Interdisciplinary Protocol for the Management of Vision-Threatening Papilledema

This study evaluates the effectiveness of a multidisciplinary protocol for management of patients with papilledema and vision loss secondary to increased intracranial pressure.

Original Contribution Section Editors: Clare Fraser, MD Susan Mollan, MD Interdisciplinary Protocol for the Management of VisionThreatening Papilledema Taylor Brady, MD, Sravanthi Vegunta, MD, Alison V. Crum, MD, Douglas Marx, MD, Bhupendra C. K. Patel, MD, Meagan D. Seay, DO, Richard H. Schmidt, MD, PhD, Judith E. A. Warner, MD, Kathleen B. Digre, MD, Bradley J. Katz, MD, PhD Background: This study evaluates the effectiveness of a multidisciplinary protocol for management of patients with papilledema and vision loss secondary to increased intracranial pressure. Methods: Retrospective record review of all adult patients who presented with vision-threatening papilledema (VTPE) and were treated under this protocol. Patients are admitted for lumbar drain placement and diuretics and followed daily to determine if they may be managed medically or require surgery (optic nerve sheath fenestration [ONSF] and/or cerebrospinal fluid [CSF] shunting). Results: Nineteen patients were included. Twelve had body mass index in the obese range and 6 were morbidly obese. Fourteen had idiopathic intracranial hypertension. Five had secondary pseudotumor cerebri syndrome related to medication use, dural venous sinus thrombosis, hypothyroidism, end-stage renal disease, pulmonary disease, and diastolic heart failure. Three patients did not require surgery and were discharged on oral diuretics; 3 patients underwent unilateral ONSF, 9 underwent bilateral ONSF, and 4 underwent bilateral ONSF followed by ventriculoperitoneal shunt placement. The average follow-up was 10.1 months. The visual acuity improved bilaterally in 12 patients and unilaterally in 4 patients. The remaining 3 patients had worsened vision in both eyes. Fifteen patients Department of Ophthalmology and Visual Sciences (TB, SV, AVC, DM, BCKP, MDS, JEAW, KBD, BJK), John A Moran Eye Center, University of Utah, Salt Lake City, Utah; and Departments of Neurology (AVC, MDS, JEAW, KBD, BJK) and Neurosurgery (RHS), University of Utah, Salt Lake City, Utah. Supported in part by an unrestricted grant to the Department of Ophthalmology and Visual Sciences from Research to Prevent Blindness, Inc, New York, NY. 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 full text and PDF versions of this article on the journal’s Web site (www. jneuro-ophthalmology.com). T. Brady and S. Vegunta are cofirst authors. K. B. Digre and B. J. Katz are cosenior authors. Address correspondence to Bradley J. Katz, MD, PhD, Department of Ophthalmology, University of Utah, Moran Eye Center, 65 Mario Capecchi Dr., Salt Lake City, UT 84132; E-mail: Bradley.Katz@hsc. utah.edu Brady et al: J Neuro-Ophthalmol 2022; 42: 495-501 had bilateral improvement in their visual fields. Five eyes in 3 patients showed further constriction of the visual field at follow-up. Conclusions: We demonstrate how a multidisciplinary complex care protocol for treating VTPE can expedite and streamline treatment and restore vision. We found that most patients had improved symptoms and signs, including visual acuity, visual fields, and papilledema. We encourage institutions that manage VTPE to adopt similar institutional protocols. Journal of Neuro-Ophthalmology 2022;42:495–501 doi: 10.1097/WNO.0000000000001594 © 2022 by North American Neuro-Ophthalmology Society A lthough most patients with idiopathic intracranial hypertension (IIH) are managed as outpatients, a subset of these patients may present with declining vision or acute vision loss. Thambisetty et al (1) first brought attention to this subset. In their review of 14 patients with “fulminant” IIH at 2 tertiary referral centers, 7 patients remained legally blind at the most recent follow-up. Partially in response to the increased recognition of this small but acutely ill patient subset, consensus guidelines for IIH treatment were published in 2018 (2). These guidelines included a protocol for treatment of patients with IIH whose vision was threatened. Patients with vision loss in the setting of increased intracranial pressure (ICP) often present acutely and must be treated aggressively, as treatment delay is associated with poor visual prognosis (3). Management of these patients requires cooperation of several different hospital service lines, including the on-call ophthalmology and neurology services, neurology critical care, neuro-ophthalmology, and neurosurgery. For these reasons, in 2014, we instituted an interdisciplinary protocol to streamline patient identification and management at our hospital. 495 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution We describe our experience treating a series of 19 patients with vision-threatening papilledema (VTPE) who were treated under our protocol between 2014 and 2020. We expand the population of patients with VTPE to include not only patients with “fulminant IIH” but also patients with secondary pseudotumor cerebri syndrome (2ndPTCS) and better define the term “vision threatening.” METHODS The Institutional Review Board of the University of Utah approved this study and granted a waiver of consent. We reviewed records of all patients who presented to the University of Utah Health with VTPE between 2014 and 2020 and were treated under the protocol. This protocol applies to patients aged 18 and older; however, 1 patient was younger than 18; she was also treated under this protocol and is included. Most patients with VTPE have primary pseudotumor cerebri syndrome (PTCS), also known as IIH. However, some patients with VTPE have 2ndPTCS associated with an identifiable cause of increased ICP such as dural venous sinus thrombosis, severe anemia, hypothyroidism, or certain medications. These patients are included in our institutional protocol. Our protocol is shown in Figure 1. The on-call ophthalmology service or neuro-ophthalmology service determines if the patient’s papilledema is vision-threatening. This determination takes several factors into account. The 2 primary parameters we consider are loss of 3 or more lines of the best-corrected visual acuity (BCVA) in either eye and/or visual field depression on automated perimetry by at least 6 db in either eye. Additional factors include 1) evidence of declining BCVA or visual fields if the patient is followed serially in the clinic, 2) evidence of nonorganic vision loss, 3) concern about the patient’s potential for compliance with treatment recommendations and follow-up visits, and 4) atypical features associated with worse prognosis, such as Frisén papilledema Grade 4 or 5, male sex, and optic nerve drusen. Patients are directly admitted to the neuro critical care unit where the neurosurgery service measures ICP and places a lumbar drain (LD). The LD is inserted and maintained by neurosurgery according to the guidelines shown in Supplemental Digital Content 1 (http://links. lww.com/WNO/A590). The neuro-ophthalmology service follows the patients daily and makes recommendations for continued treatment. When the protocol was first instituted, patients were transported to the neuroophthalmology clinic for visual field testing. More recently, we have used a virtual reality visual field analyzer that can be used at the bedside in the neuro intensive care unit (ICU) (4). Virtual visual field analyzers are gradually being introduced because of their validity (5), convenience, portability, and cost-effectiveness (6). Depending on how patients 496 respond to treatment, they are either discharged on medical management or undergo a surgical procedure. In general, if patients show improvement in the visual acuity (0.1–0.2 logarithm of the minimum angle of resolution) and visual field testing (either 10° expansion in the Goldmann III4e isopter or 25% improvement in automated perimetry mean defect) within 48 hours after admission, they are discharged on maximum medical management. If patients do not show improvement in visual acuity and visual field testing, or if their visual acuity or visual fields deteriorate, they undergo surgery. Medical and surgical treatments are both used in the management of VTPE, often in combination. The goal is to reduce ICP, reduce optic nerve compression and congestion, and preserve or restore vision (1). Medical treatment of VTPE is primarily with diuretics, most commonly acetazolamide (7). Surgical treatment may include optic nerve sheath fenestration (ONSF), in an attempt to reduce optic nerve congestion, and/or ventriculoperitoneal shunts (VPS), to reduce ICP. We collected patient demographics, presenting symptoms, medical history, medications, and recent weight gain history. We collected presenting body mass index (BMI), BCVA (measured either at 20 feet in the clinic or with a near card in the ICU), Frisén papilledema grade, visual field testing results (automated or manual perimetry), and lumbar puncture opening pressure. We recorded how many days the LD remained in place, medical treatments, and surgical interventions. We recorded outcomes from the most recent follow-up visit, including length of follow-up, BCVA, Frisén papilledema grade, and visual field testing results. RESULTS Between 2014 and 2020, 19 patients (18 female and 1 male) were diagnosed with VTPE and treated under the protocol. Demographics of these patients are in Table 1, and their clinical courses are described in Supplemental Digital Content 2 (http://links.lww.com/WNO/A591). The mean age was 31.2 years (range: 17–57). Headache was a common presenting symptom (N = 18, 94.7%). Other presenting symptoms included bilateral blurred vision or vision loss (N = 18, 94.7%), pulsatile tinnitus (N = 13, 68.0%), transient visual obscurations (N = 8, 42.1%), and diplopia (N = 8, 42.1%). Ten patients reported significant weight gain during the prior year. Within the 3 months before presentation, patients had used doxycycline (N = 1), oral corticosteroids (N = 1), and/or hormonal contraception (N = 5). No patients had a history of lithium, retinol, or retinol derivative usage. Pertinent medical history included end-stage renal disease and kidney transplant (N = 1), hypoparathyroidism (N = 2), hypothyroidism (N = 2), essential or poorly-controlled hypertension (N = 2), type 2 diabetes mellitus (N = 2), obstructive sleep Brady et al: J Neuro-Ophthalmol 2022; 42: 495-501 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution FIG. 1. Papilledema protocol: for the evaluation of patients with vision-threatening papilledema (VTPE). If a patient has optic nerve edema due to increased ICP, an ophthalmology consult is placed. If the patient has VTPE, they are admitted to the neuro critical care unit for placement of a lumbar drain. Secondary causes of increased ICP are ruled out, and maximum medical therapy is instituted. Patients are followed daily by neuro-ophthalmology. Patients who are not responding to medical management are considered for optic nerve sheath fenestration or ventriculoperitoneal shunt. Patients who do not have VTPE and who do not have a neurologic condition needing inpatient evaluation are followed as outpatients in the neuro-ophthalmology clinic. ICP, intracranial pressure; IV, intravenous. apnea (N = 2), and pulmonary hypertension with diastolic heart failure (N = 1). The average BMI of these patients was 37.9 kg/m2 (range: 20.4–59.4 kg/m2), with 12 patients in the obese range (BMI = 30–40 kg/m2) and 6 patients in the morbidly obese range (BMI . 40 kg/m2). The presenting BCVA ranged from 20/ 20 to light perception. The average Frisén grade (38 total eyes) was 3.4 (range: 1–5). Bilateral, severe papilledema (Frisén Grade of 4 or 5) was documented in 9 patients (47.4%). At baseline examination, automated visual fields (AVF) were completed on 25 eyes. Four of these eyes were tested with a size V target. Eighteen eyes had AVF testing with a size III target at baseline and the most recent follow-up. The baseline average mean deviation for these 18 eyes was 217.40 dB (range: 22.42 to 227.29 db). Thirteen eyes had baseline Goldmann visual field (GVF) testing. Most of these eyes exhibited significant or severe visual field constriction and central scotomas that ranged from shallow to dense. Brady et al: J Neuro-Ophthalmol 2022; 42: 495-501 Fourteen patients were diagnosed with IIH and 5 with 2ndPTCS. One had 2ndPTCS caused by cerebral venous thrombosis, and a second patient had 2ndPTCS in the setting of recent doxycycline and corticosteroid use and weight gain. A third 2ndPTCS patient had disc edema in the context of uncontrolled hypothyroidism and hypoparathyroidism. A fourth patient had labile hypertension, parathyroidectomy history, a recently failed kidney transplant, and BMI of 20.4 kg/m2. The fifth patient had pulmonary hypertension, diastolic heart failure, chronic obstructive pulmonary disease (COPD), and a 20 packyear smoking history. Seventeen patients had a brain magnetic resonance imaging (MRI) that was normal other than findings associated with increased ICP. One had dural venous sinus thrombosis, and another had microvascular disease. Eighteen patients also had normal venous imaging, either magnetic resonance venography, computed tomographic venography, or MRI with spoiled gradient-recalled (SPGR) 497 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution TABLE 1. Nineteen patients with vision-threatening papilledema were admitted to the neuro intensive care unit and treated under a protocol for vision-threatening papilledema Patient Number 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 Age (Decade) Sex Diagnosis Opening Pressure (cm H2O) BMI (kg/m2) Presenting BCVA OD Presenting BCVA OS Third Sixth Third Fourth Fifth Third Third Second Third Second Fifth Third Fifth Third Third Fourth Third Sixth Fourth F F F F F F F F F F M F F F F F F F F IIH 2ndPTCS 2ndPTCS IIH IIH IIH IIH IIH IIH 2ndPTCS IIH 2ndPTCS IIH IIH IIH IIH IIH 2ndPTCS IIH 56 30 34 41 28.5 .60 30* 34 46 .60 .60 † 55 56 56 36 56 .55 .55 36.6 33.6 20.4 41.3 45.3 46.3 37.7 36.9 32.7 35.1 46.7 35.1 30.7 37.1 37.5 59.4 41.2 31.3 34.6 20/300 20/50 20/70 20/20 LP 20/100 20/30 20/40 20/25 20/40 20/70 20/60 20/20 20/300 20/20 20/40 20/70 20/30 20/30 CF 20/70 20/50 20/200 HM 20/100 20/30 20/40 20/80 20/50 20/50 20/20 20/20 HM 20/20 20/20 20/70 HM 20/25 Patient Presenting Presenting Number VF MD OD (db) VF MD OS (db) 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 GVF 24.9 GVF 224.14 GVF GVF 222.22 220.84 217.5 GVF 226.46 Size V 22.42 GVF 223.68 223.62 Size V Size V 219.00 GVF 27.81 GVF 227.29 GVF GVF 230.31 210.95 233.09 GVF 216.01 223.21 211.64 GVF 217.08 221.12 Size V GVF 215.46 Drain Days Surgery Most Recent Most Recent Most Recent Most Recent BCVA OD BCVA OS VF MD OD (db) VF MD OS (db) 7 4 9 3 6 5 4 4 4 3 3 4 4 4 5 5 2 4 4 Bilateral ONSF Bilateral ONSF None Bilateral ONSF and VPS Bilateral ONSF and VPS Bilateral ONSF and VPS Bilateral ONSF OD ONSF Bilateral ONSF Bilateral ONSF Bilateral ONSF OD ONSF None Bilateral ONSF None Bilateral ONSF Bilateral ONSF; VPS Bilateral ONSF OD ONSF 20/20 20/30 20/30 20/25 20/50 20/20 20/30 20/30 20/30 20/25 20/25 20/200 20/20 20/20 20/20 20/70 20/25 4/200 20/15 20/20 20/30 20/40 20/50 20/25 20/25 20/20 20/30 20/400 20/25 20/15 20/15 20/15 20/30 20/20 20/30 20/30 NLP 20/20 GVF 27.89 GVF 217.43 GVF GVF GVF 211.73 29.81 24.26 211.43 Size V 21.09 GVF 28.98 218.23 Size V GVF 25.13 GVF 22.2 GVF 225.99 GVF GVF GVF 29.79 GVF 27.86 28.15 211.34 25.19 GVF 25.77 211.56 Size V NLP 26.06 If a patient’s visual field mean defect is reported as “Size V” or “GVF,” a description of the visual field may be found in the Supplemental Digital Content 1—Case Histories (http://links.lww.com/WNO/A591). *Opening pressure was measured on the second hospital day. † Opening pressure was not measured. 2ndPTCS, secondary pseudotumor cerebri syndrome; BCVA, best-corrected visual acuity; BMI, body mass index; CF, counting fingers; GVF, Goldmann visual field (unable to perform automated perimetry); HM, hand motions; IIH, idiopathic intracranial hypertension; LP, light perception; MD, mean defect (automated perimetry size III target); NLP, no light perception; OD, right eye; ONSF, optic nerve sheath fenestration; OS, left eye; Size V, automated perimetry size V target; mean defect not reported; VF, visual field (automated perimetry size III target); VPS, ventriculoperitoneal shunt. sequences. CSF opening pressure was recorded in 16 of 19 patients before LD placement. One had her opening pressure measured 2 days after LD placement. Two did not have opening pressure measurements during their hospitalization. The average opening pressure of the 16 patients was 46.1 cm H2O (range: 28.5–60 cm). LDs were maintained for an average of 4.4 days (range: 2–9 days). No patient experienced a complication due to 498 LD placement. Fourteen received 1–2 g of oral acetazolamide per day. Three received oral topiramate (100 mg/ day); 2 received a combination of acetazolamide (500– 1,250 mg/day) and topiramate (50–100 mg/day). Three patients improved on medical management and were discharged on oral medications. Three underwent unilateral ONSF, and 9 underwent bilateral ONSF before discharge on oral medications. Four underwent bilateral ONSF, but Brady et al: J Neuro-Ophthalmol 2022; 42: 495-501 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution when their visual status did not improve, they underwent bifrontal VPS placement. The average follow-up time for patients was 10.1 months after discharge (range: 4 days–38 months). At the most recent follow-up, 8 patients reported complete resolution of their headaches. The remaining patients described milder or less frequent headache symptoms. Four reported persistent pulsatile tinnitus, 2 described continuing transient visual obscurations, and 4 reported persistent diplopia. BMI had decreased in 10 patients (52.6%), increased in 2 (10.5%), and remained the same in 7 (36.8%). BCVA improved bilaterally in 12 patients (63.2%) and unilaterally in 4. The remaining 3 patients had worsened vision in both eyes. One was found to have bilateral optic nerve drusen. Another only had a 7-day follow-up appointment and was lost to follow-up. The third suffered sequential nonarteritic anterior ischemic optic neuropathy in the setting of bilateral papilledema and increased ICP. She underwent sequential ONSFs and continued to have high ICP on repeat lumbar punctures. She could not tolerate acetazolamide due to chronic kidney disease. General anesthesia was deemed unsafe because of her diastolic heart failure, history of myocardial infarction, and kidney disease, so she could not undergo VPS placement. The average Frisén grade at follow-up was 1 (range: 0– 3). At the most recent follow-up, the average AVF mean deviation improved to 29.88 db (range: 21.09 to 225.99 db) among the 18 eyes that had also undergone AVF at the baseline examination. Three eyes that had undergone AVF testing at presentation underwent GVF testing at follow-up. Subjectively, these 3 eyes appeared to have improved, but these visual fields could not be directly compared. Four of 5 eyes that had undergone AVF testing with a size V stimulus at baseline and at follow-up exhibited improvement. Ten eyes had GVF testing at baseline and at follow-up. Eight of these eyes showed expansion of the visual field at follow-up compared with baseline. Overall, 5 eyes in 3 patients showed further constriction of the visual field at follow-up compared with baseline. DISCUSSION A number of case reports describe management of patients with fulminant IIH. Shaikh et al (8) described an 18-yearold woman presenting with light perception vision and bilateral Grade 5 papilledema. She was treated with LD followed by VPS, resulting in marked improvement in acuity, visual fields, and papilledema. Espino Barros Palau et al (9) reported a 34-year-old woman presenting with constricted visual fields and bilateral Grade 4–5 papilledema. She was successfully treated medically. Bhandohal and Mirza (10) reported a 33-year-old woman presenting with decreased visual acuity, Grade 3–4 papilledema, and CSF Brady et al: J Neuro-Ophthalmol 2022; 42: 495-501 opening pressure .55 cm H2O, who underwent VPS with improved vision and papilledema. Onder et al (11) described a 42-year-old woman with IIH who experienced progressive vision loss despite medical management. Her signs and symptoms improved after lumboperitoneal shunt placement. However, to the best of our knowledge, no group has previously described an institutional protocol for VTPE management, attempted to define VTPE, or reviewed outcomes of protocol implementation. To improve visual outcomes, we implemented a multidisciplinary protocol for rapid evaluation and treatment of patients with VTPE. Building on observations and recommendations from Thambisetty et al (1) and Mollan et al (2), we suggest that the protocol include not only patients with IIH but also any patients with VTPE, including patients with PTCS secondary to an identifiable cause. We have further built on the recommendations of Thambisetty et al (1) and Mollan et al (2) by attempting to better define the term “vision-threatening,” to guide other physicians called on to treat these critically ill patients. An important component of this definition is that visual acuity need not be decreased for patients to meet criteria: substantial loss of visual field, in the presence of normal or stable visual acuity, is sufficient for a patient to fall into the category of VTPE (e.g., our patient 13). Although our protocol allows for the admission of patients with nonorganic vision loss on top of organic vision loss, as well as patients who may be unreliable in follow-up, none of the patients documented in this series were admitted for either of these reasons. Use of Lumbar Drains in the Acute Management of Vision-Threatening Papilledema Our decision to include LD placement in the protocol is based on the recommendations published in “Idiopathic intracranial hypertension: consensus guidelines on management” (2). These guidelines state that patients in whom vision is threatened should undergo LD placement if surgical treatment will take place in more than 24 hours. In our protocol, the LD buys us time to determine if the patient indeed requires a surgical procedure or if they can be safely discharged on maximum medical management. Although patients with VTPE could go directly to surgery, 3 of 19 patients were able to have their LDs removed and leave the hospital without undergoing another surgical procedure. LD placement is a common and generally low-risk neurosurgical procedure performed for a number of conditions, including pituitary surgery, cranial and spinal CSF leak management, subarachnoid hemorrhage, temporary treatment of communicating hydrocephalus, and meningitis. In a retrospective analysis of 530 patients undergoing LD placement in conjunction with transsphenoidal surgery, the risk of neurologic injury was less than 1% (12). 499 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution Limitations The limitations of this retrospective study include the small number of patients in the series, inconsistent follow-up, and the fact the patients were treated at a tertiary eye care center. Because VTPE is uncommon, it is unlikely that a randomized treatment trial will ever take place. In fact, a multicenter attempt to determine if ONSF or VPS placement was the treatment of choice for VTPE was recently terminated because of slow enrollment (13). For this reason, small series such as this one may be the best guidance for treatment of these patients. Implementation of a Multidisciplinary Protocol When implementing a multidisciplinary complex care protocol at any institution, the success or failure of the protocol relies heavily on obtaining agreement between all service lines. This agreement ensures that all practitioners in all 3 departments will treat these patients in a consistent manner. The 3 departments must also agree that ophthalmology or neuro-ophthalmology will determine which patients meet the criteria for admission under the protocol and determine which patients need surgical intervention to preserve vision. Once it is determined that a patient with VTPE needs surgery, the decision to recommend ONSF or a VPS depends on what expertise is available at the institution. In general, when vision is threatened, we prefer to try ONSF first and only go to a shunt if ONSF fails to reverse the vision loss. However, it is currently unknown which procedure has the best long-term outcomes regarding increased ICP and vision symptoms. If a patient presents with VTPE and the required expertise in the management of these patients is not available, consideration should be given to transferring these patients to a tertiary center more familiar with managing VTPE. Performing visual fields on patients in the neuro ICU can be impractical. Most of the patients in this series had to be transported to the neuro-ophthalmology clinic, accompanied by a neurology intensive care nurse, with LDs in place, for visual field testing. Recently, we have adopted a virtual reality visual field testing technology that can be brought to patients. We have also acquired a nonmydriatic fundus camera to document and follow optic nerve appearance. Although we have not encountered safety issues transporting patients to our clinics, these portable technologies should make the evaluation of these patients safer and less impractical. CONCLUSIONS In summary, we present a definition and multidisciplinary complex care protocol for management of VTPE. We reviewed our outcomes for patients treated under this protocol and found that visual outcomes are significantly improved compared with historical reports. We hope other 500 institutions treating VTPE will consider adopting a similar protocol for patient management. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: T. Brady, S. Vegunta, A. V. Crum, D. Marx, B. C. K. Patel, M. D. Seay, R. H. Schmidt, J. E. A. Warner, K. B. Digre, and B. J. Katz; b. Acquisition of data: T. Brady, S. Vegunta, K. B. Digre, and B. J. Katz; c. Analysis and interpretation of data: T. Brady, S. Vegunta, A. V. Crum, D. Marx, B. C. K. Patel, M. D. Seay, R. H. Schmidt, J. E. A. Warner, K. B. Digre, and B. J. Katz. Category 2: a. Drafting the manuscript: T. Brady, S. Vegunta, A. V. Crum, J. E. A. Warner, K. B. Digre, and B. J. Katz; b. Revising the manuscript for intellectual content: T. Brady, S. Vegunta, A. V. Crum, D. Marx, B. C. K. Patel, M. D. Seay, R. H. Schmidt, J. E. A. Warner, K. B. Digre, and B. J. Katz. Category 3: a. Final approval of the completed manuscript: T. Brady, S. Vegunta, A. V. Crum, D. Marx, B. C. K. Patel, M. D. Seay, R. H. Schmidt, J. E. A. Warner, K. B. Digre, and B. J. Katz. ACKNOWLEDGMENTS The authors thank Ms. Susan Schulman, Links to Clinical Research, Salt Lake City, UT, for professional editing of the final article, Samuel Passi, MD for developing the original protocol, and their colleagues in the University’s Departments of Neurology and Neurosurgery for their assistance in establishing the protocol and for their care of the patients described in this article. REFERENCES 1. Thambisetty M, Lavin PJ, Newman NJ, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology. 2007;68:229–232. 2. Mollan SP, Davies B, Silver NC, Shaw S, Mallucci CL, Wakerley BR, Krishnan A, Chavda SV, Ramalingam S, Edwards J, Hemmings K, Williamson M, Burdon MA, HassanSmith G, Digre K, Liu GT, Jensen RH, Sinclair AJ. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018;89:1088–1100. 3. KesKın AO, _Idıman F, Kaya D, Bircan B. 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