Idiopathic Intracranial Hypertension: Glymphedema of the Brain

xloma.fota13 shared this article with you from Inoreader Idiopathic Intracranial Hypertension: Glymphedema of the Brain Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print Background: During the last decade, our understanding of cerebrospinal fluid (CSF) physiology has dramatically improved, thanks to the discoveries of both the glymphatic system and lymphatic vessels lining the dura mater in human brains. Evidence Acquisition: We detail the recent basic science findings in the field of CSF physiology and connect them with our current understanding of the pathophysiology of idiopathic intracranial hypertension (IIH). Results: Transverse sinus (TS) stenoses seem to play a major causative role in the symptoms of IIH, as a result of a decrease in the pressure gradient between the venous system and the subarachnoid space. However, the intracranial pressure can be highly variable among different patients, depending on the efficiency of the lymphatic system to resorb the CSF and on the severity of TS stenoses. It is likely that there is a subclinical form of IIH and that IIH without papilledema is probably under-diagnosed among patients with chronic migraines or isolated tinnitus. Conclusions: IIH can be summarized in the following pathological triad: restriction of the venous CSF outflow pathway—overflow of the lymphatic CSF outflow pathway—congestion of the glymphatic system. To better encompass all the stages of IIH, it is likely that the Dandy criteria need to be updated and that perhaps renaming IIH should be considered. Address correspondence to Stéphanie Lenck, MD, Department of Neuroradiology, Service de Neuroradiologie, Bâtiment Babinski, Groupe Hospitalier Pitié Salpêtrière, 47-83 Boulevard de l'Hôpital, 75013 Paris, France; Email: stephanie.lenck@orange.fr The authors report no conflicts of interest. © 2020 by North American Neuro-Ophthalmology Society View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Recurrent Orbital Inflammation Secondary to Acute Myeloblastic Leukemia

xloma.fota13 shared this article with you from Inoreader A Case of Recurrent Orbital Inflammation Secondary to Acute Myeloblastic Leukemia Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print No abstract available View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Optic Nerve Angle in Idiopathic Intracranial Hypertension

xloma.fota13 shared this article with you from Inoreader Optic Nerve Angle in Idiopathic Intracranial Hypertension Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print Background: The tortuosity of the optic nerve can be quantified radiologically by measuring the angle of optic nerve deformation (the "optic nerve angle" [ONA]). In patients with idiopathic intracranial hypertension (IIH), lowering the intracranial pressure (ICP) to a normal range by lumbar puncture leads to straightening of the optic nerve and an increase in the measured sagittal ONA on MRI. It is uncertain whether there is any correlation between ONA and cerebrospinal fluid (CSF) opening pressure or visual function. Methods: Retrospective study of patients with and without IIH who had MRI of the brain followed by lumbar puncture with CSF opening pressure within 24 hours of MRI. Before LP and within 24 hours of MRI of the brain, all patients with IIH had neuro-ophthalmologic assessment including visual acuity, Humphrey Visual Field (HVF), and fundus photography. Sagittal ONA was measured on multiplanar T2-SPACE images on a DICOM viewer. Papilledema on the fundus photographs was graded using the Frisén scale. Results: Fifty-four patients with IIH and 30 unmatched controls were included. The IIH group was 6.3 years younger (95% CI 2.4–10.3, P = 0.002), had 8.7 kg/m2 higher body mass index (4.9–12.5, P View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Atypical Ocular Coloboma in Tuberous Sclerosis-2: Report of Two Novel Cases

xloma.fota13 shared this article with you from Inoreader Atypical Ocular Coloboma in Tuberous Sclerosis-2: Report of Two Novel Cases Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print Abstract: Tuberous sclerosis complex (TSC) is an autosomal dominant multisystemic disorder caused by mutations in either TSC1 or TSC2 genes and is characterized by hamartomas in multiple organs. The most frequent and best-known ocular manifestation in TSC is the retinal hamartoma. Less frequent ocular manifestations include punched out areas of retinal depigmentation, eyelid angiofibromas, uveal colobomas, papilledema, and sector iris depigmentation. In this article, we report 2 patients carrying known pathogenic variants in the TSC2 gene who exhibited an atypical, unilateral, iris coloboma associated with localized areas of retinal dysembryogenesis. Address correspondence to Giacomo M. Bacci, MD, PhD, Pediatric Ophthalmology Unit, Children's Hospital A. Meyer-University of Florence, Viale Pieraccini 24, Florence 50139, Italy; E-mail: giacomo.bacci@meyer.it The authors report no conflicts of interest. © 2020 by North American Neuro-Ophthalmology Society View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Optical Coherence Tomography Angiography Characteristics and Predictors of Visual Outcomes in Patients with Acute and Chronic Nonarteritic Anterior Ischemic Optic Neuropathy

xloma.fota13 shared this article with you from Inoreader Optical Coherence Tomography Angiography Characteristics and Predictors of Visual Outcomes in Patients with Acute and Chronic Nonarteritic Anterior Ischemic Optic Neuropathy Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print Background/Aims: To investigate the correlation between optical coherence tomography angiography (OCTA) characteristics and visual outcomes in patients with acute and chronic nonarteritic anterior ischemic optic neuropathy (NAION). Methods: We retrospectively reviewed clinical data and OCTA images of 26 eyes of 26 patients who had been diagnosed with unilateral NAION. OCTA images were acquired from 17 eyes at the acute stage and from 21 eyes at the chronic stage of NAION. We analyzed the peripapillary vessel density (VD) and macular VD in various layers of the retina and choroid for all images. Possible correlations between the OCTA parameters and visual outcomes were investigated. Results: Among the OCTA parameters for the acute stage of NAION, the temporal peripapillary VD was found to be positively correlated with final visual acuity and visual field with statistical significance (P = 0.039 and 0.009, respectively). In the chronic stage of NAION, both peripapillary and superficial macular VDs were positively correlated with visual outcomes. The nasal perifoveal VD in the superficial capillary plexus (SCP) also had a significant correlation with final visual acuity for both acute and chronic stages (the Spearman correlation coefficient = 0.565 and 0.685, respectively). Conclusions: In patients with NAION, significant correlations were found between OCTA parameters and visual outcomes. The temporal peripapillary VD measured during the acute stage was a significant predictor of final visual outcomes. The decreased nasal perifoveal VD in the SCP was strongly associated with poor visual prognosis. Address correspondence to Hyun Taek Lim, MD, PhD, Department of Ophthalmology, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Pungnap-2-dong, Songpa-gu, Seoul, Korea 05505; E-mail: htlim@amc.seoul.kr The authors report no conflicts of interest. © 2020 by North American Neuro-Ophthalmology Society View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Prevalence of Adrenal Insufficiency and Glucocorticoid Use in Pediatric Pseudotumor Cerebri Syndrome

xloma.fota13 shared this article with you from Inoreader Prevalence of Adrenal Insufficiency and Glucocorticoid Use in Pediatric Pseudotumor Cerebri Syndrome Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print Background: The pathophysiology underlying pseudotumor cerebri syndrome (PTCS) is complex and not well understood. There are clear differences between PTCS in adults and pediatrics. Few and isolated case reports have suggested that adrenal function may be involved, yet no large cohort study has examined this relationship. Methods: We conducted a retrospective single-center study of children who presented with a diagnosis of PTCS and had cortisol testing measured between January 2010 and September 2019. We included all subjects meeting the revised PTCS diagnostic criteria after the chart review. Based on morning, random or 1-μg cosyntropin stimulated cortisol levels, adrenal functioning was classified as: (1) insufficient (peak cortisol 20 μg/dL and AM or random cortisol >13 μg/dL). Results: A total of 398 individuals were reviewed, and 64 were included for analysis. Of these, 40.6% were men, of mixed race and ethnicity with a mean age of 10.5 (SD 4.7) years. Of these, 23% and 52% had insufficient or at-risk cortisol levels. The majority of those in the insufficient (70%) or at-risk (80%) groups were exposed to topical, nasal, or inhaled glucocorticoids but not systemic. Only 60% and 12% of those with PTCS with insufficient or at-risk cortisol testing, respectively, underwent definitive testing with a stimulation test. Conclusions: Glucocorticoid use and hypocortisolism are prevalent in PTCS and need consideration as a potential underlying cause. Most children had insufficient or at-risk cortisol levels, and many did not undergo further testing/workup. Children who present with PTCS, particularly young, males should be evaluated for adrenal insufficiency and its risk factors, including nonsystemic steroids. Prospective studies are necessary to further evaluate the effect of cortisol in relation to pediatric PTCS. Address correspondence to Veeral Shah, MD, PhD, Cincinnati Children's Hospital Medical Center, Abrahamson Pediatric Eye Institute/Division of Pediatric Ophthalmology, 3333 Burnet Avenue, MLC 7003, Cincinnati, OH 45229-3039 E; E-mail: Veeral.Shah@cchmc.org The authors report no conflicts of interest. © 2020 by North American Neuro-Ophthalmology Society View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Bilateral Morning Glory Anomaly With Optic Nerve Multiple Cysts

xloma.fota13 shared this article with you from Inoreader Bilateral Morning Glory Anomaly With Optic Nerve Multiple Cysts Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print Abstract: We herein describe a pediatric case of bilateral morning glory anomaly whose retrobulbar cysts and renal disease were underdiagnosed, and finally, he was diagnosed with renal coloboma syndrome. We recommend patients with colobomatous optic nerve malformations undergo a complete workup to avoid missed diagnosis, which includes (i) general physical examination looking for heart defects, genitourinary, auricular, and midline facial defects among other abnormalities; (ii) simple nephrologic investigations such as routine urine test and/or renal ultrasound to check for renal hypoplasia; and (iii) neuroimaging check for cerebrovascular anomalies and midline cranial defects. Genetic testing can be a useful tool to guide the workup. Address correspondence to Jianhong Liang, MD, Department of Ophthalmology and Clinical Centre of Optometry, Peking University People's Hospital, 11 Xizhimen South Street, Xicheng District, Beijing, 100044, China; E-mail: drliangjianhong@126.com The authors report no conflicts of interest. © 2020 by North American Neuro-Ophthalmology Society View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Comparison of Peripapillary Vessel Density of Acute Nonarteritic Anterior Ischemic Optic Neuropathy and Other Optic Neuropathies With Disc Swelling Using Optical Coherence Tomography Angiography: A Pilot Study

xloma.fota13 shared this article with you from Inoreader Comparison of Peripapillary Vessel Density of Acute Nonarteritic Anterior Ischemic Optic Neuropathy and Other Optic Neuropathies With Disc Swelling Using Optical Coherence Tomography Angiography: A Pilot Study Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print Background: The purpose of this study is to quantitatively compare the peripapillary vessel density (PPVD), measured with optical coherence tomography angiography (OCT-A), between acute nonarteritic anterior ischemic optic neuropathy (NAION) and other causes of disc swelling ("others"). Methods: In this prospective comparative case series, patients with unilateral disc swelling due to acute NAION (n = 7) and "others" (n = 7) underwent OCT-A scanning of the optic nerve head with a swept-source OCT (Triton DRI-OCT), in addition to functional assessment. OCT-A images were analyzed using an automated customized MATLAB program. Comparison was made between total and 6 sectoral PPVD (radial peripapillary capillary [RPC] and choroid layers) of affected and fellow eyes; and between the 2 groups' affected eyes. Five NAION patients had repeated assessments at 1, 3, and 6 months. Results: Acute NAION eyes had a significantly lower total and superonasal PPVD (both layers) compared to fellow eyes. No such difference was observed in "others" group for the RPC layer. NAION eyes also had significantly lower total RPC PPVD than affected eyes in the "others" group. Over 6 months, NAION eyes had persistently lower RPC PPVD compared to fellow eyes but the reduced choroidal PPVD resolved by 1 month. Conclusion: The study demonstrated reduced superonasal and total RPC PPVD in acute NAION, which persisted over 6 months. Because there is currently no single diagnostic test for NAION, use of OCT-A images to analyze RPC PPVD may potentially help distinguish acute NAION from other causes of disc swelling by quantitatively demonstrating capillary dropout in the RPC layer. Address correspondence to Carmen K. M. Chan, MRCP, FRCSEd(Ophth), Hong Kong Eye Hospital, 147K, Argyle Street, Hong Kong, China; E-mail: kmcc2001@hotmail.com Support from the Kowloon Central Cluster research grant. Presented in part as an Oral Presentation at the Hong Kong Ophthalmological Symposium, December 15, 2018, Hong Kong, China. The authors report no conflicts of interest. © 2020 by North American Neuro-Ophthalmology Society View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Optic Nerve Drusen Anterior Displacement and Retinal Nerve Fiber Layer Thinning Over Time

xloma.fota13 shared this article with you from Inoreader Enhanced Depth Imaging Optical Coherence Tomography Technology Reveals a Significant Association Between Optic Nerve Drusen Anterior Displacement and Retinal Nerve Fiber Layer Thinning Over Time Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print Background: Optic disc drusen (ODD) are a dynamic phenomenon, and their appearance, size, and relative location may change. The purpose of this study is to evaluate and quantify the longitudinal changes of buried ODD with enhanced depth imaging (EDI) optical coherence tomography (OCT) over time. Methods: ODD were analyzed with Spectralis OCT (Heidelberg Engineering, Heidelberg, Germany) and EDI technology. The peripapillary retinal nerve fiber layer (RNFL) thickness was also measured. The size and depth of ODD were compared between the baseline and study visit (at least 2 years of follow-up), and the changes were correlated with mean RNFL thickness. The repeatability, coefficient of variation, and cutoff values for size and depth on EDI-OCT were calculated. Results: Of 21 previously identified patients with ODD, only 12 patients (21 eyes) met the most recent diagnostic criteria according to the ODD Studies Consortium recommendations for using OCT-EDI technology. The 21 eyes were reanalyzed after a mean period of 44.7 ± 13.2 months (range: 24–71 months). Overall, 132 ODD were evaluated with a mean of 6.1 ± 2.5 ODD per eye and 1.9 ± 1.1 ODD per scan. Overall, we found a significant forward movement of the drusen between visits (P = 0.01). Most drusen (67.4%) moved anteriorly, and in approximately one-third (35.6%), this displacement exceeded the cutoff value (64.28 µm). Furthermore, we found a significant correlation between ODD shallowing and RNFL thinning during the follow-up (P ≤ 0.03; R ≥ 0.370). We did not find any significant changes in size measurements (P = 0.10) over time. Conclusions: In approximately one-third of buried ODD, a significant anterior movement occurred over 2 years of follow-up, and this movement was associated with significant RNFL thinning. By contrast, no significant change was detected in the size of the buried ODD. Address correspondence to Sara Ortiz-Toquero, PhD, Hospital Universitario Ramón y Cajal, Carretera de Colmenar Viejo 9.1 Km, 28034 Madrid, Spain; E-mail: saraortizt@gmail.com The authors report no conflicts of interest. © 2020 by North American Neuro-Ophthalmology Society View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

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

xloma.fota13 shared this article with you from Inoreader Orbital Apex Syndrome Secondary to Invasive Aspergillus Infection: A Case Series and Literature Review Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print 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. Address correspondence to Cristiano Oliveira, MD, Department of Ophthalmology, Weill Cornell Medical College, 1305 York Avenue, 11th Floor, New York, NY 10021; E-mail: cro9004@med.cornell.edu The authors report no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (www.jneuro-ophthalmology.com). © 2020 by North American Neuro-Ophthalmology Society View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Neuro-Ophthalmic Manifestations of Sarcoidosis

xloma.fota13 shared this article with you from Inoreader Neuro-Ophthalmic Manifestations of Sarcoidosis Via Journal of Neuro-Ophthalmology - Published Ahead-of-Print Background: Sarcoidosis is an idiopathic, multisystem, inflammatory disease that has central nervous system involvement in 5%–15% of cases. The presentation of neurosarcoidosis is highly variable, and the MRI findings often mimic the appearance of other central nervous system diseases. Therefore, the diagnosis can be challenging. About one-third of neurosarcoidosis cases have neuro-ophthalmic manifestations and, thus, may be evaluated by a neuro-ophthalmologist early in the disease course. Methods: We performed a retrospective review of 22 cases of biopsy-proven sarcoidosis with neuro-ophthalmic manifestations, seen at the Wilmer Eye Institute from January 2013 to September 2019, in which we described the demographic information, clinical presentations, neuroimaging findings, and diagnostic evaluations. Results: Twenty-two patients were included. Fifteen patients identified as black and 7 as white. Fifteen were women, and 7 men. Mean age at sarcoidosis diagnosis was 45.9 years (range 26–66). Neuro-ophthalmic findings included optic neuropathy in 11 (50%); proptosis/orbital inflammation in 5 (23%); abducens palsy in 5 (23%); trochlear palsy, trigeminal distribution numbness, and bitemporal hemianopia in 2 each (9%); and oculomotor palsy, facial palsy, optic perineuritis, dorsal midbrain syndrome, central vestibular nystagmus, and papilledema in 1 each (5%). Eight (36%) had a pre-existing diagnosis of sarcoidosis; however, in 14 (64%), the neuro-ophthalmic presentation led to the sarcoidosis diagnosis. Patients with a pre-existing sarcoidosis diagnosis were younger than those without this diagnosis (38.5 vs 50.1 years, P = 0.035). Brain MRI was abnormal in all but 1 case (95%). In patients without a pre-existing sarcoidosis diagnosis, all 7 tested for angiotensin converting enzyme (ACE) had normal values, and 75% of the 12 who had computed tomography (CT) chest had findings suggestive of sarcoidosis (86% of black patients vs 50% of white patients). Conclusions: Patients with neurosarcoidosis may present initially with a neuro-ophthalmic manifestation, and this presentation may be more common in older patients. MRI usually is abnormal, although findings may be nonspecific. Serum testing for ACE is not helpful. Normal CT chest does not rule out underlying sarcoidosis, particularly in white patients. Address correspondence to Amanda D. Henderson, MD, Wilmer Eye Institute, Johns Hopkins University School of Medicine, 600 N. Wolfe Street, Wilmer 233, Baltimore, MD 21287; E-mail: ahende24@jhmi.edu Supported by Wilmer Biostatistics Core Grant EY01765. The authors report no conflicts of interest. © 2020 by North American Neuro-Ophthalmology Society View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.