better understand the role of stenting for this patient population. EVIDENCE ACQUISITION: A search in PubMed was made for "IIH," "papilledema," and "venous stenting." Data pre and post stenting, including symptoms attributable to IIH, intracranial pressure, papilledema, retinal nerve fiber layer thickening on optical coherence tomography, and visual field assessment (mean deviation), were collected. Need for retreatment and complications were assessed among all studies. Studies using stenting for special circumstances, such as cerebrospinal leaks or for stenosis along anomalous vessels, were reviewed. RESULTS: In total, 49 studies (45 retrospective and 4 prospective) and 18 case reports (with 3 or less patients) were found and included in the analysis, for a total of 1,626 patients. In 250 patients in whom poststent intracranial pressure was measured, the mean value was 19.7 cm H 2 O, reduced from a mean of 33 cm H 2 O. Transient visual obscurations resolved in 79.6% of 201 patients who complained of it, pulsatile tinnitus resolved in 84.7% of 515, diplopia resolved in 93% of 86 patients, and nonspecific visual symptoms such as "blurry vision" improved in 76.2% of 537 patients. Headaches resolved in 36% and improved in a further 40.7% of 1,105 patients in whom they were documented before stenting. Of 1,116 with papilledema, 40.8% demonstrated resolution and 38.2% improvement. The mean retinal nerve fiber layer thickness improved from 170.2 µm to 89.2 µm among 402 eyes in which optical coherence tomography was used to measure it. Among 135 eyes in which formal visual fields were performed pre and post stenting, the prestent average mean deviation of -7.35 dB improved to -4.72 dB after stenting. Complications associated with stenting included in-stent stenosis or thrombosis, subdural hematoma, intracerebral hematoma, cerebral edema, stent migration, and death. A recurrence of symptoms requiring a follow-up surgical intervention occurred in 9%. CONCLUSIONS: A growing body of evidence supports the use of venous sinus stenting as a viable option for medically refractory IIH, especially when papilledema threatens visual function. Complication and failure rates seem to be similar to alternative surgical approaches, although serious neurological sequalae can rarely occur. Emerging studies evaluating stent type, including novel stents designed for use in the venous system, may help improve ease of the procedure and long-term success rates. Prospective head-to-head studies are needed to better understand the performance of stenting compared with other interventions.

An elusive case of malignant optic nerve glioma with a suprasellar mass.

A 67-year-old man with multiple comorbidities presented with 3 weeks of orbital pain, proptosis, and no light perception vision in the left eye. Examination was notable for a left orbital apex syndrome with CN III, VI palsies, an optic neuropathy, and central retinal vein occlusion. Magnetic resonance imaging of the orbits was notable for extensive enhancement, enlargement, and T2 hyperintensity of the optic nerve, with perineural sheath enhancement, and chiasmal hyperintensity. Inflammatory workup and lumbar puncture were unremarkable. No improvement was seen after a 3-day course of intravenous solumedrol. Initial optic nerve biopsy revealed necrotic nerve tissue, macrophage infiltration, increased vascularization, and peripheral gliosis. The volume of tissue was inadequate for genomic analysis. The patient was lost to follow-up but returned 5 months later with right-sided vision loss. Repeat neuroimaging showed a new suprasellar mass and progressive expansion and enhancement of both optic nerves. Biopsies of the suprasellar mass and left nerve at this time were consistent with a high-grade glial neoplasm, WHO grade IV. This is a rare case of glioblastoma involving the optic nerves and suprasellar region. In such cases, molecular profiling can improve diagnosis and may provide for targeted treatments in the future.

Multilayered macular hemorrhages as an unusual complication of transorbital neuroendoscopic surgery.

Purpose: To report a case of multilayered intraocular hemorrhage at the posterior pole as a complication of transorbital neuroendoscopic surgery. Observations: Our patient underwent an uncomplicated endoscopic transorbital resection of a left sphenoid wing meningioma. In the immediate post-operative period, the patient reported blurred vision of her left eye, and dilated fundus examination demonstrated multilayered hemorrhages at the posterior pole. No intracranial hemorrhage was identified on post-operative imaging. Due to persistent subnormal visual acuity and non-clearing hemorrhage over several weeks of follow-up, a pars plana vitrectomy with peeling of the internal limiting membrane was performed to clear the hemorrhagic component obscuring the macula. Conclusions and Importance: We report the first case of multilayered intraocular hemorrhages at the posterior pole, mimicking Terson syndrome, in the absence of intracranial hemorrhage or elevated intracranial pressure as a complication of transorbital surgery.

Ophthalmic Virtual Visit Utilization and Patient Satisfaction During the COVID-19 Pandemic.

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.

Telemedicine Evaluations in Neuro-Ophthalmology During the COVID-19 Pandemic: Patient and Physician Surveys.

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.

One Giant Step for Giant Cell Arteritis: Updates in Diagnosis and Treatment.

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.

Predicting the need for retreatment in venous sinus stenting for idiopathic intracranial hypertension.

BACKGROUND: Idiopathic 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. METHODS: We 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. RESULTS: Retreatment 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 (P<0.001). CONCLUSIONS: Higher 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.

Management of idiopathic intracranial hypertension in children utilizing venous sinus stenting.

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.

Orbital Apex Syndrome Secondary to Invasive Aspergillus Infection: A Case Series and Literature Review.

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.

Men Are from Mars, Idiopathic Intracranial Hypertension Is from Venous: The Role of Venous Sinus Stenosis and Stenting in Idiopathic Intracranial Hypertension.

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.

Paraneoplastic Syndromes in Neuro-ophthalmology.

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.

Increased frequency of cataract surgery in patients over age 50 with pituitary macroadenomas and chiasmal compression.

BACKGROUND: 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. OBJECTIVE: The interaction between these two pathologies in elderly patients is the subject of this study. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Venous sinus stenting lowers the intracranial pressure in patients with idiopathic intracranial hypertension.

AIMS: We report the cerebrospinal fluid opening pressure (CSF-OP) measurements obtained before and after venous sinus stenting (VSS) in 50 patients with idiopathic intracranial hypertension. METHODS: The 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. RESULTS: The 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 (P<0.01). The post-treatment CSF-OP was less than 25 cm H2O in 40/50 patients. The average acetazolamide daily dose decreased from 950 mg to 300 mg at the time of 3-month follow-up (P<0.01). No patient required an increase in acetazolamide dose 3 months after VSS. The average weight before treatment was 95.4 kg with an average BMI of 35.41. There was an average increase in body weight of 1.1 kg at the 3-month follow-up with an average increase in BMI of 0.35 (P=0.03). CONCLUSIONS: We 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.

Impaired drainage of vein of Labbé following venous sinus stenting for idiopathic intracranial hypertension.

PURPOSE: The 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 METHODS: Institutional 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. RESULTS: 70 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. CONCLUSION: Impaired 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 REGISTRATION: NCT01407809.

Ischemic Optic Neuropathy Secondary to Intravascular Lymphoma.

BACKGROUND: To describe a case of optic neuropathy associated with intravascular lymphoma (IVL). METHODS: Case report and review of the literature. RESULTS: 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. CONCLUSIONS: 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.