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Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis

Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis
Literature review current through: Jan 2024.
This topic last updated: Mar 30, 2021.

INTRODUCTION — Idiopathic intracranial hypertension (IIH) is also called pseudotumor cerebri. It is a disorder defined by clinical criteria that include symptoms and signs isolated to those produced by increased intracranial pressure (eg, headache, papilledema, vision loss), elevated intracranial pressure with normal cerebrospinal fluid (CSF) composition, and no other cause of intracranial hypertension evident on neuroimaging or other evaluations. IIH primarily affects females of childbearing age who are overweight.

While once called "benign intracranial hypertension" to distinguish it from secondary intracranial hypertension produced by a malignancy, it is not a benign disorder. Many patients suffer from intractable, disabling headaches, and there is a risk of severe, permanent vision loss. Even patients with mild vision loss have an associated reduction in quality of life [1].

This topic will discuss the clinical features and diagnosis of IIH. The epidemiology and pathogenesis, as well as the prognosis and treatment of this disorder, are discussed separately. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis" and "Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment".)

CLINICAL PRESENTATION — A typical presentation of IIH is that of an overweight female of childbearing age who complains of headaches and is found to have papilledema on funduscopic examination. Other potential high-risk groups are discussed separately. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis", section on 'Risk factors' and "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis", section on 'Associated conditions'.)

Symptoms — In one case series and in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT), the most common symptoms of IIH were [2,3]:

Headache (84 to 92 percent)

Transient visual obscurations (68 to 72 percent)

Intracranial noises (pulsatile tinnitus; 52 to 60 percent)

Photopsia (48 to 54 percent)

Back pain (53 percent)

Retrobulbar pain (44 percent)

Diplopia (18 to 38 percent), typically from nonlocalizing sixth nerve palsy

Sustained visual loss (26 to 32 percent)

Neck pain (41 percent)

These symptoms, individually or as a cluster, are not specific for IIH. In one case-control study, these symptoms were also common in age- and gender-matched controls who were recruited from hospital waiting areas, although the prevalence, severity, and frequency were lower in this group [4]. Pulsatile tinnitus was the most useful distinguishing feature.

Headache — Headache is the most common presenting symptom of IIH [2,3]. However, the features of headaches in IIH patients are variable and are not specific to IIH. Many but not all patients note that the pain is of unusual severity [2,5]. The headaches may be lateralized and throbbing or pulsatile in character. They may be intermittent or persistent, occur daily or less frequently. Associated nausea and vomiting are not infrequent. Some patients describe headache exacerbation with changes in posture, and some may report that relief occurs with nonsteroidal antiinflammatory medications and/or rest. Retrobulbar pain and mild pain with eye movement or globe compression are somewhat more specific features for IIH. In some patients, the pain follows a trigeminal or cervical nerve root distribution [5]. Neck stiffness and back pain are also commonly reported [2,6].

In most cases, the features of headache are similar to other primary headache disorders including migraine and tension-type headache [7,8]. The often refractory nature of the headache may lead the patient to overuse analgesic medication, suggesting or even causing a superimposed rebound headache, further obscuring the diagnosis and making treatment challenging [9]. (See "Medication overuse headache: Etiology, clinical features, and diagnosis" and "Medication overuse headache: Treatment and prognosis".)

Rare patients present without headache [10,11]. Among younger children, headache is a less universal finding [6,12]; in one series, 29 percent of children with IIH did not have headache [13]. In one large case series, men were less likely to complain of headache than women [14]. In the absence of headache, the diagnosis is often suggested during a routine ocular examination when papilledema is incidentally discovered.

Visual symptoms

Transient obscurations and photopsias – Transient visual obscurations occur in approximately two-thirds of patients with papilledema. These last seconds at a time and can be bilateral or unilateral [2]. The frequency is variable, ranging from rare or isolated episodes to those occurring several times a day. Some patients note that these can be precipitated by changes in position (usually standing, but sometimes lying down or bending over), Valsalva, bright light, or eye movement (ie, gaze-evoked) [2,4]. Daily occurrence of transient visual obscurations is predictive of poor visual outcome [15].

Photopsias, brief sparkles or flashes of light, can also occur in patients with IIH and, similar to visual obscurations, can be provoked by positional changes and Valsalva. They are not specific for IIH and are not uncommon in the general population [4].

Vision loss – A subset of individuals with IIH have a more malignant or fulminant course with rapid development of vision loss within a few weeks of symptom onset [16-19]. This is generally apparent at presentation. To limit the severity of permanent vision loss, more aggressive treatment measures are considered at the outset, after the diagnosis is established. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment".)

Diplopia – Patients with IIH may report intermittent or continuous horizontal diplopia. This is typically due to a unilateral or bilateral sixth cranial nerve palsy or divergence insufficiency from increased intracranial pressure. Rarely, other causes of diplopia can occur in IIH (eg, other cranial neuropathies, decompensated phoria) [20] (see 'Other cranial nerve deficits' below). While sixth nerve palsies are documented in approximately 10 percent of cases [2], the symptom of horizontal diplopia is much more common (18 to 30 percent).

Pulsatile tinnitus — Pulsatile tinnitus (also called pulse synchronous tinnitus) is common in IIH, and in the setting of headache is very suggestive of IIH [4,9,21]. Patients often describe hearing rushing water or wind. This symptom can be persistent or intermittent and is believed to represent vascular pulsations transmitted by turbulence through the venous sinuses [22]. (See 'Magnetic resonance imaging' below.)

Examination — The most common signs in IIH are:

Papilledema

Visual field loss

Sixth nerve palsy

Papilledema — Papilledema is the hallmark sign of IIH (picture 1A-B). Papilledema is described in detail separately. (See "Overview and differential diagnosis of papilledema".)

While typically bilateral and symmetric, papilledema may be asymmetric or frankly unilateral [20,23-25]. In one series, 10 percent of 478 IIH patients had highly asymmetric papilledema with greater visual loss in the eye with higher grade of papilledema [23]. In the IIHTT, asymmetric papilledema was noted in 7 percent [3]. Such patients may also have a relative afferent pupillary defect.

Papilledema can be graded in severity. We use the Frisén scale for grading papilledema (table 1 and picture 1A-F). This scale is very useful, as IIH patients with grades 3 to 5 papilledema are at high risk for visual loss [15]. Patients with more severe papilledema are at higher risk of permanent visual loss [23]. It is helpful to take photographs of both optic discs and grade the severity of papilledema at the initial visit and each follow-up. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment", section on 'Monitoring during treatment'.)

Other findings on funduscopic examination may include macular exudates and macular edema, retinal and choroidal folds across the macula (usually resulting from flattening of the globes from increased intracranial pressure [26,27]), choroidal neovascularization, and serous retinal elevation around the nerve head [28].

Patients with headaches and elevated opening pressure on lumbar puncture (LP) but no papilledema have been described but are rare [8-10,29-33]. This has been speculated to be due to anatomic differences, or to early presentation prior to the development of papilledema [34]. It is also possible that these patients may not have the same IIH syndrome as those who present with papilledema. Opening pressures may not have been recorded correctly (see 'Lumbar puncture' below). It has been noted that opening pressures tend to be lower (perhaps only borderline elevated) in such patients. Importantly, patients without papilledema are at lower risk for vision loss as compared with typical patients with IIH with papilledema [8,33,35]. Since spurious elevations in intracranial pressure can occur and this diagnosis of IIH without papilledema is so difficult to make, we do not diagnose IIH in patients without papilledema unless there are supportive neuroimaging findings (eg, the presence of bilateral transverse sinus stenosis) [36].

Ophthalmologic evaluation is advised to confirm the presence of papilledema and to evaluate those patients with questionable or subtle papilledema [37]. (See 'Ophthalmologic evaluation' below.)

Vision loss — Loss of vision is the major morbidity in IIH and may be present on initial evaluation [2,38]. Vision loss is usually gradual but can be abrupt. Such patients have a more fulminant course and more significant permanent vision loss. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Prognosis and treatment", section on 'Prognosis'.)

Visual acuity is worse than 20/20 in 10 to 29 percent of patients on presentation [2,3,39-41]. However, visual acuity is a relatively insensitive measure of vision loss in IIH.

Visual field loss occurs before loss of acuity; confrontation visual fields are abnormal (nasal loss, temporal loss, visual blurring) in up to 32 percent at presentation, but are often insensitive [2,41]. Perimetry gives a more accurate and detailed assessment of visual field abnormalities and is an essential feature of the evaluation of a patient with IIH. Typical findings are described below. (See 'Ophthalmologic evaluation' below.)

Abducens palsy — A sixth cranial nerve (abducens) palsy may be unilateral or bilateral in patients with IIH [3,10]. This reflects a nonlocalizing effect of elevated intracranial pressure on the sixth nerve, which has a long intracranial course before exiting the skull.

Other cranial nerve deficits — Other cranial nerve deficits that resolve with IIH treatment are noted in case reports. Some of these may be more common in prepubertal children than in older patients [39,42]:

Olfactory [43-46]

Oculomotor [20,35,47,48]

Trochlear nerve [35,49]

Trigeminal nerve [20,47,50]

Facial nerve [6,13,47,51]

Auditory nerve [21,52,53]

Of these, facial nerve involvement is the most commonly reported.

Other neurologic signs — Patients with IIH do not display overt cognitive deficits. However, in one cohort of 31 patients with IIH who underwent neuropsychological testing, mild deficits in reaction time and processing speed were noted that persisted on retesting at three months despite improvement in measured intracranial pressure and in headache [54]. Patients were not on medications potentially affecting cognition at the time of initial testing. This result is difficult to interpret due to the similar effects of chronic headache on these measures.

EVALUATION

When to suspect elevated intracranial pressure — Increased intracranial pressure should be suspected in any patient with headache and papilledema. Urgent neuroimaging is required to exclude secondary causes of intracranial hypertension. If the neuroimaging study reveals no structural etiology for intracranial hypertension, a lumbar puncture (LP) is performed to document an opening pressure and cerebrospinal fluid (CSF) is analyzed to exclude other conditions. Ophthalmologic evaluation is required to document the severity of optic nerve involvement and monitor response to treatment.

The diagnosis of IIH may be suspected prior to the evaluation, on the basis of a history and examination that reveals conditions or medications that are associated with IIH; neuroimaging and LP are always required to exclude other conditions. (See "Idiopathic intracranial hypertension (pseudotumor cerebri): Epidemiology and pathogenesis", section on 'Associated conditions'.)

Essential testing

Magnetic resonance imaging — In a patient with headache and papilledema, the primary purpose of neuroimaging is to exclude secondary causes of increased intracranial pressure. (See 'Secondary intracranial hypertension' below.)

Magnetic resonance imaging (MRI) with magnetic resonance venography (MRV) is the preferred test. A computed tomography (CT) scan may be necessary for patients with contraindications to MRI (eg, pacemakers, metallic clips in head, metallic foreign bodies) and some patients with obesity or claustrophobia. The use of contrast enhancement increases the sensitivity of the study, particularly for subtle intracranial masses and meningeal-based pathologies [55].

MRV, particularly with contrast administration, is a more sensitive test than standard MRI for the detection of cerebral venous thrombosis (CVT) [56]. This potential cause of secondary intracranial hypertension can have a similar clinical presentation to IIH (see "Cerebral venous thrombosis: Etiology, clinical features, and diagnosis", section on 'Clinical aspects'). It is our practice to include a postcontrast MRV with MRI when evaluating patients with suspected IIH. If a patient comes to us with a normal MRI without MRV, and there are no other risk factors for CVT (including no oral contraceptive use), and the patient has a typical risk profile for IIH (a female of childbearing age who is overweight), we may defer MRV unless there is rapid clinical progression or a poor response to treatment.

While the brain parenchyma and ventricles appear normal on MRI or CT in patients with IIH, other MRI abnormalities may be seen and suggest IIH [57-63]:

Flattening of the posterior sclera (43 to 80 percent); this is the most specific of this group of signs [64]

Distension of perioptic subarachnoid space (45 to 67 percent)

Enhancement (with gadolinium) of the prelaminar optic nerve (7 to 50 percent)

Empty sella (25 to 80 percent)

Intraocular protrusion of the prelaminar optic nerve (3 to 30 percent)

Vertical tortuosity of the orbital optic nerve (40 percent)

These findings are not, individually or in the aggregate, diagnostic of IIH; nor does their absence exclude the diagnosis [57,64-66]. Other abnormalities reported in a few cases include tonsillar ectopia, narrowing of the Meckel's cave and cavernous sinuses, meningoceles, and widening of the foramen ovale [67-70].

There are many reports of cerebral venous abnormalities on MRV in patients with IIH. In particular, narrowing of the transverse venous sinus is common in IIH and suggests increased intracranial pressure [71]. Depending on the magnetic resonance technology utilized, this finding is reported in 65 to 90 percent of patients with IIH [36,62,72]. Bilateral transverse sinus stenosis is reasonably specific for IIH, occurring in 7 percent of normals [36].

Lumbar puncture — Elevated opening pressure on LP is an essential element of the diagnosis of IIH; however, there are some pitfalls in accurate measurement and interpretation.

For accurate pressure recording, the patient should be relaxed and lying in the lateral decubitus position with legs extended [55]. Other positions (prone, sitting) can give falsely elevated readings, as can anxiety and pain and the use of sedating medications [73]. Misleading low readings can be obtained after multiple LP attempts or in the setting of an anxious patient that hyperventilates and treatment with intracranial pressure-lowering medications. CSF pressures can vary, and a normal reading in a patient with IIH may reflect a spurious low reading for that patient as CSF pressure fluctuates throughout the day. Repeating the LP may be required in a patient if suspicion for IIH remains high after one normal CSF reading [35,74]. In rare patients, CSF pressure monitoring may be required to document elevated CSF pressure, but this is exceptional [37,75,76].

Traditionally, the upper limit of normal for opening pressure in adults is 200 mmH2O. It appears that overweight patients have a higher upper limit of normal, with opening pressures that may normally be up to 250 mmH2O [77,78]. However, others have not correlated obesity with elevated intracranial pressure in the absence of IIH [79,80]. We and others consider pressures less than 200 mmH2O to be normal, greater than 250 mmH2O to be abnormal, and 200 to 250 mmH2O to be equivocal [55,74,79]. When opening pressure measures are equivocal, we use the presence or absence of the MRI findings described above (especially transverse sinus stenosis and globe flattening) to support the diagnosis of IIH.

In young children (<8 years), higher upper limits of normal have been proposed, particularly in overweight or sedated children [73,81]. In one case series of 197 pediatric patients without symptoms or other evidence for increased intracranial pressure, the authors determined the upper limit of normal (based upon the 90th percentile) to be 280 mmH2O, and 250 mmH2O in children who were not sedated or overweight [73]. In a follow-up study, only 1 of 36 children with IIH had a pressure lower than 280 mmH2O (220 mmH2O), while 16 of 40 control patients had an opening pressure greater than 200 mmH2O (all were less than 280 mmH2O) [81]. It seems reasonable to consider very elevated levels (≥280 mmH2O) to be unequivocally abnormal (in a properly performed procedure), while intermediate levels (200 to 280 mmH2O) should be interpreted along with the preponderance of other clinical data that suggest a diagnosis of IIH [82].

In addition to measuring the opening pressure, the CSF is analyzed for cell count and differential, glucose, and protein. CSF composition (protein, cells, glucose) is normal in patients with IIH. Additional CSF studies for microbial agents, CSF cytology, and antigen testing (eg, CSF VDRL) may be indicated if the CSF cell count is abnormal or the clinical situation suggests additional testing. (See "Lumbar puncture: Technique, contraindications, and complications in adults".)

Ophthalmologic evaluation — Ophthalmologic evaluation is essential for all patients with suspected IIH. A complete ocular examination should document formal visual field examination, dilated fundus examination, and optic nerve photographs. Orbital ultrasound and fluorescein angiography can be utilized if the presence of papilledema is subtle or questionable. (See "Overview and differential diagnosis of papilledema".)

Visual field testing is essential in IIH to assess the severity of optic nerve involvement and monitor response to treatment. Options include Goldmann kinetic perimetry and the computer-assisted static perimetry. While each has advantages and limitations, the latter is generally preferred when there is mild visual loss, but since variability increases with increasing damage, Goldmann perimetry is preferred for cases with moderate to advanced visual loss.

The visual field loss is typically peripheral with nerve fiber bundle type defects predominating. The central visual field can be involved late in the course or earlier if there is concomitant macular pathology (serous detachment, macular hemorrhage, or edema), choroidal folds, or choroidal neovascular membrane. In the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT), the most common findings were a partial arcuate defect (commonly with a nasal step defect) coupled with an enlarged blind spot (approximately three-fourths of the hemifields in the study eye and approximately half of the hemifields in the fellow eye) [3]. Generalized constriction is also common [2]. Less commonly, central, paracentral, arcuate, and altitudinal scotomas may occur [55].

The frequency of visual field loss and acuity loss with IIH is somewhat variable. Among various reports, visual field loss was noted in 71 to 100 percent of eyes using various forms of perimetry [2,3,12,40,83,84].

Other testing — In addition, all patients with bilateral optic disc edema should have a measurement of the systemic blood pressure as a survey for the retinopathy related to malignant hypertension and can be associated with headache and other symptoms that might be mistaken for IIH. (See "Moderate to severe hypertensive retinopathy and hypertensive encephalopathy in adults".)

If anemia is suspected, IIH patients should have a complete blood count to exclude an anemia that may be contributory to the patient's condition [85].

DIAGNOSIS — IIH is diagnosed according to the modified Dandy criteria; each of the following apply [74,86,87]:

Symptoms and signs of increased intracranial pressure (eg, headache, transient visual obscurations, pulse synchronous tinnitus, papilledema, visual loss)

No other neurologic abnormalities or impaired level of consciousness

Elevated intracranial pressure with normal cerebrospinal fluid (CSF) composition

A neuroimaging study that shows no etiology for intracranial hypertension

No other cause of intracranial hypertension apparent

While revisions to these guidelines have been proposed, they do not substantially change the approach to diagnosis [33]. Because treatment trials have used the modified Dandy criteria, we believe that these remain the most appropriate criteria for making the diagnosis of IIH.

DIFFERENTIAL DIAGNOSIS — When papilledema (optic disc edema due to raised intracranial pressure) is present, there is elevated intracranial pressure, which can have many etiologies in addition to IIH. There are also many etiologies of unilateral or bilateral optic disc swelling, which can have a similar appearance to papilledema.

Secondary intracranial hypertension — Any entity that increases intracranial pressure may lead to papilledema. These include:

Intracranial mass lesions (tumor, abscess)

Obstruction of venous outflow (eg, venous sinus thrombosis, jugular vein compression, neck surgery)

Obstructive hydrocephalus

Decreased cerebrospinal fluid (CSF) absorption (eg, arachnoid granulation adhesions after bacterial or other infectious meningitis, subarachnoid hemorrhage)

Increased CSF production (eg, choroid plexus papilloma)

Most of the conditions are excluded by magnetic resonance imaging (MRI). Magnetic resonance venography (MRV) is often required to exclude conditions causing venous outflow obstruction. In particular, cerebral venous thrombosis (CVT) can have a very similar clinical presentation as IIH [88-90]. (See "Cerebral venous thrombosis: Etiology, clinical features, and diagnosis", section on 'Clinical aspects'.)

Other unusual causes of obstructed venous outflow include transverse sinus septum causing sinus stenosis [91], osteopetrosis of the jugular foramen [92-96], and depressed skull fracture and stenosis of the superior sagittal sinus [97]. Venous hypertension and secondary increase in intracranial hypertension can also be caused by cerebral arteriovenous malformations, dural arteriovenous malformations, and arteriovenous fistulas, as well as by increased right heart pressure and superior vena cava syndrome [33,40,98-107]. Some patients thought to have IIH have later been discovered to have one of these conditions [40,56,88,108,109].

Optic disc abnormalities — There are many causes of an elevated optic nerve head. While the term "papilledema" is sometimes used to describe the findings in these conditions, it should be reserved for patients who have elevated optic disc heads as a consequence of increased intracranial pressure. The table lists the causes of optic disc swelling (table 2). These are discussed in detail separately (see "Overview and differential diagnosis of papilledema"). Funduscopic examination by an ophthalmologist is recommended to confirm the presence of true papilledema.

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

Basics topics (see "Patient education: Idiopathic intracranial hypertension (pseudotumor cerebri) (The Basics)")

SUMMARY AND RECOMMENDATIONS

A typical presentation of idiopathic intracranial hypertension (IIH) is that of a female of childbearing age who presents with headaches and is found to have papilledema on funduscopic examination.

Headache characteristics in IIH are variable and nonspecific, but usually include daily occurrence, unusual severity, and a throbbing quality. (See 'Headache' above.)

Papilledema is usually bilateral and symmetric; the severity of papilledema is associated with the risk of permanent visual loss. (See 'Papilledema' above.)

Other common features of IIH that are unusual in other primary headache disorders are transient visual obscurations, pulsatile tinnitus, and diplopia (often in the setting of abducens palsy). Of these, pulse synchronous tinnitus is the most specific for IIH. (See 'Symptoms' above.)

A neuroimaging study is required in patients suspected of having increased intracranial pressure to exclude other causes of elevated intracranial pressure. Magnetic resonance imaging (MRI) with and without contrast, including postcontrast magnetic resonance venography (MRV), is the imaging study of choice. (See 'Magnetic resonance imaging' above.)

MRI findings that suggest, but are not diagnostic for, IIH include empty sella, flattening of the posterior aspect of the globe, distension of the perioptic subarachnoid space (with or without a tortuous optic nerve), and transverse venous sinus stenosis. (See 'Magnetic resonance imaging' above.)

Lumbar puncture (LP) should follow MRI unless a source of elevated intracranial pressure is clearly delineated. An opening pressure greater than 250 mmH2O taken with the patient lying on his or her side with legs extended confirms elevated intracranial pressure. Pressures between 200 and 250 mmH2O are considered equivocal and require presence of associated clinical findings like stenosis of the transverse venous sinuses. Higher upper limits of normal may be considered in overweight patients or those that are sedated. Cerebrospinal fluid (CSF) composition is normal in IIH. (See 'Lumbar puncture' above.)

Visual field testing is an essential part of the evaluation of patients with IIH and provides a means for following the patient and directing treatment. Arcuate defects and constriction are commonly found on visual field examinations. (See 'Ophthalmologic evaluation' above.)

Diagnostic criteria of IIH require the presence of each of the following (see 'Diagnosis' above):

Papilledema or sixth (abducens) nerve palsy (unilateral or bilateral)

Normal neurologic examination, except for papilledema and cranial nerve abnormalities

Neuroimaging (MRI with and without gadolinium and MRV is preferred) shows normal brain parenchyma without evidence of hydrocephalus, mass, structural lesion, or meningeal enhancement

Normal CSF composition

Elevated LP opening pressure

IIH must be distinguished from other causes of increased intracranial pressure and other etiologies of optic nerve head swelling. Cerebral venous thrombosis (CVT) in particular may have a very similar clinical presentation to IIH. (See 'Differential diagnosis' above.)

  1. Digre KB, Bruce BB, McDermott MP, et al. Quality of life in idiopathic intracranial hypertension at diagnosis: IIH Treatment Trial results. Neurology 2015; 84:2449.
  2. Wall M, George D. Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain 1991; 114 ( Pt 1A):155.
  3. Wall M, Kupersmith MJ, Kieburtz KD, et al. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol 2014; 71:693.
  4. Giuseffi V, Wall M, Siegel PZ, Rojas PB. Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology 1991; 41:239.
  5. Wall M. The headache profile of idiopathic intracranial hypertension. Cephalalgia 1990; 10:331.
  6. Lessell S. Pediatric pseudotumor cerebri (idiopathic intracranial hypertension). Surv Ophthalmol 1992; 37:155.
  7. Friedman DI, Rausch EA. Headache diagnoses in patients with treated idiopathic intracranial hypertension. Neurology 2002; 58:1551.
  8. Mathew NT, Ravishankar K, Sanin LC. Coexistence of migraine and idiopathic intracranial hypertension without papilledema. Neurology 1996; 46:1226.
  9. Wang SJ, Silberstein SD, Patterson S, Young WB. Idiopathic intracranial hypertension without papilledema: a case-control study in a headache center. Neurology 1998; 51:245.
  10. Quattrone A, Bono F, Fera F, Lavano A. Isolated unilateral abducens palsy in idiopathic intracranial hypertension without papilledema. Eur J Neurol 2006; 13:670.
  11. Torun N, Sharpe J. Pseudotumor cerebri mimicking Foster Kennedy syndrome. Neuroophthalmology 1996; 16:55.
  12. Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, and outcome. Am J Ophthalmol 1999; 127:178.
  13. Lim M, Kurian M, Penn A, et al. Visual failure without headache in idiopathic intracranial hypertension. Arch Dis Child 2005; 90:206.
  14. Bruce BB, Kedar S, Van Stavern GP, et al. Idiopathic intracranial hypertension in men. Neurology 2009; 72:304.
  15. Wall M, Falardeau J, Fletcher WA, et al. Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension. Neurology 2015; 85:799.
  16. Thambisetty M, Lavin PJ, Newman NJ, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology 2007; 68:229.
  17. Liu GT, Glaser JS, Schatz NJ. High-dose methylprednisolone and acetazolamide for visual loss in pseudotumor cerebri. Am J Ophthalmol 1994; 118:88.
  18. Kidron D, Pomeranz S. Malignant pseudotumor cerebri. Report of two cases. J Neurosurg 1989; 71:443.
  19. Fraser CL, Biousse V, Newman NJ. Minocycline-induced fulminant intracranial hypertension. Arch Neurol 2012; 69:1067.
  20. Chari C, Rao NS. Benign intracranial hypertension--its unusual manifestations. Headache 1991; 31:599.
  21. Rudnick E, Sismanis A. Pulsatile tinnitus and spontaneous cerebrospinal fluid rhinorrhea: indicators of benign intracranial hypertension syndrome. Otol Neurotol 2005; 26:166.
  22. Sismanis A, Butts FM, Hughes GB. Objective tinnitus in benign intracranial hypertension: an update. Laryngoscope 1990; 100:33.
  23. Wall M, White WN 2nd. Asymmetric papilledema in idiopathic intracranial hypertension: prospective interocular comparison of sensory visual function. Invest Ophthalmol Vis Sci 1998; 39:134.
  24. Greenfield DS, Wanichwecharungruang B, Liebmann JM, Ritch R. Pseudotumor cerebri appearing with unilateral papilledema after trabeculectomy. Arch Ophthalmol 1997; 115:423.
  25. Saito J, Kami M, Taniguchi F, et al. Unilateral papilledema after bone marrow transplantation. Bone Marrow Transplant 1999; 23:963.
  26. Sibony PA, Kupersmith MJ, Feldon SE, et al. Retinal and Choroidal Folds in Papilledema. Invest Ophthalmol Vis Sci 2015; 56:5670.
  27. Sibony PA, Kupersmith MJ, Feldon SE, Kardon R. Author Response: Choroidal Folds in Astronauts. Invest Ophthalmol Vis Sci 2016; 57:593.
  28. Acheson JF. Idiopathic intracranial hypertension and visual function. Br Med Bull 2006; 79-80:233.
  29. Krishna R, Kosmorsky GS, Wright KW. Pseudotumor cerebri sine papilledema with unilateral sixth nerve palsy. J Neuroophthalmol 1998; 18:53.
  30. Marcelis J, Silberstein SD. Idiopathic intracranial hypertension without papilledema. Arch Neurol 1991; 48:392.
  31. Vieira DS, Masruha MR, Gonçalves AL, et al. Idiopathic intracranial hypertension with and without papilloedema in a consecutive series of patients with chronic migraine. Cephalalgia 2008; 28:609.
  32. Beri S, Gosalakkal JA, Hussain N, et al. Idiopathic intracranial hypertension without papilledema. Pediatr Neurol 2010; 42:56.
  33. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013; 81:1159.
  34. Galetta SL, Balcer LJ. All choked up about the pseudotumor cerebri syndrome. Neurology 2013; 81:1112.
  35. Soler D, Cox T, Bullock P, et al. Diagnosis and management of benign intracranial hypertension. Arch Dis Child 1998; 78:89.
  36. Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology 2003; 60:1418.
  37. Krishnakumar D, Pickard JD, Czosnyka Z, et al. Idiopathic intracranial hypertension in childhood: pitfalls in diagnosis. Dev Med Child Neurol 2014; 56:749.
  38. Corbett JJ, Savino PJ, Thompson HS, et al. Visual loss in pseudotumor cerebri. Follow-up of 57 patients from five to 41 years and a profile of 14 patients with permanent severe visual loss. Arch Neurol 1982; 39:461.
  39. Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 2007; 52:597.
  40. Celebisoy N, Seçil Y, Akyürekli O. Pseudotumor cerebri: etiological factors, presenting features and prognosis in the western part of Turkey. Acta Neurol Scand 2002; 106:367.
  41. Salman MS, Kirkham FJ, MacGregor DL. Idiopathic "benign" intracranial hypertension: case series and review. J Child Neurol 2001; 16:465.
  42. Warman R. Management of pseudotumor cerebri in children. Int Pediatr 2000; 15:147.
  43. Kunte H, Schmidt F, Kronenberg G, et al. Olfactory dysfunction in patients with idiopathic intracranial hypertension. Neurology 2013; 81:379.
  44. Schmidt C, Wiener E, Hoffmann J, et al. Structural olfactory nerve changes in patients suffering from idiopathic intracranial hypertension. PLoS One 2012; 7:e35221.
  45. Bershad EM, Urfy MZ, Calvillo E, et al. Marked olfactory impairment in idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 2014; 85:959.
  46. Kapoor KG. Do patients with idiopathic intracranial hypertension suffer from hyposmia? Med Hypotheses 2008; 71:816.
  47. Capobianco DJ, Brazis PW, Cheshire WP. Idiopathic intracranial hypertension and seventh nerve palsy. Headache 1997; 37:286.
  48. Tan H. Bilateral oculomotor palsy secondary to pseudotumor cerebri. Pediatr Neurol 2010; 42:141.
  49. Speer C, Pearlman J, Phillips PH, et al. Fourth cranial nerve palsy in pediatric patients with pseudotumor cerebri. Am J Ophthalmol 1999; 127:236.
  50. Arsava EM, Uluc K, Nurlu G, Kansu T. Electrophysiological evidence of trigeminal neuropathy in pseudotumor cerebri. J Neurol 2002; 249:1601.
  51. Selky AK, Dobyns WB, Yee RD. Idiopathic intracranial hypertension and facial diplegia. Neurology 1994; 44:357.
  52. Dorman PJ, Campbell MJ, Maw AR. Hearing loss as a false localising sign in raised intracranial pressure. J Neurol Neurosurg Psychiatry 1995; 58:516.
  53. Malomo AO, Idowu OE, Shokunbi MT, et al. Non-operative management of benign intracranial hypertension presenting with complete visual loss and deafness. Pediatr Neurosurg 2006; 42:62.
  54. Yri HM, Fagerlund B, Forchhammer HB, Jensen RH. Cognitive function in idiopathic intracranial hypertension: a prospective case-control study. BMJ Open 2014; 4:e004376.
  55. Friedman DI. Papilledema and pseudotumor cerebri. Ophthalmol Clin North Am 2001; 14:129.
  56. Lin A, Foroozan R, Danesh-Meyer HV, et al. Occurrence of cerebral venous sinus thrombosis in patients with presumed idiopathic intracranial hypertension. Ophthalmology 2006; 113:2281.
  57. Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 1998; 105:1686.
  58. Gibby WA, Cohen MS, Goldberg HI, Sergott RC. Pseudotumor cerebri: CT findings and correlation with vision loss. AJR Am J Roentgenol 1993; 160:143.
  59. Jacobson DM, Karanjia PN, Olson KA, Warner JJ. Computed tomography ventricular size has no predictive value in diagnosing pseudotumor cerebri. Neurology 1990; 40:1454.
  60. Manfré L, Lagalla R, Mangiameli A, et al. Idiopathic intracranial hypertension: orbital MRI. Neuroradiology 1995; 37:459.
  61. Yuh WT, Zhu M, Taoka T, et al. MR imaging of pituitary morphology in idiopathic intracranial hypertension. J Magn Reson Imaging 2000; 12:808.
  62. Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol 2011; 32:1986.
  63. Hoffmann J, Huppertz HJ, Schmidt C, et al. Morphometric and volumetric MRI changes in idiopathic intracranial hypertension. Cephalalgia 2013; 33:1075.
  64. Agid R, Farb RI, Willinsky RA, et al. Idiopathic intracranial hypertension: the validity of cross-sectional neuroimaging signs. Neuroradiology 2006; 48:521.
  65. Maralani PJ, Hassanlou M, Torres C, et al. Accuracy of brain imaging in the diagnosis of idiopathic intracranial hypertension. Clin Radiol 2012; 67:656.
  66. Ridha MA, Saindane AM, Bruce BB, et al. MRI findings of elevated intracranial pressure in cerebral venous thrombosis versus idiopathic intracranial hypertension with transverse sinus stenosis. Neuroophthalmology 2013; 37:1.
  67. Degnan AJ, Levy LM. Narrowing of Meckel's cave and cavernous sinus and enlargement of the optic nerve sheath in Pseudotumor Cerebri. J Comput Assist Tomogr 2011; 35:308.
  68. Butros SR, Goncalves LF, Thompson D, et al. Imaging features of idiopathic intracranial hypertension, including a new finding: widening of the foramen ovale. Acta Radiol 2012; 53:682.
  69. Banik R, Lin D, Miller NR. Prevalence of Chiari I malformation and cerebellar ectopia in patients with pseudotumor cerebri. J Neurol Sci 2006; 247:71.
  70. Bialer OY, Rueda MP, Bruce BB, et al. Meningoceles in idiopathic intracranial hypertension. AJR Am J Roentgenol 2014; 202:608.
  71. Kelly LP, Saindane AM, Bruce BB, et al. Does bilateral transverse cerebral venous sinus stenosis exist in patients without increased intracranial pressure? Clin Neurol Neurosurg 2013; 115:1215.
  72. Riggeal BD, Bruce BB, Saindane AM, et al. Clinical course of idiopathic intracranial hypertension with transverse sinus stenosis. Neurology 2013; 80:289.
  73. Avery RA, Shah SS, Licht DJ, et al. Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med 2010; 363:891.
  74. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002; 59:1492.
  75. Spence JD, Amacher AL, Willis NR. Benign intracranial hypertension without papilledema: role of 24-hour cerebrospinal fluid pressure monitoring in diagnosis and management. Neurosurgery 1980; 7:326.
  76. Torbey MT, Geocadin RG, Razumovsky AY, et al. Utility of CSF pressure monitoring to identify idiopathic intracranial hypertension without papilledema in patients with chronic daily headache. Cephalalgia 2004; 24:495.
  77. Whiteley W, Al-Shahi R, Warlow CP, et al. CSF opening pressure: reference interval and the effect of body mass index. Neurology 2006; 67:1690.
  78. Berdahl JP, Fleischman D, Zaydlarova J, et al. Body mass index has a linear relationship with cerebrospinal fluid pressure. Invest Ophthalmol Vis Sci 2012; 53:1422.
  79. Corbett JJ, Mehta MP. Cerebrospinal fluid pressure in normal obese subjects and patients with pseudotumor cerebri. Neurology 1983; 33:1386.
  80. Bono F, Lupo MR, Serra P, et al. Obesity does not induce abnormal CSF pressure in subjects with normal cerebral MR venography. Neurology 2002; 59:1641.
  81. Avery RA, Licht DJ, Shah SS, et al. CSF opening pressure in children with optic nerve head edema. Neurology 2011; 76:1658.
  82. Aylward SC. Pediatric idiopathic intracranial hypertension: a need for clarification. Pediatr Neurol 2013; 49:303.
  83. Wall M, George D. Visual loss in pseudotumor cerebri. Incidence and defects related to visual field strategy. Arch Neurol 1987; 44:170.
  84. Galvin JA, Van Stavern GP. Clinical characterization of idiopathic intracranial hypertension at the Detroit Medical Center. J Neurol Sci 2004; 223:157.
  85. Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol 2003; 135:437.
  86. Friedman DI, McDermott MP, Kieburtz K, et al. The idiopathic intracranial hypertension treatment trial: design considerations and methods. J Neuroophthalmol 2014; 34:107.
  87. Smith JL. Whence pseudotumor cerebri? J Clin Neuroophthalmol 1985; 5:55.
  88. Biousse V, Ameri A, Bousser MG. Isolated intracranial hypertension as the only sign of cerebral venous thrombosis. Neurology 1999; 53:1537.
  89. Leker RR, Steiner I. Features of dural sinus thrombosis simulating pseudotumor cerebri. Eur J Neurol 1999; 6:601.
  90. Sylaja PN, Ahsan Moosa NV, Radhakrishnan K, et al. Differential diagnosis of patients with intracranial sinus venous thrombosis related isolated intracranial hypertension from those with idiopathic intracranial hypertension. J Neurol Sci 2003; 215:9.
  91. Subramaniam RM, Tress BM, King JO, et al. Transverse sinus septum: a new aetiology of idiopathic intracranial hypertension? Australas Radiol 2004; 48:114.
  92. Angeli SI, Sato Y, Gantz BJ. Glomus jugulare tumors masquerading as benign intracranial hypertension. Arch Otolaryngol Head Neck Surg 1994; 120:1277.
  93. Jicha GA, Suarez GA. Pseudotumor cerebri reversed by cardiac septal defect repair. Neurology 2003; 60:2016.
  94. Kiers L, King JO. Increased intracranial pressure following bilateral neck dissection and radiotherapy. Aust N Z J Surg 1991; 61:459.
  95. Lam BL, Schatz NJ, Glaser JS, Bowen BC. Pseudotumor cerebri from cranial venous obstruction. Ophthalmology 1992; 99:706.
  96. Siatkowski RM, Vilar NF, Sternau L, Coin CG. Blindness from bad bones. Surv Ophthalmol 1999; 43:487.
  97. Fuentes S, Metellus P, Levrier O, et al. Depressed skull fracture overlying the superior sagittal sinus causing benign intracranial hypertension. Description of two cases and review of the literature. Br J Neurosurg 2005; 19:438.
  98. Adelman JU. Headaches and papilledema secondary to dural arteriovenous malformation. Headache 1998; 38:621.
  99. Chimowitz MI, Little JR, Awad IA, et al. Intracranial hypertension associated with unruptured cerebral arteriovenous malformations. Ann Neurol 1990; 27:474.
  100. Cockerell OC, Lai HM, Ross-Russell RW. Pseudotumour cerebri associated with arteriovenous malformations. Postgrad Med J 1993; 69:637.
  101. Cognard C, Casasco A, Toevi M, et al. Dural arteriovenous fistulas as a cause of intracranial hypertension due to impairment of cranial venous outflow. J Neurol Neurosurg Psychiatry 1998; 65:308.
  102. David CA, Peerless SJ. Pseudotumor syndrome resulting from a cerebral arteriovenous malformation: case report. Neurosurgery 1995; 36:588.
  103. Lee A, Hayman L, Alpert J, et al. Occult cerebral vascular causes of pseudotumor cerebri. Neuroophthalmology 1999; 21:157.
  104. Martin TJ, Bell DA, Wilson JA. Papilledema in a man with an "occult" dural arteriovenous malformation. J Neuroophthalmol 1998; 18:49.
  105. Rosenfeld JV, Widaa HA, Adams CB. Cerebral arteriovenous malformation causing benign intracranial hypertension--case report. Neurol Med Chir (Tokyo) 1991; 31:523.
  106. Silberstein P, Kottos P, Worner C, et al. Dural arteriovenous fistulae causing pseudotumour cerebri syndrome in an elderly man. J Clin Neurosci 2003; 10:242.
  107. Vorstman EB, Niemann DB, Molyneux AJ, Pike MG. Benign intracranial hypertension associated with arteriovenous malformation. Dev Med Child Neurol 2002; 44:133.
  108. Tehindrazanarivelo A, Evrard S, Schaison M, et al. Prospective study of cerebral sinus venous thrombosis in patients presenting with benign intracranial hypertension. Cerebrovasc Dis 1992; 2:22.
  109. Wall M. Idiopathic intracranial hypertension. Neurol Clin 2010; 28:593.
Topic 5257 Version 19.0

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