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Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

by Michael Wall, md
Idiopathic intracranial hypertension (pseudotumor cerebri) is a
disorder of elevated intracranial pressure of unknown cause. Patients present with daily headache, pulse synchronous tinnitus, transient visual obscurations, papilledema with its associated visual loss, and diplopia from VI nerve paresis. Many disease associations have been alleged but few other than obesity, hypervitaminosis A and related compounds, steroid withdrawal and female gender have been proven. While CSF absorption occurs through both the arachnoid granulations and extracranial lymphatics, it appears that in IIH, outflow resistance is increased; intracranial pressure must then increase for CSF to be absorbed. The mainstays of medical treatment are use of a reduced-sodium weight-reduction program and acetazolamide. If patients fail medical therapy, surgical procedures, most commonly optic nerve sheath fenestration and CSF shunting procedures, are employed. The main morbidity of IIH is from visual loss. This is present in most patients and can usually be reversed if recognized early in the patients course and treated.

Michael Wall, MD University of Iowa College of Medicine Veterans Administration Hospital, Iowa City Iowa City, IA 52242 319.353.6942 Fax: 319.356.4505 michael-wall@uiowa.edu

Introduction
Idiopathic intracranial hypertension (IIH), also called pseudotumor cerebri, is a disorder of raised intracranial pressure occurring in women of the childbearing years of unknown cause. It is characterized by increased intracranial pressure with its attendant signs and symptoms in an alert and oriented patient, but without localizing neurologic findings. MRI shows only smooth-walled venous stenoses, empty sella syndrome and signs related to the unfolded optic nerve sheath such as globe flattening. Neurodiagnostic studies are otherwise normal except for increased cerebrospinal fluid pressure. In addition, no secondary cause of intracranial hypertension is apparent. IIH can have a self-limited or a life-long chronic course. Although obesity and weight gain are clearly established as related factors, as is female gender, the cause of the disorder remains unknown. The major morbidity of the disease is visual loss related to papilledema with blindness as a potential outcome of treatment failure. Headache is ubiquitous but various headache syndromes can be present.

Epidemiology
The annual incidence of IIH is 0.9/100,000 persons and 3.5/100,000 in females 15 to 44 years of age.1 In obese women aged 20 to 44 years who were 20% or more over ideal weight, the incidence is 19 per 100,000.1 With the current obesity epidemic, these figures likely underestimate incidence.2 Weight gain in the year prior to diagnosis is associated with disease onset in both obese and non-obese patients.3 More than 90% of IIH patients are obese and over 90% are women of childbearing age. This female preponderance and association of obesity only holds for post-pubertal patients.4 The mean age at the time of diagnosis is about 30 years.1

Etiology and Pathogenesis


Studies of conditions associated with IIH are mostly uncontrolled and retrospective. This has led to erroneous conclusions because investigators have reported chance

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and spurious associations with common medical conditions and medications. Also, there are many case reports of associations with intracranial hypertension where the cases do not meet the modified Dandy criteria of IIH. For example, pregnancy, irregular menses and oral contraceptive use are reported associations that have been shown to be due to chance alone.57 In case-control studies, no significant association is found between IIH and multivitamin, oral contraceptive or antibiotic use.6,7 A case-control study has found strong associations between IIH and obesity and weight gain during the 12 months before diagnosis.7 Any hypothesis of pathogenesis of IIH should explain the following observations of patients with the disorder: (1) high rate of occurrence in women of childbearing years, (2) association of obesity, (3) decreased conductance to CSF outflow, and (4) normal ventricular size and no hydrocephalus.

Frequency in Percent of Symptoms in the IIH Patient and Control Groups


100 IIH Patients Controls 80

FIGUR E 1

Frequency (%)

60

40

20

Headache

TVO

Tinnitus

Photopsia

Eye Pain

Changes in cerebral hemodynamics, that is, TVO stands for transient visual obscurations. increased cerebral blood volume and decreased cerebral blood flow, have been reported. However, others have found no significant changes in these factors. The as pulsatile. They are different from previous headaches, may most popular hypothesis is that IIH is a syndrome of reduced awaken the patient and usually last hours. Nausea is common CSF absorption. Decreased conductance to CSF outflow may and vomiting less so. The headache is usually reported as the be due to dysfunction of the absorptive mechanism of the worst head pain experienced. In addition, other headache arachnoid granulations or the extracranial lymphatics.8 This syndromes frequently coexist such as rebound headache from latter mechanism of an alternative route of drainage through analgesic overuse.12 extracranial lymphatics, proposed by Miles Johnston, may be Transient Visual Obscurations Visual obscurations are episodes an important factor in the mechanism of IIH, as this route may of transient blurred vision that usually last less than 30 seconds account for a substantial percentage of CSF absorption. and are followed by visual recovery to baseline. Visual obscuraSo, regardless of the outflow mechanism, if outflow resistance is tions occur in about of IIH patients.7 The symptom may be increased, then intracranial pressure must increase for CSF to monocular or binocular. The cause of these episodes is thought be absorbed. Although interstitial and intracellular edema have to be transient ischemia of the optic nerve head due to increased been reported in brain biopsy specimens,9 study with current tissue pressure. Visual obscurations do not appear to be associmethods of analysis has concluded that the histological features ated with poor visual outcome. of the brain parenchyma are normal and the findings from Pulse-synchronous Tinnitus Pulsatile intracranial noises or pulse previous reports are artifactual.10 synchronous tinnitus is common (60%) in IIH.7 The sound is often unilateral with neither side predominating. In patients Clinical Features with intracranial hypertension, jugular compression ipsilateral The symptoms of IIH patients are headache (94%), transient to the sound abolishes it.13 The sound is thought to be due to visual obscurations (68%), pulse synchronous tinnitus (58%), transmission of intensified vascular pulsations by means of CSF photopsia (54%), and retrobulbar pain (44%). Diplopia (38%) under high pressure and turbulence through smooth-walled and visual loss (30%) are less common accompaniments of IIH; venous stenoses related to transverse sinus compression by high however, some of these symptoms are common in controls CSF pressure.14 The major signs of IIH are papilledema and (Figure 1). sixth nerve paresis. The presence of headache is nearly ubiquitous in patients with IIH and is the usual presenting symptom. The headache profile of the IIH patient11 is that of severe daily headaches described
Ophthalmoscopic Examination Papilledema is the cardinal sign of IIH. Optic disc edema either directly or indirectly is the cause of visual loss with IIH. The more high grade the papilledema,

Symptom

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the worse the visual loss.15 But, in the individual patient, the severity of visual loss cannot accurately be predicted from the severity of the papilledema. A partial explanation for this is that with axonal death from compression of the optic nerve, the amount of papilledema decreases.
Ocular Motility Disturbances Horizontal diplopia occurs in about

Modified Dandy Criteria for IIH17


1.Signs and symptoms of increased intracranial pressure 2. Absence of localizing findings on neurologic examination 3. Absence of deformity, displacement or obstruction of the ventricular system and otherwise normal neurodiagnostic studies, except for increased cerebrospinal fluid pressure (greater than 250 mm of water in obese patients) 21 4. Awake and alert patient 5. No other cause of increased intracranial pressure present

TA BL E 1

of IIH patients and sixth nerve palsies are found in 1020%. Motility disturbances other than sixth nerve palsies have been reported. Some of these reflect erroneous conclusions from the small vertical ocular motor imbalance that is known to accompany sixth nerve palsies. The diagnosis of IIH should be viewed with suspicion in patients with ocular motility disturbances other than sixth nerve palsies.

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Sensory Visual Function Visual acuity usually is normal in patients with papilledema except when the condition is longstanding and severe or a serous retinal detachment occurs from the optic disc edema. Snellen acuity testing is insensitive to the amount of visual loss found by perimetry and to worsening of papilledema grade.16 Perimetry Visual field loss is ubiquitous in IIH. In a prospective

change.16 Other groups at risk for severe visual loss are the black male, those with glaucoma and patients being rapidly tapered off corticosteroids. The course of IIH is often chronic with recurrences especially during periods of weight gain.

Diagnostic Criteria
The accepted criteria initially proposed by Walter Dandy have been modified.17 Patients who fulfill these criteria are diagnosed as having the idiopathic form of the disease. These criteria are found in Table 1. Patients with findings on examination other than papilledema, sixth nerve and rarely seventh nerve paresis should be suspected of having a diagnosis other than IIH. Laboratory evaluation is normal except for increased intracranial pressure. There are several issues surrounding the criteria of the measurement and limits of the opening pressure. Whether the patient is supine, prone or sitting, one must be sure that the reference level for measurement is the level of the left atrium. Next, spuriously high values can occur with Valsalva18 and the hypoventilation associated with sedation. The latter is particularly a recurring issue in the pediatric population. Artifactually low values can occur with hyperventilation in the anxious patient from reduction in carbon dioxide levels; of course, the patient undergoing multiple needle punctures may have falsely low results. The final issue here is that CSF pressure fluctuates throughout the day and at times is normal, so a single normal CSF measurement does not exclude IIH as the diagnosis. The normal limits for CSF opening pressure remain controversial. Normal limits are less than 200 mm water in non-obese subjects but in obese patients there are conflicting studies. Whiteley and coworkers19 prospectively recorded CSF opening pressure in 242 adults and measured patients weights and

study of patients with IIH, visual loss in at least one eye (other than enlargement of the physiological blind spot) was found in 96% of patients with Goldmann perimetry using a diseasespecific strategy and in 92% with automated perimetry. About of this visual loss is mild and unlikely to be noticed by the patient but serves as a marker with which to gauge therapy.16

The visual field defects found in IIH are the same types as those reported to occur in papilledema due to other causes. The most common defects are enlargement of the physiologic blind spot and loss of inferonasal portions of the visual field along with constriction of isopters. Central defects are distinctly uncommon and warrant a search for another diagnosis unless there is a large serous retinal detachment from high-grade optic disc edema spreading toward the macula. The loss of visual field may be progressive and severe, leading to blindness. The temporal profile of visual loss is usually gradual; however, acute severe visual loss can occur. Blind spot enlargement is ubiquitous in IIH. Since refraction often eliminates this defect, blind spot enlargement should not be considered significant visual loss unless it encroaches on fixation. Also, since blind spot size is so dependent on refraction, it should not be used to follow the course of therapy. With treatment there is significant perimetric improvement in about 50% of patients.16 A study that evaluated a subgroup of patients with worsening of their vision showed recent weight gain was the only factor significantly associated with this
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heights. The 95% reference interval for the CSF opening pressure was 10 to 25 cm CSF. However, neither neck nor hip flexion was altered in their protocol, suggesting some of their subjects may have been prone to Valsalva and falsely elevated pressures. In addition, the inclusion and exclusion criteria of the study may not have been appropriate.20 Corbett and colleagues21 found a cutoff of 250 but their numbers were small. Bono and coworkers22 measured CSF pressure in obese and non-obese subjects with neck and legs extended and no patients had pressures over 200 mm water. In summary, the cutoff value for increased intracranial pressure remains unclear. Values between 200 and 250 may be considered borderline with values over 250 mm water definitely elevated.

treating symptoms directly such as headache. Unfortunately, there have not been any controlled clinical treatment trials for idiopathic intracranial hypertension. years. Newborg in 1974 reported remission of papilledema in all nine patients placed on a low-calorie adaptation of Kempners rice diet. The patients intake was 4001,000 calories per day by fruits, rice, vegetables and occasionally 12 oz. of meat. Fluids were limited to 7501,250 ml/day and sodium to less than 100 mg/day. All patients had reversal of their papilledema. Unfortunately, there was no mention of the patients visual testing.27 Others have also documented successful outcomes associated with weight loss,2830 and it appears that only modest degrees of weight loss in the range of 510% total body weight are needed for reversal of symptoms and signs. Gastric weight-reduction surgery has been used with some success in 24 morbidly obese women with IIH.31 Symptoms resolved in all but one patient within four months of the procedure. Two patients regained weight associated with return of their symptoms. There were many significant but treatable complications of this surgery. Since marked recent weight gain is a predictor of visual deterioration16 and papilledema can resolve with modest weight loss as the only treatment, we strongly encourage our patients to pursue a supervised weight-loss program. Institution of a low-salt diet and mild fluid restriction appear to be beneficial for many IIH patients. This may be especially true in patients who lose only 510 percent of their total body mass yet have resolution of their papilledema. It is not yet clear whether improvement occurs because of weight loss per se or other changes in diet such as fluid or sodium restriction or decrease in the intake of a molecule such as vitamin A.
Lumbar Puncture Use of repeated lumbar punctures is controversial. Lumbar puncture has only a short-lived effect on CSF pressure32 with a return of pressure to pre-tap level after only 82 minutes. Lumbar puncture measures CSF pressure at only one point in time. Since CSF pressure fluctuates, this information has only limited clinical use for modifying treatment plans. However, since transverse sinus collapse (smooth-walled venous stenoses) can resolve immediately with lowering pressure,33 CSF circulatory dynamics may be restored with this procedure and may give temporary relief until the sinus recollapses. Corticosteroids Steroids are still occasionally used to treat IIH Weight Loss Weight loss has been used to treat IIH for many

Differential Diagnosis
The differential diagnosis of papilledema is broad. If we restrict ourselves to the differential of patients who meet the modified Dandy criteria, the list is limited. There is reasonable evidence that the following disease associations with intracranial hyper tension exist: nalidixic acid, nitrofurantoin, indomethacin or ketoprofen in Bartters syndrome, vitamin A intoxication, retinoic acid,23 thyroid replacement therapy in hypothyroid children, lithium and anabolic steroids. Although tetracycline and minocycline use may well be related,24,25 the common use of these medications in the age group, coupled with incomplete proof of the association, suggests further study is needed. Cryptic arteriovenous malformations or dural fistulae with high flow may overload venous return and result in elevation of intracranial pressure. Although steroid withdrawal and Addisons disease are clearly associated with IIH, as is hypoparathyroidism, links to other endocrine abnormalities remain unproven. A more in-depth discussion of differential diagnosis is found elsewhere.26

Treatment
Once intracranial hypertension is discovered, one should first eliminate presumed causal factors such as excessive vitamin A or tetracyclines and begin a low-sodium weight-reduction diet. Therapy aimed at reversing and preventing visual loss should then be instituted. Then symptomatic headache treatment can be instituted if this symptom persists in the face of intracranial pressurelowering agents and procedures. Many treatments have been used for idiopathic intracranial hypertension with varying success. All reports to date are anecdotal. Visual loss is the only serious complication and it may occur anywhere from the time of first appearance to many years later. We therefore recommend tailoring the treatment primarily to the presence and progression of visual loss.

Medical Therapy
Treatment of raised intracranial pressure can be both medical and surgical. It is aimed at lowering of intracranial pressure and

but their mechanism of action remains unclear. The side effects of weight gain, striae, and acne are especially unfortunate for these obese patients. Although patients treated with steroids often respond well, there usually is recurrence of papilledema with rapid tapering of the dose. This may be accompanied by marked deterioration of visual function. A prolonged tapering may prevent return of symptoms and signs in some patients. Use of long-term steroids to treat IIH has largely been abandoned.
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(Diamox) decreases CSF flow but not until over 99.5% of choroid plexus carbonic anhydrase was inhibited. Gcer and Viernstein35 used intracranial pressure monitoring before and after treatment in four IIH patients. They monitored acetazolamide treatment in two of the patients and showed gradual CSF pressure reduction in both. They reported the dose in only one of the patients (four grams of acetazolamide were needed per day). Apparent efficacy of acetazolamide has also been shown by others.36,37 We start with to 1 gram a day of acetazolamide in divided doses and gradually increase the dose until either symptoms and signs regress, side effects become intolerable or a dose of 34 grams per day is reached. Most patients respond in the 12 grams per day range. The mechanism of action of acetazolamide is likely multifactorial. It has been found to reduce CSF production; also, it changes the taste of foods and sometimes causes anorexia, aiding in weight loss. Patients nearly always experience tingling in the fingers, toes, and perioral region, and less commonly have malaise. Renal stones occur in a few percent of patients. Metabolic acidosis, evidenced by lowered serum bicarbonate, is a good measure of compliance. A rare but serious side effect is aplastic anemia. It occurs in one in 15,000 patient-years of treatment with acetazolamide and usually occurs in the first six months of therapy. Aplastic anemia from acetazolamide has been reported most often in the elderly and is probably less common in younger idiopathic intracranial hypertension patients. Since this side effect is so rare and finding the case and stopping the medication does not necessarily cure the patient, repeated blood testing is not usually performed.38
Topiramate (Topamax) has also been used to treat IIH since

Acetazolamide McCarthy and Reed 34 showed acetazolamide

Surgery
The surgical forms of therapy now used are various shunting and decompression procedures: lumbar subarachnoid-peritoneal shunt and optic nerve sheath fenestration. Subtemporal or Suboccipital Decompression Subtemporal or suboccipital decompression was used from the 1940s to the 1960s for patients with visual loss from IIH. These procedures have largely been abandoned because of complications, which include seizures, otorrhea, subdural hematoma, and postoperative visual deterioration. However, long-term success has been reported.42 Optic Nerve Sheath Fenestration Optic nerve sheath fenestration consists of either creating a window or making a series of slits in the optic nerve sheath just behind the globe. This treatment is preferred for the patient with progressive visual loss with mild or easily controlled headaches, although over 50% of patients with the procedure gain adequate headache control (especially if the headache is frontal). Since improvement in papilledema may occur in the unoperated eye and fistula formation has been demonstrated, the mechanism of action may be local decompression of the subarachnoid space. Occasional failure of the fellow eye to improve and the asymmetry of papilledema may be explained by the resistance to CSF flow produced by the trabeculations of the subarachnoid space. The mechanism of action may also be closure of the subarachnoid space in the retrolaminar optic nerve by scarring. It is likely that both mechanisms contribute to protection of the optic nerve head. Many large case series attest to the efficacy of this technique.4349 In these series, postoperative visual acuity or perimetry results were as good as or better than preoperative studies in about 90%. However, occasionally patients lose vision in the peri operative period.46 CSF Shunting Procedures Various shunting procedures have been employed for the treatment of idiopathic intracranial hypertension such as lumbar subarachnoid-peritoneal shunts, ventriculoatrial, ventriculojugular and ventriculoperitoneal shunts. In general, the indication for a CSF diversion procedure is failed medical therapy or intractable headache. Its use appears to be increasing.50 Eggenberger and coworkers51 studied lumboperitoneal shunt retrospectively in 27 IIH patients. While initially successful, 56% required a shunt revision. Rosenberg and colleagues52 reported on 37 IIH patients who underwent 73 lumboperitoneal

it has carbonic anhydrase activity and weight loss commonly occurs. In studies to date, it appears comparable to acetazolamide.37,39

has been well documented that furosemide (Lasix) can lower intracranial pressure.41 It appears to work by both diuresis and reducing sodium transport into the brain. We initiate furosemide at a dose of 20 mg p.o. b.i.d. and gradually increase the dose, if necessary, to a maximum of 40 mg p.o. t.i.d. Potassium supplementation is given as needed.

Furosemide Furosemide has also been used to treat IIH.40 It

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shunts and nine ventricular shunts with modest success (38% of patients successfully treated after one shunting procedure). Shunt failure occurring in 55% and low-pressure headaches in 21% were the most common causes for reoperation. The vision of most patients improved or stabilized from the procedure, but three who had initially improved later lost vision and six had a decrease in vision postoperatively. Serious complications occurred in 3.6%. Other series are similar53,54 with the conclusion that there is initial success but at least half need reoperations. Also, when the procedure is done primarily for headache relief, long-term success is only about 50%.55 In summary, shunting procedures are successful in selected patients. Shunt occlusion that occurs in about half can be accompanied by severe visual loss, limiting the effectiveness of this procedure. Gastric Exclusion Surgery As discussed above, for the morbidly obese patient, successful treatment has been reported using gastric exclusion procedures.31 This procedure may be especially useful in treating other conditions comorbid with obesity such as arterial hypertension, diabetes mellitus, and sleep apnea. Complications include major wound infection and stenosis at the gastrojejunal anastomosis. Venous Sinus Stenting It has been suggested that the cause of IIH is collapse of the proximal transverse sinus. However, King and colleagues have shown lowering of CSF pressure from a cervical puncture abolishes the pressure gradient.56 Since this collapse of the transverse sinus, which is ubiquitous in IIH14 (and occurs in about 7% of normals), may obstruct venous return and hence CSF outflow, stents have been placed to keep this portion of the transverse sinus open. This procedure has been reviewed by Friedman with the conclusion that it can have major morbidity (subdural hematoma), remains unproven and needs further study.57

loss occurs, optic nerve sheath fenestration is used. If intractable headache dominates the course, a CSF shunting procedure is recommended. Treatment decisions may be complicated and are further discussed elsewhere.26

Treatment of Headache
Headache in IIH patients may improve after a lumbar puncture, but may remain as a management problem even after medications have been given to reduce edema. We have had success treating these patients with standard prophylactic vascular headache remedies. However, we try to avoid medications that cause hypotension, such as beta blockers or calcium channel blockers, because they may cause reduced perfusion of the optic nerve head. Tricyclic antidepressants can be problematic because of their side effect of weight gain. We use tricyclics in very low doses such as amitriptyline 1025 mg at bedtime. Nonsteroidal anti-inflammatory agents are used as an adjunct but their use is limited to two days per week to prevent the development of rebound headaches. Topiramate may be useful both for its migraine prophylaxis, side effect of weight loss and for carbonic anhydrase inhibition. Uncommonly, lumbar-peritoneal shunting or another CSF diversion procedure is needed for persistent headache, but it can produce the hindbrain herniation headache in return. Patients with IIH also have other headache syndromes. Especially in patients with a migraine history, analgesic rebound or caffeine rebound headaches may coexist. These patients may require IV dihydroergotamine to treat this troublesome headache syndrome.

Conclusion
Idiopathic intracranial hypertension is characterized by elevated CSF pressure of unknown cause. For unclear reasons, it is a disease of women in the childbearing years. CSF outflow resistance appears to increase, so intracranial pressure must rise for CSF to be absorbed. Whether the increased resistance is due to outflow block at the level of the arachnoid granulations or extracranial lymphatics is unclear. Many diseases and conditions have been reported to be associated with IIH but few are confirmed with case control studies, and many of these associations are chance associations related to the predominance of IIH in young women. IIH can result in blindness if inadequately treated. Fortunately, there appear to be effective treatment strategies available. Headaches usually becomes manageable, papilledema regresses, and vision improves in most patients. Unfortunately, treatment is as much art as science since there are no controlled clinical trials.

Treatment Overview
Medical and surgical treatment of patients with idiopathic intracranial hypertension is often challenging, requiring integration of the history, examination and clinical course. Many factors are involved and each is weighted in creating individualized therapy. The most important factor is usually the amount and progression of visual loss. Next in importance is the severity of the patients symptoms with regard to how much they are interfering with the patients activities of daily living. Headache is the most problematic symptom but pulse synchronous tinnitus, and diplopia, can be challenging to treat. Also factored in is the degree and change in papilledema gradi. Mild cases are treated with weight loss and sodium restriction, often with the addition of acetazolamide. If progressive visual

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References
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Continuing Nursing Education (CNE) Article

34. McCarthy K. D., & Reed D. J. The effect of acetazolamide and furosemide on CSF production and choroid plexus carbonic anhydrase activity. J Pharmacol Exp Ther 1974; 189:194201. 35. Gcer G., & Vierenstein L. Long-term intracranial pressure recording in management of pseudotumor cerebri. J Neurosurg 1978; 49:256263. 36. Tomsak R. L., Niffenegger A. S., & Remler B. F. Treatment of pseudo tumor cerebri with Diamox (acetazolamide). J Clin Neuro-Ophthalmol 1988; 8:9398. 37. Celebisoy N., Gokcay F., Sirin H., & Akyurekli O. Treatment of idiopathic intracranial hypertension: Topiramate vs. acetazolamide, an open-label study. Acta Neurol Scand 2007; 116(5):322327. A small series suggesting similar efficacy of acetazolamide and topiramate for medical therapy of IIH. 38. Zimran A., & Beutler E. Can the risk of acetazolamide-induced aplastic anemia be decreased by periodic monitoring of blood cell counts? Am J Ophthalmol 1987; 104(6):654658. 39. Shah V. A., Fung S., Shahbaz R., Taktakishvili O., Wall M., & Lee A. G. Idiopathic intracranial hypertension. Ophthalmology 2007; 114(3):617. 40. Corbett J. J. The 1982 Silversides lecture. Problems in the diagnosis and treatment of pseudotumor cerebri. Can J Neurol Sci 1983; 10:221229. 41. Pollay M., Fullenwider C., Roberts P. A., & Stevens F. A. Effect of manni tol and furosemide on blood-brain osmotic gradient and intracranial pressure. J Neurosurg 1983; 59(6):945950. 42. Kessler L. A., Novelli P. M., & Reigel D. H. Surgical treatment of benign intracranial hypertensionsubtemporal decompression revisited. Surg Neurol 1998; 50(1):7376. 43. Goh K. Y., Schatz N. J., & Glaser J. S. Optic nerve sheath fenestration for pseudotumor cerebri. J Neuro-Ophthalmol 1997; 17(2):8691. 44. Acheson J. F., Green W. T., & Sanders M. D. Optic nerve sheath decompression for the treatment of visual failure in chronic raised intracranial pressure. J Neurol Neurosurg Psychiatr 1994; 57(11):14261429. 45. Sergott R. C., Savino P. J., & Bosley T. M. Modified optic nerve sheath decompression provides long-term visual improvement for pseudotumor cerebri. Arch Ophthalmol 1988; 106:13911397. 46. Corbett J. J., Nerad J. A., Tse D., & Anderson R. L. Optic nerve sheath fenestration for pseudotumor cerebri: The lateral orbitotomy approach. Arch Ophthalmol 1988; 106:13911397.

47. Plotnik J. L., & Kosmorsky G. S. Operative complications of optic nerve sheath decompression. Ophthalmology 1993; 100(5):683690. 48. Brourman N. D., Spoor T. C., & Ramocki J. M. Optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol 1988; 106:13781383. 49. Chandrasekaran S., McCluskey P., Minassian D., & Assaad N. Visual outcomes for optic nerve sheath fenestration in pseudotumour cerebri and related conditions. Clin Experiment Ophthalmol 2006; 34(7):661665. 50. Curry W. T., Jr., Butler W. E., & Barker F. G. Rapidly rising incidence of cerebrospinal fluid shunting procedures for idiopathic intracranial hypertension in the United States, 19882002. Neurosurgery 2005; 57(1):97108. 51. Eggenberger E. R., Miller N. R., & Vitale S. Lumboperitoneal shunt for the treatment of pseudotumor cerebri. Neurology 1996; 46(6):15241530. 52. Rosenberg M., Smith C., & Beck R. The efficacy of shunting procedures in pseudotumor cerebri. Neurology 1989; 39:209. 53. Burgett R. A., Purvin V. A., & Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology 1997; 49(3):734739. 54. Johnston I., Besser M., & Morgan M. K. Cerebrospinal fluid diversion in the treatment of benign intracranial hypertension. J Neurosurg 1988; 69:195202. 55. McGirt M. J., Woodworth G., Thomas G., Miller N., Williams M., & Rigamonti D. Cerebrospinal fluid shunt placement for pseudotumor cerebriassociated intractable headache: Predictors of treatment response and an analysis of long-term outcomes. Journal of Neurosurgery 2004, 101(4):62732. 56. King J. O., Mitchell P. J., Thomson K. R., & Tress B. M. Manometry combined with cervical puncture in idiopathic intracranial hypertension. Neurology 2002; 58(1):2630. 57. Friedman D. I. Cerebral venous pressure, intra-abdominal pressure, and dural venous sinus stenting in idiopathic intracranial hypertension. J Neuroophthalmol 2006; 26(1):6164.

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I N S i G H T The Journal of the American Society of Ophthalmic Registered Nurses, Inc. |

AprilJune 2008

| Vol. XXXIII, No. 2

Continuing Nursing Education (CNE) Test

Idiopathic Intracranial Hypertension


This activity is provided by ASORN, which is accredited as a provider of continuing nursing education by the American Nurses Credentialing Centers Commission on Accreditation. ASORN is a provider approved by the California Board of Registered Nursing, provider number 11901. To receive (check appropriate box) : M 1.0 nursing contact hour M 1.0 Group A JCAHPO CEC for this activity, read the article, submit the completed posttest and general evaluation form within one year of the publication date, and achieve a passing rate of 80% or higher on the post-test. *Please note that JCAHPO CECs expire 2-22-09. Non-members, send your post-test with payment of $20 (free for members/afliates through June 30, 2009, $15 thereafter) to: ASORN PO Box 193030 San Francisco, CA 94119 Fax: 415.561.8531
Registration Information and Evaluation Response Form
Name Address City Work Phone RN# State Home Phone State of Licensure Zip Fax Exp. Date

CNE TE S T

Objectives
After completing this independent study activity, the participant should be able to:  Dene idiopathic intracranial hypertension (IIH) and the multiple effects it has on the visual system.  Discuss the clinical signs and subjective symptoms of IIH.  Deliberate the medical and surgical treatment protocols. Please allow a minimum of three weeks from date of receipt of post-test to receive your attendance verication certicate. Requests for certicate less than three weeks from date of receipt should be accompanied by an additional $10 fee.

Payment:

Check

Visa

Mastercard

CC#

Exp

Test response: Circle the most appropriate response matching test question number and response number. 1. A B C D 2. A B C D 3. A B C D 4. A B C D 5. A B C D 6. A B C D 7. A B C D 8. A B C D 9. A B C D 10. A B C D

General Evaluation: Please use the scale below to evaluate this educational activity and objectives. Circle your response. As a result of completing this offering, I am able to: Very Moderately Fairly Not at
well well well all

1.  Define idiopathic intracranial hypertension and the multiple effects it has on the visual system. 2. Discuss the clinical signs and subjective symptoms of IIH. 3. Deliberate the medical and surgical treatment protocols. 4. The content matches the objectives. 5. Independent study was an effective teaching method. 6. This course helped me achieve personal objectives. 7. The time required to complete this offering (in minutes) and take the test was:

4 4 4 4 4 4 60

3 3 3 3 3 3 75

2 2 2 2 2 2 90

1 1 1 1 1 1 >90

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I N S i G H T The Journal of the American Society of Ophthalmic Registered Nurses, Inc. |

AprilJune 2008

| Vol. XXXIII, No. 2

Continuing Nursing Education (CNE) Test

Idiopathic Intracranial Hypertension


1.  Which of the following patients is most likely to have IIH? A.  67-year-old female with a recent weight gain B.  28-year-old male with chronic headaches C. 35-year-old female with anorexia/bulimia D.  30-year-old female weighing 25% over ideal body weight 6.  Most patients respond to acetazolamide at a dosage level of: A. to one gram per day taken all at once B. to one gram per day taken in divided doses C. 12 grams per day taken all at night D. 12 grams per day taken in divided doses

CNE P O S T-TE S T

2. The most common symptoms of IIH are: A.  Headache and transient visual obscurations B. Headache and pulse synchronous tinnitus C. Photopsia and retrobulbar pain D.  Pulse synchronous tinnitus and retrobulbar pain

7.  An uncommon but possible serious side effect of treatment with acetazolamide is: A. Aplastic anemia B. Tingling in ngers and toes C. Metabolic acidosis D. Renal stones

3.  The most common visual defects found with IIH are: A.  Enlargement of the physiologic blind spot and central defect B. Central defect with temporal vision loss C.  Enlargement of the physiologic blind spot and loss of inferonasal portions of the visual eld D.  360-degree peripheral defect moving toward an enlarging physiological blind spot

8.  Use of CSF shunting procedures for treatment of IIH: A. was halted years ago B.  has been eliminated with the availability of new medications C. is decreasing D. is increasing

9. The cardinal sign of IIH is: A. 6th nerve palsy B. Papilledema C. Headache D. Temporal visual eld loss

4. When measuring CSF pressure it is important to: A.  Have the patient sedated to avoid the Valsalva maneuver B.  Have the patient supine and breathing quickly C.  Make the reference level for measurement the level of the left atrium D. Measure the pressure rst thing in the morning

10.  Surgical forms of treatment for IIH include all of the following except: A. Optic nerve sheath fenestration B. Subtemporal or suboccipital decompression C. Ethmoidectomy D. CSF shunts

5. The rst line treatment for IIH is: A. Lumbar puncture to reduce CSF pressure B. Low-sodium weight-reduction diet C. Vitamin A supplementation D. Lap band or gastric bypass surgery

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