sign in sign up
<<
Home , Papilledema, Photophobia

Pseudotumor Cerebri and AN OVERVIEW OF THIS PERPLEXING SYNDROME AND ITS HALLMARK PRESENTATION.

BY RUI WANG; ASHWINI KINI, MD; BAYAN AL OTHMAN, MD; AND ANDREW G. LEE, MD

seudotumor cerebri, also IIH and secondary intracranial hyper- specific, associated with IIH known as idiopathic intra- tension due to cerebral venous throm- are often daily and are retroocular.9 Pcranial hypertension (IIH), bosis and other causes of obstructed The headaches may resemble describes the perplexing syndrome of venous outflow. headaches with associated symptoms increased (ICP) Additionally, several systemic dis- of nausea, vomiting, and . in the absence of a space-occupying eases, drugs, and vitamin deficiencies In fact, many patients with IIH have lesion on or other or excesses have been reported to be coexisting migraine headaches, mak- etiology. Although the disease can associated with IIH. Of medications ing the diagnosis difficult.10 be observed in patients of any age, associated with IIH, growth hormones, Transient visual obscurations IIH classically presents among obese tetracyclines, and have been (TVOs) have been found to occur (body mass index >30) women of the most often reported.3-5 Other in about two-thirds of patients with childbearing age. Due to the increased systemic illnesses associated with papilledema. TVOs typically last only prevalence of in recent years, pseudotumor cerebri include Addison seconds at a time and may be bilater- a significant increase in cases of IIH disease, hypoparathyroidism, sleep al or unilateral and postural. TVOs are has been noted, and, as such, an abil- apnea, systemic erythematous, likely a manifestation of disc edema ity to recognize and understand the and polycystic ovary syndrome.6-8 leading to transient ischemia of the disease is important.1 head.11 CLINICAL MANIFESTATIONS Perhaps the most suggestive clinical PATHOGENESIS is the most common presentation of IIH is pulsatile tin- Although IIH is, by definition, idio- symptom of IIH. Although not nitus, with which patients describe pathic, increased ICP can be caused by increased (CSF) production, reduced CSF absorption, AT A GLANCE increased cerebral venous pressure,

venous sinus stenosis, increased s water content, or a combination Papilledema is the hallmark sign of idiopathic intracranial hypertension of these mechanisms.2 One theory and occurs due to raised intracranial pressure transmitted to the optic has postulated elevated intracranial nerve sheath. venous pressure as both the primary

mechanism and a final common path- s way for IIH. This theory is supported Patients with papilledema should be evaluated for an intracranial cause. by the similar clinical appearance of

34 TODAY | JANUARY/FEBRUARY 2019 DIFFERENTIAL DIAGNOSIS s

the setting of IIH may be asymmetric TABLE 1. SYMPTOMS OF IDIOPATHIC INTRACRANIAL HYPERTENSION or frankly unilateral; in one series of Symptoms Frequency 478 IIH patients, 10% had highly asym- Headache 84% metric papilledema and greater visual loss in the eye with the higher grade of Transient visual obscuration 68% papilledema.15 In addition, the absence Back 53% of papilledema does not exclude Pulsatile tinnitus 52% elevated ICP. Due to the evolving course of pap- Photopsia 48% illedema, the Frisén scale was devel- Sustained vision loss 32% oped to describe papilledema in clini- 18% cally meaningful stages.13 B-scan ultra- Reproduced with permission from Wall et al9 sonography can assist in diagnosis of IIH by measuring the diameter of the optic nerve sheath.16 The diameters in primary gaze and in 30° eccentric gaze are compared, and a 20% reduc- tion in diameter in eccentric gaze (30° test) bilaterally is considered a posi- tive test result, indicating increased subarachnoid fluid surrounding the optic nerve (Figure 1). Additionally, B-scan ultrasonography can aid in dif- ferentiating pseudopapilledema from

(Courtesy of Thomas Prager, PhD; Houston Eye Associates) papilledema by identifying Figure 1. A-scan ultrasonography can be a useful diagnostic tool. Reduction by greater than 20% on eccentric gaze drusen (Figure 2).17 (30° test), as shown above, indicates increased subarachnoid fluid level surrounding the optic nerve. DIAGNOSIS When elevation of ICP is suspected, MRI of the brain with gadolinium and magnetic resonance venography should be promptly conducted to exclude secondary causes of ICP. If no structural abnormality is identified, a should be performed, documenting the opening pressure, and Figure 2. B- and A-scan ultrasonography of . Note that the optic nerve head is highly reflective. a CSF sample should be forwarded for Calcified drusen leading to pseudopapilledema maintain high signal intensity, whereas the signal intensity is analysis and culture. IIH is diagnosed decreased in the setting of papilledema. according to the Idiopathic Intracranial Hypertension Treatment Trial modified Dandy criteria (Table 2).18 rushing water or wind. This PAPILLEDEMA symptom can be persistent or inter- Papilledema is the hallmark sign of MEDICAL MANAGEMENT mittent, and it is believed to repre- IIH. It occurs due to raised ICP trans- The goal of treatment for IIH is allevi- sent vascular pulsations transmitted mitted to the optic nerve sheath. The ation of symptoms and preservation of by the CSF under high pressure to the elevated pressure, in turn, disrupts vision. Weight loss in the range of 6% venous sinus.12 Other ocular presen- the axoplasmic flow within the nerve, to 10% has been demonstrated to lead tations include horizontal diplopia, resulting in swelling of the axons and to IIH remission.19 Although a low-sodi- often in association with sixth nerve leakage of water, protein, and other um weight reduction program is rec- palsy; transient episodes of visual loss; cellular content into the extracellular ommended for all obese patients with and disturbance, with pre- space of the optic disc, leading to optic IIH, those unable to lose weight may dilection for inferonasal defect and disc edema.14 Although typically bilat- benefit from bariatric surgery. A meta- arcuate defect (Table 1).9,13 eral and symmetric, papilledema in analysis of bariatric surgery’s effects on

JANUARY/FEBRUARY 2019 | GLAUCOMA TODAY 35 - - - - Pract - - Am J Ophthalmol. Clin Pediatr (Phila). J Neurol Neurosurg 2004;22(1):99-131. 2008;23(2):127-133. 2002;58:1551-1553. 2006;16(1):178-180. 1990;100(1):33-36. Neurol Clin. . Semin Ophthalmol. n Eur J Ophthalmol.

Laryngoscope. 1998;39(1):134-142. . 2003;142(1):35-45. . 2004;62(12):2297-2299. . 1984;16:489-494. 28 . 1991;114:155-180. . 2005;6(1):29-37. . 2011;12(5):e372-e380. Brain Neurology Ann Neurol J Lab Clin Med 2012;83(5):488-494. 2014;14:380-390. Obes Rev Patients with papilledema shouldpapilledema with Patients 14. Minckler DS, Tso MO, Zimmerman LE. A microscopic, autoradio graphic study of axoplasmic transport in the optic nerve head during ocular hypotony, increased intraocular pressure, and papilledema. 1976;82(5):741-757. 15. Wall M, White WN 2nd. Asymmetric papilledema in idiopathic intracranial hypertension: prospective interocular comparison of sensory visual function. Invest Ophthalmol Vis Sci. 16. Mollan SP, Markey KA, Benzimra JD, et al. A practical approach to diagno sis, assessment and management of idiopathic intracranial hypertension. Neurol. medical therapy may require surgery,require may therapy medical venousor diversion, CSF ONSF, as such stenting. sinus hyperten Friesner D, Rosenman R, Lobb BM, Tanne E. Idiopathic intracranial 1. sion in the USA: the role of obesity in establishing prevalence and healthcare costs. 2. Biousse V, Bruce BB, Newman NJ. Update on the pathophysiology and management of idiopathic intracranial hypertension. . 3. Vischi A, Guerriero S, Giancipoli G, Lorusso V, Sborgia G. Delayed onset of pseudotumor cerebri syndrome 7 years after starting human recombinant growth hormone treatment. 4. Friedman DI, Gordon LK, Egan RA, et al. Doxycycline and intracranial hypertension. 5. Friedman DI. Medication-induced intracranial hypertension in dermatology. Am J Clin Dermatol 6. Condulis N, Germain G, Charest N, Levy S, Carpenter TO. Pseudotumor cerebri: a presenting manifestation of Addison’s disease. 1997;36(12):711-713. 7. Dave S, Longmuir R, Shah VA, Wall M, Lee AG. Intracranial hypertension in systemic lupus erythematosus. 8. Glueck CJ, Iyengar S, Goldenberg N, Smith LS, Wang P. Idiopathic intracra nial hypertension: associations with coagulation disorders and polycystic-ovary syndrome. 9. Wall M, George D. Idiopathic intracranial hypertension. A prospective study of 50 patients. 10. Friedman DI, Rausch EA. Headache diagnoses in patients with treated idiopathic intracranial hypertension. 11. Sadun AA, Currie J, Lessell S. Transient visual obscurations with elevated optic discs. 12. Sismanis A, Butts FM, Hughes GB. Objective tinnitus in benign intracranial hypertension: an update. 13. Friedman DI. Pseudotumor cerebri. final definitive surgical procedure and procedure surgical definitive final patients in evaluation preoperative dete rapidly and IIH fulminant with further prevent to vision riorating worsening. SUMMARY cause.intracranial an for evaluated be cranial,(eg, neuroimaging Negative resonancemagnetic with MRI contrast andcontent; CSF normal venography); pressureopening on ICP elevated an diag the establish puncture lumbar on weightwith Treatment IIH. of nosis stepfirst the is and loss patientsSome IIH. of treatment the in maximumto respond not do who 17. del Saz-Saucedo P, Redondo-Gonzales O, Mateu-Mateu A, et al. Sonographic assessment of the optic nerve sheath diameter in the diagnosis of - - - - O

2

O (abnormal O (abnormal 2 26 especially those especially 25,26 A lumbar drain can be can drain lumbar A 27 Transverse dural venous sinus venous dural Transverse CSF shunts (eg, lumboperitoneal(eg, shunts CSF setting of IIH. of setting awaiting while interim the in placed sion. However, CSF shunts have ahave shunts CSF However, sion. (0.9%risk mortality inpatient higher shuntingventriculoperitoneal with shunt lumboperitoneal with 0.3% and towhich, ONSF, with compared ing) nohad has knowledge, our of best the mortality. of case reported a be to found been has stenting option management novel promising controlled randomized, a but IIH, for safety the establish to needed is study the in procedure the of efficacy and patients with IIH with predominantlywith IIH with patients symptoms, visual deterio rapid with IIH fulminant with acuityCentral fields. visual of ration andstages final until preserved be can guidea as alone used be not should surgicalof necessity the on decide to management. shunt,ventriculoperitoneal shunt, beenhave shunt) ventriculoatrial or head alleviate to primarily reported remis visual of stabilization with ache optic nervealsohas ONSF function. ineffective and safe be to shown been foroption an be could and children - - -

- - 24 In aIn 21

20 TABLE 2. IIHTT MODIFIED DANDY CRITERIA MODIFIED DANDY TABLE 2. IIHTT

22 Topiramate has demonstrat has Topiramate 23 Patients who do not respond to respond not do who Patients ACZ should be initiated at 500 mg500 at initiated be should ACZ Acetazolamide (ACZ) is the mostthe is (ACZ) Acetazolamide Echography negative for drusen and no other disc anomalies mimicking disc edema Echography negative for drusen and no other disc anomalies or stenosis, preferably using the auto- Magnetic resonance venography with lateral sinus collapse triggered elliptic centric ordered technique optic nerve sheaths with filled-out CSF spaces Partially empty sella on coronal or sagittal views and next to the on T2 weighted axial scans Pulse synchronous tinnitus Frisén grade 2 papilledema • • • 4. Awake and alert patient of 200 to 250 mm H increased intracranial pressure; opening CSF pressure 5. No other known cause of and at least one of the following: • • • 1. of increased intracranial pressure of increased intracranial 1. Signs and symptoms neurologic examination 2. No localizing findings on normal or obstruction of the ventricular system and otherwise 3. No deformity, displacement, >200 mm H for increased cerebrospinal fluid (CSF) pressure neurodiagnostic studies, except CSF spaces, and smooth- sella turcica, optic nerve sheath with filled-out neuroimaging except for empty diagnosis) sinus stenosis or collapse should lead to another walled, non–flow-related venous noncompliant, or who are intoler are who or noncompliant, from benefit may medication to ant nerve Optic intervention. surgical been has (ONSF) fenestration sheath edema nerve optic reverse to shown of recovery some to lead may and regard to visual field improvement andimprovement field visual to regard addi the has it and relief, symptom loss,weight causing of benefit tional alternative. excellent an it making are who treatment, medical maximal rence of IIH compared with no ACZno with compared IIH of rence treatment. asto increased be can and daily twice theby tolerated if day per g 4 as much patient. withACZ of that to similar efficacy ed however, treatment with ACZ led toled ACZ with treatment however, visualin improvement modest only IntracranialIdiopathic the in function Trial. Treatment Hypertension treat ACZ study, follow-up long-term reoccur decrease to found was ment IIH demonstrated 100%demonstrated IIH papilledema in reduction 90.2% a and resolution symptoms. headache randomizedonly the and effective IIH;for therapy trial–proven control

s DIFFERENTIAL DIAGNOSIS

Financial disclosure: None Clinical Professor, University of Texas MD University of Texas MD Clinical Professor, College of Medicine, Adjunct Professor, Texas A&M University of Adjunct Professor of , [email protected] Financial disclosure: None Blanton Department of Ophthalmology, Financial disclosure: None Student, Texas A&M College of Medicine, Anderson Cancer Center Anderson Cancer Bryan, Texas Iowa City, Iowa Iowa Hospitals and Clinics, Hospital, Eye Institute, Houston Methodist Houston, Texas Bryan, Texas         n n n n n n BAYAN AL OTHMAN, MD n n RUI WANG n

Professor of Ophthalmology, Neurology, and Professor of Ophthalmology, Neurology, and Clinical Professor, Department of Department of Ophthalmology, Blanton Ophthalmology, Blanton Department of Financial disclosure: None Chairman, Department Neuro-ophthalmologist and Research Clinical Member, Houston Methodist Institute, Houston Methodist Hospital, Hospital, Institute, Houston Methodist Houston, Texas , Weill Cornell Medicine, New York, New York Ophthalmology, University of Texas Medical Branch, Galveston, Texas Eye Institute, Houston Methodist Hospital, Hospital, Eye Institute, Houston Methodist Houston, Texas Eye Institute, Houston of Ophthalmology, Blanton Texas Methodist Hospital, Houston,       n n ASHWINI KINI, MD ASHWINI KINI, n n ANDREW G. LEE, MD n n - - - - . J Neurol . 9 201 2017;37(2):197-205. . 1998;29:514-517. Neuroophthalmology 2004. . 2016;361:122-127. . 2017;27(2):513-521. J Neurol Neurosurg Psychiatry Neurology. J Neuroophthalmol. J Neurol Sci . 2004;63:1737-1739. . 2014;62(10):1015-1021. Ophthalmic Surg Lasers Obes Surg Neurology JANUARY/FEBRUARY |

. 2007;116:322-327. 2016;36(1):13-19. Indian J Ophthalmol . 2003;60(9):1418-1424. 26. Spitze A, Lam P, Al-Zubidi N, et al. Controversies: optic nerve sheath fenestration versus shunt placement for the treatment of idiopathic intracranial hypertension. 27. Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension. ogy 28. Gates P, McNeill P. A possible role for temporary lumbar drainage in the management of idiopathic intracranial hypertension. 2016;40(6):277-280. sion: risk of recurrences. et al. Safety and tolerability of 23. ten Hove MW, Friedman DI, Patel AD, Hypertension Treatment Trial. acetazolamide in the Idiopathic Intracranial Neuroophthalmol. O. Treatment of idiopathic 24. Celebisoy N, Gökçay F, Sirin H, Akyürekli acetazolamide, an open-label study. intracranial hypertension: topiramate vs Acta Neurol Scand 25. Lee AG, Patrinely JR, Edmond JC. Optic nerve sheath decompression in pediatric pseudotumor cerebri. nial hypertension: a narrative review. nial hypertension: a al. Bariatric surgery or non-surgical 20. Manfield, JH, Yu KK, Efthimiou E, et A systematic review and weight loss for idiopathic intracranial hypertension? comparison of meta-analyses. Idiopathic intracranial hypertension: 21. Mollan SP, Davies B, Silver NC, et al. consensus guidelines on management. 2018;89(10):1088-1100. et al. Idiopathic intracranial hyperten 22. Kesler A, Hadayer A, Goldhammer Y, idiopathic intracranial hypertension. idiopathic intracranial hyperten Dandy criteria for idiopathic intracranial 18. Wall M. The modified is not broken. sion, no need to fix what idiopathic intracra Fletcher WA. Obesity and weight loss in 19. Subramaniam S, GLAUCOMA TODAY

46 s DIFFERENTIAL DIAGNOSIS