Idiopathic Intracranial REVIEW ARTICLE


Hypertension C O N T I N U UM A U D I O
I NT E R V I E W A V A I L AB L E
ONLINE
By Matthew J. Thurtell, MBBS, MSc, FRACP

ABSTRACT
PURPOSE OF REVIEW: Idiopathic intracranial hypertension is a syndrome of
increased intracranial pressure of unclear etiology that most often occurs
in obese women of childbearing age but can also occur in men, children,
and older adults. This article reviews the diagnostic criteria, clinical
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features, neuroimaging findings, differential diagnosis, and management

options for this condition.

RECENT FINDINGS: Recent population studies have found that the annual
incidence of idiopathic intracranial hypertension is increasing in association
with obesity rates, whereas recent scientific studies indicate a possible
role for androgen sex hormones and adipose tissue in the pathogenesis of
the disease. Prospective clinical trials have demonstrated a role for weight
loss, acetazolamide, and topiramate in the management of mild disease. A
CITE AS:
recently begun randomized multicenter trial of surgical interventions will
CONTINUUM (MINNEAP MINN) 2019;
provide insight into the indications for surgical intervention, optimal timing 25(5, NEURO-OPHTHALMOLOGY):
and choice of intervention, and long-term outcomes. 1289–1309.

Address correspondence to
SUMMARY: Idiopathic intracranial hypertension is a disorder producing Dr Matthew J. Thurtell,
symptoms and signs of increased intracranial pressure in the absence of an University of Iowa Hospitals &
alternative cause. The main goals of treatment are to preserve visual Clinics, 200 Hawkins Dr,
Pomerantz Family Pavilion,
function and alleviate symptoms, which can usually be achieved with a Iowa City, IA 52242,
combination of weight loss, medical therapies, and surgical interventions matthew-thurtell@uiowa.edu.
depending on the severity of symptoms and vision loss, response to
RELATIONSHIP DISCLOSURE:
treatment, and subsequent clinical course. Dr Thurtell serves on the
editorial board of the Journal of
Neuro-Ophthalmology, receives
research/grant support from
the National Eye Institute
INTRODUCTION (U10-EY025990), and receives

I
diopathic intracranial hypertension (IIH; formerly known as pseudotumor book royalties from Oxford
cerebri or benign intracranial hypertension) is a syndrome of increased University Press.

intracranial pressure of unclear etiology that most often occurs in obese UNLABELED USE OF
women of childbearing age. Since IIH is a diagnosis of exclusion, other PRODUCTS/INVESTIGATIONAL

etiologies of increased intracranial pressure (TABLE 5-11) must be ruled out. A USE DISCLOSURE:
Dr Thurtell discusses the
number of diagnostic criteria for IIH have been proposed, but a diagnosis can unlabeled/investigational use
usually be confidently made in accordance with the modified Dandy criteria: of acetazolamide, furosemide,
methazolamide, and topiramate
(1) awake and alert patient; (2) symptoms and signs of increased intracranial for the treatment of idiopathic
pressure; (3) absence of focal signs on neurologic examination (although intracranial hypertension.
sixth and seventh nerve palsies are permitted); (4) normal diagnostic studies
(ie, neuroimaging and CSF evaluation), except for evidence of increased © 2019 American Academy
intracranial pressure (ie, a CSF opening pressure greater than 20 cm H2O with of Neurology.

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IDIOPATHIC INTRACRANIAL HYPERTENSION

signs of increased intracranial pressure on neuroimaging); and (5) no other etiology

for increased intracranial pressure identified.2
The pathogenesis of IIH remains poorly understood and controversial.3 A
variety of mechanisms have been proposed, including blockage of CSF absorption
at the level of the arachnoid villi, perhaps as a consequence of or exacerbated by
cerebral venous hypertension secondary to transverse venous sinus stenosis.3
Given the increased incidence in women and strong association with obesity, sex
hormones (eg, androgens) and adipose tissue may play a role in the pathogenesis
of IIH.4

EPIDEMIOLOGY
IIH most commonly occurs in obese women of childbearing age. The incidence
of IIH is variable, being higher in geographic areas that have a higher prevalence
of obesity. A study published in 1988 reported an annual incidence of IIH of
about 1 per 100,000 in the general populations of Iowa and Louisiana.5 However,
a study published in 2017 reported that the incidence of IIH had more than
doubled from 1.0 per 100,000 (in 1990–2001) to 2.4 per 100,000 (in 2002–2014)
in Minnesota.6 The incidence increased to 22 per 100,000 in obese women aged
15 to 44 years.6 Of note, this study reported a strong correlation between IIH
incidence and obesity rates (R2 = 0.7).6 A high body mass index (BMI) was found
to be associated with increased risk of IIH in a multicenter case-control study
that compared women with newly diagnosed IIH to women with other neuro-
ophthalmic disorders.7 This study found that greater levels of weight gain were
associated with an increased risk of IIH, although an increased risk of IIH also
existed in women who were not obese (BMI <30) in the setting of moderate
weight gain.7
IIH can also occur, albeit much less commonly, in men, children, and older
adults. A 2017 study reported that the annual incidence of IIH in Minnesota
was 0.3 per 100,000 in men compared to 3.3 per 100,000 in women.6 However,
in the Idiopathic Intracranial Hypertension Treatment Trial, only four of the
165 participants (2.4%) were men; of note, patients who had diagnosed
untreated obstructive sleep apnea were excluded, which may partly account
for the low percentage of men recruited.8 The BMI of men with IIH is similar
to that of women with IIH, although men tend to be older at the time of initial

TABLE 5-1 Differential Diagnosis of Increased Intracranial Pressurea

◆ Intracranial mass: eg, tumor, hemorrhage

◆ Blockage of ventricular system (obstructive hydrocephalus): eg, tumor
◆ Blockage of CSF absorption (communicating hydrocephalus): eg, subarachnoid hemorrhage
◆ Obstruction of venous outflow: eg, cerebral venous sinus thrombosis
◆ Diffuse cerebral edema: eg, following head injury
◆ Increased CSF secretion: eg, choroid plexus tumor
◆ Idiopathic: eg, idiopathic intracranial hypertension

CSF = cerebrospinal fluid.

a
Modified with permission from Thurtell MJ, Tomsak RL.1 © 2019 Oxford University Press.

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presentation.9 IIH also occurs in children but is not common. In a 2017 British KEY POINTS
study, the annual incidence of IIH in children and adolescents (aged 1 to 16 years)
● Idiopathic intracranial
was 0.71 per 100,000.10 The incidence increased to 4.18 per 100,000 in obese hypertension is a syndrome
males aged 12 to 15 years and 10.7 per 100,000 in obese females aged 12 to of increased intracranial
15 years.10 Another large retrospective multicenter study confirmed a similar pressure that usually occurs
trend in children and adolescents, suggesting the presence of three distinct in obese women of
childbearing age.
groups of patients in this population: a young group that is not overweight, an
early adolescent group that is overweight or obese, and a late adolescent group ● Idiopathic intracranial
that is mostly obese.11 Only a small percentage of patients present at an older hypertension is a diagnosis
age (ie, >50 years of age).12 Although older patients with IIH are usually obese, of exclusion. Therefore,
they tend to have a more benign clinical course compared to their younger other etiologies of increased
intracranial pressure must
counterparts.12 be ruled out based on
clinical history,
CLINICAL FEATURES neuroimaging, and CSF
Patients with IIH usually present with symptoms and signs of increased examination.
intracranial pressure. Common symptoms include headache, transient visual ● The incidence of
obscurations, and pulse-synchronous (pulsatile) tinnitus, whereas common idiopathic intracranial
signs include papilledema with or without associated retinal hemorrhages, hypertension appears to be
folds, cotton wool spots, and exudates. increasing and is strongly
correlated with obesity
rates.
Symptoms
Headache is the most common symptom of IIH. In the Idiopathic Intracranial ● Greater levels of weight
Hypertension Treatment Trial, 84% of participants had headache at presentation, gain are associated with
although neck and back pain were often reported too.8 The headache of increased increased risk of idiopathic
intracranial hypertension,
intracranial pressure is typically a global headache that is most severe in the although the condition can
morning and is often aggravated by maneuvers that increase the intracranial also develop in the setting of
pressure (eg, Valsalva-like maneuvers), with associated nausea and vomiting. moderate weight gain in
However, many patients with IIH have headaches with features of other headache patients who are not obese.
disorders, such as migraine and tension headache.13,14 Some have a significant ● Headache is the most
rebound component to their headache due to excessive use of simple analgesic common symptom of
medications.13,14 Although headache is often disabling and associated with poor idiopathic intracranial
quality of life, headache disability (based on the Headache Impact Test-6 score) is hypertension. However,
many patients have
not correlated with CSF opening pressure.14 Furthermore, the headache may or
headaches that have
may not improve with lowering of intracranial pressure.14 features of other primary
Transient visual obscurations (TVOs) occur in about 68% of patients with headache disorders, such as
IIH.8,15 TVOs are characterized by a partial or complete loss of vision that lasts for migraine and tension
headache.
several seconds, followed by a rapid recovery of vision back to baseline. TVOs
can occur many times per day and are often precipitated by postural changes or ● Headache in idiopathic
Valsalva-like maneuvers. TVOs are thought to result from transient ischemia of intracranial hypertension is
the edematous optic nerve head. They are associated with higher grades of often disabling and
papilledema and were found to be a predictor of treatment failure in the associated with poorer
quality of life but is not
Idiopathic Intracranial Hypertension Treatment Trial.16 correlated with intracranial
Patients with IIH are less likely to report persisting visual symptoms than pressure and, thus, may not
TVOs at initial presentation.8,17 Some have blurred vision due to hyperopic improve with lowering of
shift (from shortening of the globe due to increased intracranial pressure) or intracranial pressure.
metamorphopsia (distortion of vision) due to retinal folds.18 While an observant
patient might notice an enlarged blind spot, many do not notice visual field loss.
Consequently, the visual field loss from papilledema can go unnoticed until it is
severe and irreversible, underscoring the importance of perimetry (visual field
testing) in the evaluation and monitoring of patients with IIH.17,19 Central vision

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IDIOPATHIC INTRACRANIAL HYPERTENSION
KEY POINTS
● Transient visual
obscurations are the second
most common symptom of
idiopathic intracranial
hypertension. They are
thought to result from
transient ischemia of the
optic nerve head and are
associated with higher
grades of papilledema.
● Progressive visual field
loss may not be appreciated
by patients, underscoring
the importance of formal
perimetry (visual field
testing) in the evaluation and
monitoring of idiopathic
intracranial hypertension.
● Pulse-synchronous
(pulsatile) tinnitus occurs in
about half of patients with
idiopathic intracranial
hypertension and is thought
to arise because of turbulent
blood flow across
transverse venous sinus
stenoses.
● Papilledema is the most
common and important sign
in idiopathic intracranial
hypertension. It is usually
bilateral and symmetric. The
threat of vision loss is
correlated with its severity.
● If untreated, papilledema
can result in progressive and
irreversible vision loss with
optic atrophy.
● Visual field loss is difficult
to exclude with
confrontation visual field
testing. Consequently,
formal perimetry is
mandatory in the evaluation
and monitoring of idiopathic
intracranial hypertension.
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(including visual acuity and color vision) is usually spared until late in the
disease course, although a small percentage of patients have a central visual
field defect at presentation, usually due to retinal pathology, such as retinal
fluid or folds.20
Pulse-synchronous (pulsatile) tinnitus occurs in about 52% to 60% of patients.8,17
It may not be spontaneously reported; therefore, patients must be specifically asked
about its presence. Pulse-synchronous tinnitus can be unilateral or bilateral.21 While
it is frequently intermittent, it can also be continuous.21 Since it can often be
decreased with ipsilateral jugular compression and often resolves following
stenting of transverse venous sinus stenoses, it likely arises because of turbulent
blood flow across stenoses in the transverse venous sinuses.22,23
Other, less common, symptoms in IIH include diplopia due to unilateral or
bilateral sixth nerve palsy, usually with moderate to severe disease. Occasional
patients have facial weakness at presentation, although this is not common and
should prompt a thorough workup for alternative diagnoses.24 Of note, up to 25%
of patients are asymptomatic, with their papilledema being discovered during a
routine eye examination.25
Signs
Papilledema (optic disc edema secondary to increased intracranial pressure) is
the most common and important sign in IIH. It is usually bilateral and
symmetric, although occasional patients have highly asymmetric papilledema.26,27
Papilledema is a result of axoplasmic flow stasis secondary to increased intracranial
pressure, producing edema of the retinal nerve fibers emanating from the optic
disc. The threat of vision loss is correlated with the severity of papilledema.16,26
Thus, it is important to determine the severity of papilledema to help guide
management. The severity of papilledema can be graded based on the appearance
of the optic disc using the modified Frisén scale (FIGURE 5-1): grade I (minimal
papilledema) is characterized by a C-shaped halo with sparing of the temporal
margin of the optic disc; grade II (mild papilledema) is characterized by a
circumferential halo; grade III (moderate papilledema) is characterized by
obscuration of at least one segment of a major blood vessel leaving the optic disc;
grade IV (marked papilledema) is characterized by total obscuration of a segment
of a major blood vessel on the optic disc; and grade V (severe papilledema) is
characterized by total obscuration of all blood vessels on and leaving the
optic disc.28,29
Hemorrhages in the peripapillary retinal nerve fiber layer commonly occur
in association with papilledema (FIGURE 5-2A) and are correlated with the
severity of papilledema.30 Subretinal hemorrhages can occur in association
with papilledema (FIGURE 5-2B). Since they can also occur with pseudopapilledema,
they do not help to distinguish papilledema from pseudopapilledema
(TABLE 5-2).30,31 In rare cases, subretinal hemorrhage can result from peripapillary
choroidal neovascularization (FIGURE 5-2C).32 Retinal folds can often be detected
with careful observation; the folds may be circumferential around the optic disc
(Paton lines or peripapillary wrinkles [FIGURE 5-3A]) or radial with extension into
the macula (FIGURE 5-3B).33 Cotton wool spots (ie, retinal nerve fiber layer
infarcts) and retinal exudates can also be present, especially in patients with
more severe grades of papilledema (FIGURE 5-4A).30 Pseudodrusen are small
white refractile deposits overlying the optic disc that can develop in patients
with long-standing papilledema (FIGURE 5-4B).30,34 Pseudodrusen must be
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FIGURE 5-1
Appearance of papilledema of increasing severity, graded using the modified Frisén scale,
from grade 0 (no papilledema) through to grade V (severe papilledema). The major features
of each grade are described in the text.

distinguished from optic disc drusen, which are larger yellow refractile bodies
arising from the substance of the optic disc.
If untreated, papilledema can result in progressive and irreversible vision
loss with optic atrophy.17,19 Since the vision loss is typically slow and insidious,
it may not be appreciated by the patient. However, it can be rapidly progressive
in patients with a fulminant presentation, resulting in early and sometimes
irreversible central vision loss.35
Visual field defects are often difficult to exclude with confrontation visual
field testing. Consequently, formal perimetry (visual field testing) is mandatory
in the evaluation and monitoring of patients with IIH.17,19 Automated perimetry
(eg, Humphrey visual field testing using the 24-2 or 30-2 SITA [Swedish
Interactive Threshold Algorithm]-standard protocols) is usually adequate for
patients who have minimal to moderate visual field loss. Automated perimetry
is quantitative and compares the patient’s responses to those of age-matched
controls. The sensitivities at each test location are expressed in decibels. The
total deviation plot shows the difference (in decibels) between the patient’s
sensitivities and those of age-matched controls at each test location, whereas the
pattern deviation plot shows the patient’s sensitivities adjusted for generalized
depression of the entire visual field (eg, due to refractive error or media
opacities, such as cataract). The mean deviation is a measure (in decibels) of the

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IDIOPATHIC INTRACRANIAL HYPERTENSION

average deviation of all test

locations compared to
age-matched controls. Patients
with a normal visual field will
usually have a mean deviation
greater than –2 dB. Patients
with mild papilledema (less
than grade II) might have
no visual field defects on
automated perimetry
(FIGURE 5-5A). An enlarged
physiologic blind spot is the
first visual field defect to
develop, producing a slight
decrease in mean deviation
(FIGURE 5-5B). The enlarged
blind spot is a refractive
scotoma resulting from
peripapillary hyperopia.36 With
increasing severity and
duration of papilledema, FIGURE 5-2
arcuate visual field defects can Peripapillary hemorrhages occurring in association
with papilledema include flame-shaped
develop, initially in the retinal nerve fiber layer hemorrhages (A) and
inferonasal portion of the subretinal hemorrhages (B). Occasionally,
visual field (FIGURE 5-5C).37 extensive subretinal hemorrhage may be seen
With more severe or secondary to peripapillary choroidal
neovascularization (C).

TABLE 5-2 Differentiation of Papilledema From Pseudopapilledemaa

Clinical Feature Papilledema Pseudopapilledema

Transient visual obscurations Yes Sometimes

Visual field defects Yes Sometimes

Spontaneous venous pulsations No Yes

Changing optic disc appearance Yes No

Obscuration of vessels Yes No

Anomalous vascular branching No Sometimes

Hemorrhages Yes (usually retinal nerve fiber layer, Occasionally (subretinal)

but occasionally subretinal)

Preserved physiologic cup Yes (until late) No

Retinal folds Often No

Leakage on fluorescein angiogram Yes (if moderate-severe) No

Symptoms of increased intracranial pressure Often No

a
Modified with permission from Thurtell MJ, & Tomsak RL.1 © 2019 Oxford University Press.

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FIGURE 5-3
Peripapillary retinal folds occurring in association with papilledema include circumferential
folds around the optic disc (A, arrowheads) and radial folds extending into the macula
(B, arrows).

long-standing papilledema, the visual field becomes progressively constricted,

with sparing of the central visual field until late.37 Nonphysiologic visual field
constriction can occur in patients with coexisting organic visual field loss;
such constriction can also result from a poor performance in a patient having
difficulty concentrating or staying awake during the test, giving a
characteristic cloverleaf appearance on automated perimetry (FIGURE 5-5D).38
Manual perimetry, such as kinetic perimetry using the Goldmann perimeter,
may give more reliable results in patients who have severe visual field
constriction or difficulties with performance on automated perimetry.

FIGURE 5-4
Cotton wool spots (retinal nerve fiber layer infarcts) and retinal exudates can develop with
more severe degrees of papilledema (A). Small white refractile deposits overlying the optic
disc, known as pseudodrusen, can occasionally develop with chronic severe papilledema (B).

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IDIOPATHIC INTRACRANIAL HYPERTENSION

FIGURE 5-5
Formal perimetry, obtained using the Humphrey 24-2 SITA-standard protocol in these
examples, is mandatory in the evaluation and monitoring of patients with idiopathic
intracranial hypertension. Patients with mild papilledema can have a normal visual field (A).
However, with increasing severity and duration of papilledema, patients will develop an
enlarged physiologic blind spot (B), arcuate visual field defects (C), and ultimately
generalized constriction with sparing of central vision. Patients who struggle with perimetry
testing (eg, difficulty concentrating or staying awake during the test) often have a cloverleaf
pattern of constriction on automated perimetry (D).

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 Other examination findings in IIH include unilateral or bilateral sixth nerve KEY POINTS
palsy causing an esotropia with limitation of abduction, although other ocular
● An enlarged physiologic
motility deficits (eg, third nerve palsy, fourth nerve palsy, and skew deviation) blind spot is the first visual
can rarely occur.39,40 Occasional patients have a facial nerve palsy at field defect to develop in
presentation.24 idiopathic intracranial
Rare patients may have normal optic discs (ie, no papilledema) but have hypertension, followed by
arcuate visual field defects
symptoms and imaging findings suggesting increased intracranial pressure as
(initially in the inferonasal
well as an increased CSF opening pressure; this controversial entity is known as visual field) and,
IIH without papilledema.41,42 It has been proposed that papilledema might not subsequently, progressive
develop in such cases because of anatomic compartmentalization of the constriction with sparing of
central vision until late.
subarachnoid space around the optic nerve stopping the CSF pressure gradient
from reaching the retrolaminar portion of the optic nerve.43 Another possibility is ● Sixth and seventh nerve
that papilledema might not develop or could resolve because of the presence of a palsies can occur as false
CSF leak (eg, causing CSF rhinorrhea or otorrhea) helping to decrease the localizing signs in patients
intracranial pressure in a patient with IIH. When papilledema is absent and no with idiopathic intracranial
hypertension.
damage to the optic nerve from resolved papilledema is evident (ie, no optic
atrophy or evidence of structural damage to the optic nerve on the basis of optical ● Ophthalmic investigations
coherence tomography [OCT]), visual function should be normal; the presence are necessary to determine
of visual field defects should raise concern for nonorganic vision loss.42 the severity of vision loss
and papilledema. In
patients with equivocal
INVESTIGATIONS papilledema or possible
When evaluating a patient with presumed IIH, further investigations are obtained pseudopapilledema,
for two broad purposes. First, neuroimaging and CSF evaluation are required to consultation with an
ophthalmologist or, ideally,
exclude other etiologies of increased intracranial pressure (TABLE 5-1). Second,
a neuro-ophthalmologist is
ophthalmic investigations should be obtained to determine the severity of vision suggested.
loss and papilledema to help guide management. However, before further
investigations are obtained, other etiologies of optic disc edema and conditions ● In patients with an
that mimic optic disc edema (eg, optic disc drusen) should be considered. atypical or fulminant
presentation of idiopathic
Differentiation of papilledema from pseudopapilledema can be challenging; a intracranial hypertension,
distinction can usually be made based on clinical and investigation findings magnetic resonance
(TABLE 5-2). However, it is important to keep in mind that occasional patients venography of the head with
have papilledema that is superimposed on pseudopapilledema. Consultation with contrast should be obtained
to exclude cerebral venous
an ophthalmologist or neuro-ophthalmologist is suggested for patients with sinus thrombosis.
equivocal papilledema or pseudopapilledema, or when another etiology for optic
disc edema is suspected. Specialized ophthalmic investigations (eg, fundus
autofluorescence, ultrasonography, and OCT) are often needed for definitive
diagnosis of optic disc drusen (FIGURE 5-6).

Neuroimaging
Neuroimaging is the first step in the evaluation of a patient with increased
intracranial pressure. Most structural causes of increased intracranial pressure
can be identified on MRI of the brain with contrast. However, magnetic resonance
venography (MRV) of the head with contrast should also be obtained to ensure
that cerebral venous sinus thrombosis is excluded, especially in patients with an
atypical or fulminant presentation for IIH (CASE 5-1).44
Several somewhat subtle findings on neuroimaging can suggest increased
intracranial pressure. An empty sella turcica is a common finding (FIGURE 5-7A)
but can also be present in the absence of increased intracranial pressure.45
Dilation and increased tortuosity of the optic nerve sheaths may be seen as
well as posterior globe flattening (FIGURE 5-7B).45 Occasionally, the swollen

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IDIOPATHIC INTRACRANIAL HYPERTENSION

KEY POINTS

● Common imaging findings

in idiopathic intracranial
hypertension include an
empty sella turcica,
increased optic nerve
sheath dilation and
tortuosity, posterior globe
flattening, optic disc
elevation and enhancement,
inferior cerebellar tonsillar
descent, and transverse
venous sinus stenosis.

● In adults, a CSF opening

pressure of greater than
25 cm H2O is high, while an
opening pressure of 20 cm
H2O to 25 cm H2O is
probably abnormal if
symptoms, signs, and
imaging findings are
consistent with increased
intracranial pressure. In
children, recent studies
suggest that a CSF opening
pressure of greater than
28 cm H2O is high.

FIGURE 5-6
Optic disc drusen can be mistaken for papilledema. With buried optic disc drusen (A), the
optic disc drusen are located beneath the surface of the disc and are not visible on funduscopic
examination; the optic disc is often elevated and can have an appearance that can be
difficult to distinguish from mild papilledema. When optic disc drusen become exposed,
they are yellow in color and refractile, with a “rock candy” appearance (B). Since exposed
optic disc drusen display autofluorescence, they are often prominent on fundus
autofluorescence (C). Buried optic disc drusen may not be visible on fundus autofluorescence
but can usually be detected on ultrasonography (D) as a focus of increased reflectivity
within the elevated optic nerve head (arrowheads) with a characteristic posterior reduplication
artifact (arrows).

optic discs may be visible and enhancing (FIGURE 5-7C).45 In some patients,
acquired cerebellar tonsillar descent below the level of the foramen magnum
is seen; this can be mistaken for a (congenital) Chiari malformation
(FIGURE 5-7A).46
MRV of the head often shows smoothly tapered stenoses in the transverse
venous sinuses (FIGURE 5-8).47 These are thought to result from mechanical
compression of the venous sinus in the setting of increased intracranial pressure.48
Less commonly, stenoses can result from intrinsic factors, such as arachnoid
granulations, septations, and organized thrombus. Catheter venography with
manometry often shows a pressure gradient across these stenoses, with increased
venous pressures in the superior sagittal sinus and transverse venous sinuses
proximal to the stenoses.49 The stenoses might play a role in the pathogenesis of

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IIH or exacerbate it. Thus, transverse venous sinus stenting has been proposed as
a potential surgical treatment for the disease.22,50

CSF Evaluation
The lumbar puncture has a dual role in the diagnosis of IIH. First, it is obtained
to confirm the presence of an increased CSF opening pressure. Second,
evaluation of the CSF constituents is required to exclude other etiologies of
increased intracranial pressure (eg, infectious, inflammatory, or neoplastic
meningitis).
Ideally, the lumbar puncture should be obtained with the patient positioned in
the left lateral recumbent position. The CSF opening pressure should be
measured with the legs extended, head in a neutral position, and the patient
breathing normally. The normal CSF opening pressure in adults is 10 cm H2O to
20 cm H2O. A CSF opening pressure of greater than 25 cm H2O is considered
high, whereas a pressure of 20 cm H2O to 25 cm H2O is considered borderline,
although probably abnormal in a patient who has symptoms, signs, and
neuroimaging findings suggesting increased intracranial pressure. Recent
studies have found that the normal range for CSF opening pressure in children

A 23-year-old woman with a normal body mass index had a motor vehicle CASE 5-1
accident resulting in a head injury without loss of consciousness. She
subsequently developed severe headaches, transient visual obscurations,
and pulse-synchronous tinnitus. Her eye care provider noted bilateral
papilledema. MRI of her brain with contrast was reported to be
unremarkable. Subsequent lumbar puncture showed a CSF opening
pressure of 32 cm H2O with normal CSF constituents. Thus, she was
diagnosed with idiopathic intracranial hypertension and started on
acetazolamide 1000 mg 2 times a day.
She presented for a second opinion because of worsening of her
papilledema on treatment. On examination, her visual acuity was 20/15 in
both eyes. Her pupils were equal and briskly reactive without a relative
afferent pupillary defect. Ocular motility was normal. Funduscopic
examination showed grade IV optic disc edema in the right eye and grade
III optic disc edema in the left eye. Visual fields showed an enlarged blind
spot in both eyes. Review of her previous MRI was unrevealing. However,
a repeat MRI of her brain with contrast and magnetic resonance
venography (MRV) of her head with contrast showed superior sagittal
venous sinus thrombosis with left parietal venous infarction.
She was admitted for anticoagulation, and her acetazolamide dose
was increased to 1500 mg 2 times a day. Her symptoms and signs
eventually resolved, and the acetazolamide dose was gradually
decreased over months.

This case highlights the importance of considering cerebral venous sinus COMMENT
thrombosis in a patient with increased intracranial pressure but an atypical
presentation for idiopathic intracranial hypertension.

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IDIOPATHIC INTRACRANIAL HYPERTENSION

FIGURE 5-7
MRI findings suggesting increased intracranial pressure. A, Sagittal T1-weighted MRI showing
an empty sella turcica with mild inferior cerebellar tonsillar descent. B, Axial T2-weighted
MRI showing dilated and tortuous optic nerve sheaths with posterior globe flattening.
C, Axial T1-weighted postcontrast MRI showing enhancing optic discs (arrowheads).

is higher than in adults; less than 28 cm H2O is considered normal in children.51

The CSF opening pressure can be influenced by a number of factors, such as
incorrect positioning of the patient or manometer during the opening pressure
measurement and use of sedation during the procedure; in children who
receive minimal or no sedation, less than 25 cm H2O is considered normal.51
The CSF constituents should be normal (ie, normal white cell count with
normal protein and glucose concentrations) in patients with IIH. The presence of
an increased white cell count or protein concentration should raise concern for
another etiology of increased intracranial pressure.

Ophthalmic Investigations
Formal perimetry is mandatory for evaluation and monitoring of patients with
IIH (as discussed earlier). Other investigations, such as fundus autofluorescence
and ultrasonography, can be helpful in the evaluation of suspected
pseudopapilledema. OCT may have a role in quantifying the severity of
papilledema (FIGURE 5-9); the retinal nerve fiber layer thickness correlates well
with papilledema severity based on the modified Frisén scale, especially for lower
grades of papilledema.29 However, OCT measures of retinal nerve fiber layer
thickness must be interpreted with caution, since combined retinal nerve fiber
layer edema and atrophy might give a retinal nerve fiber layer thickness that
appears to be close to normal despite significant visual field loss from optic nerve
damage. In such cases, OCT might show thinning of the retinal ganglion cell and
inner plexiform layer complex (containing the cell bodies for retinal nerve
fibers), which correlates well with the severity of vision loss secondary to optic
nerve damage.20 Finally, high-resolution raster scans obtained through the optic

1300 OCTOBER 2019

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 KEY POINTS

● Retinal nerve fiber layer

thickness from optical
coherence tomography
correlates with papilledema
severity. However, retinal
nerve fiber layer thickness
measurements must be
interpreted with caution in
patients who could have
combined optic disc edema
and atrophy.

● Raster scans obtained

through the optic nerve
head with optical coherence
tomography may show
biomechanical changes that
correlate with increased
intracranial pressure and
FIGURE 5-8
might be useful for
Lateral (A) and frontal (B) reconstructions of magnetic resonance venography (MRV) of the
monitoring response to
head with contrast demonstrating bilateral transverse venous sinus stenoses (arrowheads).
treatment.

● Several medications (eg,

tetracycline antibiotics,
nerve head using OCT can demonstrate biomechanical changes that correlate retinoids, and lithium) and
well with increased intracranial pressure; an inward deflection of the cerebral venous outflow
peripapillary retinal pigment epithelium and Bruch membrane complex toward obstruction (eg, due to
cerebral venous sinus
the vitreous of the eye (FIGURE 5-10) appears to reverse with a decrease in thrombosis) can cause a
intracranial pressure.52,53 clinical syndrome that
mimics idiopathic
intracranial hypertension.
DIFFERENTIAL DIAGNOSIS
Several etiologies of increased intracranial pressure can mimic IIH and,
therefore, must be specifically considered. Several medications are associated
with a clinical syndrome that mimics IIH, although they might also precipitate
or worsen preexisting IIH. These medications include the tetracycline
antibiotics (eg, minocycline), retinoids (eg, vitamin A derivatives and
all-trans retinoic acid), and lithium. Corticosteroid withdrawal has also
been reported to cause rebound intracranial hypertension. Thus, a thorough
review of medication use is mandatory in the evaluation of a patient with
suspected IIH (CASE 5-2).
Cerebral venous hypertension due to cerebral venous sinus thrombosis,
extrinsic venous sinus compression (eg, by a meningioma), or arterialization of
the sinus by a dural arteriovenous fistula can cause a clinical syndrome that
mimics IIH.44,54,55 Features suggesting cerebral venous sinus thrombosis are
listed in TABLE 5-3. When cerebral venous sinus thrombosis is suspected, MRV of
the head with contrast should be obtained (CASE 5-1).

MANAGEMENT
The two main goals of treatment are to preserve visual function and alleviate
symptoms. Many treatment approaches have been proposed for IIH, including
lifestyle interventions (weight loss), medical therapies, and surgical
interventions.

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IDIOPATHIC INTRACRANIAL HYPERTENSION
KEY POINT
● Weight loss of 6% to 10%
of initial body weight can be
effective in inducing a
remission of idiopathic
intracranial hypertension.
Bariatric surgery can be
effective in patients who are
morbidly obese and struggle
to lose weight.
FIGURE 5-9
Optical coherence tomography showing diffuse
retinal nerve fiber layer (RNFL) edema in a patient
with grade II papilledema (A). The peripapillary
RNFL thickness in micrometers (μm) is determined
after segmentation of the retinal layers (B, purple
circle). The peripapillary RNFL thickness of the
right eye (OD, solid line) and left eye (OS, dashed
line) can be plotted and compared to an
age-matched normal dataset (C, shaded green
area indicates the 95% confidence limits of RNFL
thickness for the age-matched normal dataset).
The RNFL quadrant analysis indicates the average
RNFL thickness for the superior (S), nasal (N),
inferior (I), and temporal (T) quadrants (D). The
average RNFL thickness for this patient was 218 μm
for the right eye and 177 μm for the left eye (normal
range is about 80 to 100 μm).
INF = inferior; NAS = nasal; SUP = superior; TEMP =
temporal.
1302
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Weight Loss
Studies suggest that weight loss
of about 6% to 10% of initial
body weight is adequate to
induce remission in most
patients with IIH. In a 2010
prospective cohort study, a
low-calorie diet resulted in a
significant reduction in CSF
opening pressure, papilledema,
and headache disability based on
the Headache Impact Test-6
score; participants lost an
average of about 15.5 kg (34 lb).56
While effective in the long term,
weight loss is not a practical or
effective treatment in the short
term; other treatments must be
initiated in parallel for most
patients with IIH. Of note,
bariatric surgery is an option for
patients who are morbidly obese
whose weight loss attempts have
been unsuccessful, although
visual outcomes from bariatric
surgery have not been studied
in detail.57
Medical Therapy
Carbonic anhydrase inhibitors,
such as acetazolamide and
methazolamide, are the mainstay
of medical therapy for IIH. These
drugs are thought to decrease
CSF production, although they
do have a mild diuretic effect.
The 2014 Idiopathic Intracranial
Hypertension Treatment Trial
was a double-masked
randomized controlled trial of
diet plus placebo versus diet
plus maximally tolerated
acetazolamide in patients with
newly diagnosed IIH and mild
vision loss (mean deviation of
–2 dB to –7 dB).58 The
acetazolamide dose was titrated
up, as tolerated, to a maximum
of 2000 mg 2 times a day. The
primary outcome measure was
OCTOBER 2019
FIGURE 5-10
High-resolution raster scans obtained using optical coherence tomography through the
optic nerve head (A, blue line) can show inward deflection of the retinal pigment epithelium
and Bruch membrane complex toward the vitreous cavity (B, arrowheads) when increased
intracranial pressure is present.

A 15-year-old girl presented with headaches and transient visual CASE 5-2
obscurations in both eyes. She reported weight gain of 11.3 kg (25 lb) over
12 months. Her local eye care provider noted bilateral papilledema and
referred her to a pediatric neurologist for further evaluation. MRI of her
brain with contrast and magnetic resonance venography (MRV) of her
head with contrast showed signs suggesting increased intracranial
pressure but no cause for it. Subsequent lumbar puncture showed a CSF
opening pressure of 27 cm H2O with normal CSF constituents. Based on
these findings, she was felt to have idiopathic intracranial hypertension
and was started on acetazolamide 500 mg 2 times a day.
The patient’s parents requested a second opinion regarding the
diagnosis. At the time of evaluation, the patient’s visual acuity was 20/20
in both eyes. Her pupils were equal and briskly reactive without a relative
afferent pupillary defect. Ocular motility was normal. Funduscopic
examination showed grade II optic disc edema in both eyes. Visual fields
showed an enlarged blind spot in both eyes.
Further history revealed that the patient had been started on
doxycycline for acne 1 month before the onset of her symptoms. The
doxycycline was discontinued. Acetazolamide was continued until her
symptoms and signs had fully resolved. The patient had no recurrence of
symptoms or signs after the acetazolamide was discontinued.

This case highlights the importance of a thorough review of medication use COMMENT
in the evaluation of a patient with suspected idiopathic intracranial
hypertension.

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IDIOPATHIC INTRACRANIAL HYPERTENSION

change in mean deviation (from Humphrey 24-2 SITA-standard perimetry).

Secondary outcome measures included changes in papilledema grade,
symptoms, quality of life, and weight. Treatment with acetazolamide was
associated with statistically significant improvements in mean deviation,
papilledema grade, symptoms, and quality of life.58 Of note, participants in the
acetazolamide group lost more weight than those in the placebo group.58
Acetazolamide was well tolerated by most participants, although common side
effects included paresthesia, dysgeusia, nausea, vomiting, and diarrhea.59 The
risk factors for treatment failure included male sex, higher papilledema grade
(ie, grades III–V), decreased visual acuity at presentation, greater than 30
transient visual obscurations per month, and peripapillary retinal nerve fiber
layer hemorrhages at presentation, suggesting that such patients require closer
monitoring and may need more aggressive treatment.16,30 While the
acetazolamide dose was increased to a maximum of 2000 mg 2 times a day,
most patients with IIH and mild vision loss seem to respond well to doses of
500 mg to 1000 mg 2 times a day. The optimum acetazolamide dose for
patients with moderate to severe vision loss at presentation remains unclear,
although many clinicians rapidly titrate up to high doses (eg, 1500 mg to
2000 mg 2 times a day), as tolerated, before considering surgical interventions.
Topiramate is often used for treatment of primary headache disorders, such
as migraine. It is also a weak carbonic anhydrase inhibitor that seems to have
similar efficacy to acetazolamide in treating patients with mild to moderate
IIH.60 Since topiramate often causes some weight loss, it can be considered when
acetazolamide cannot be tolerated or when headache is prominent. The dose of
topiramate required for a therapeutic response has not been specifically studied.
However, many patients appear to respond to low doses of 25 mg/d to 50 mg/d,
although the dose can be titrated up to 100 mg 2 times a day for improved
symptom control. Topiramate seems to be better tolerated than acetazolamide,
although common side effects include mental slowing, lethargy, paresthesia, and
decreased appetite. Other important, but less common, side effects include renal
stones and acute angle-closure glaucoma.
Other diuretics, such as furosemide, can be administered alone or in combination
with other medications for a synergistic effect.61 However, monitoring of

TABLE 5-3 Clinical Features Suggesting Cerebral Venous Sinus Thrombosisa

◆ Nonobese
◆ Pregnant or postpartum
◆ Acute or fulminant presentation
◆ History of clotting or thrombophilia (eg, deep venous thrombosis, pulmonary embolus)
◆ History of connective tissue disease (eg, systemic lupus erythematosus, Behçet disease)
◆ History of recent ear, mastoid, or sinus infection
◆ History of recent head or neck surgery or trauma
◆ CSF abnormalities (eg, raised protein, pleocytosis)

a
Modified with permission from Thurtell MJ, Tomsak RL.1 © 2019 Oxford University Press.

1304 OCTOBER 2019

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electrolytes and potassium supplementation is required when furosemide is KEY POINTS
used. Corticosteroids were used for treatment in the past but produce
● Treatment of idiopathic
undesirable long-term complications, such as weight gain. Furthermore, intracranial hypertension
corticosteroid withdrawal can result in a rebound increase in intracranial with acetazolamide
pressure. High-dose IV corticosteroids are sometimes used for the short-term produces improvement in
treatment of patients who have a fulminant presentation while awaiting visual field loss,
papilledema, symptoms,
definitive surgical intervention (eg, CSF shunting).35,62
and quality of life. Common
side effects of
Surgical Therapies acetazolamide therapy
Surgical intervention is often required for patients with a fulminant presentation include paresthesia,
dysgeusia, nausea, vomiting,
of IIH but may also be needed in those who fail to improve or worsen despite
and diarrhea.
maximally tolerated medical therapy. The three most commonly used
interventions are CSF shunting, optic nerve sheath fenestration, and transverse ● Topiramate is effective in
venous sinus stenting.63 treatment of mild to
CSF shunting is very effective for rapidly reducing intracranial pressure moderate idiopathic
intracranial hypertension
and papilledema. Stereotactic ventriculoperitoneal shunting is preferred over and can be considered in
lumboperitoneal shunting because of its lower complication rate.64 Incorporation patients who are unable to
of an adjustable valve into the shunt apparatus allows the CSF flow rate to be tolerate acetazolamide or
adjusted according to symptoms and signs. Unfortunately, shunting has a when headache is
prominent. Common side
significant complication rate, including infection, obstruction, and migration of effects of topiramate
shunt tubing.63 Consequently, shunt revisions are often needed. Given the therapy include mental
potential for complications and need for revision, CSF shunting should not be slowing, lethargy,
considered for the management of isolated intractable headache unless the paresthesia, and loss of
appetite.
headache is known to respond to decreases in intracranial pressure (eg, following
a lumbar puncture) and noninvasive management options have been ineffective. ● Surgical therapies are
One large retrospective study found that headache initially improved in most usually reserved for patients
patients with IIH following CSF shunting, but almost 50% had recurrent with idiopathic intracranial
headaches at 36 months following CSF shunting.65 hypertension who have a
fulminant presentation and
Optic nerve sheath fenestration is an effective intervention to consider for patients who fail to
when vision is threatened. A superior or medial orbital approach is used to improve or worsen despite
create slits or a window in the retrolaminar optic nerve sheath, thereby creating maximally tolerated medical
a fistula between the subarachnoid space and orbital cavity. The resultant therapy.
decrease in pressure on the optic nerve results in reduced papilledema with ● CSF shunting is effective
improved visual function.63 In some patients, unilateral optic nerve sheath for rapidly reducing
fenestration improves the papilledema and visual function on the contralateral intracranial pressure.
side, but many patients will require bilateral sequential optic nerve sheath Complications can include
infection, obstruction,
fenestrations. Complications of optic nerve sheath fenestration include transient
and migration of shunt
or persistent vision loss (eg, from optic nerve trauma), tonic pupil (eg, from tubing; shunt revision is
damage to the ciliary ganglion or postganglionic parasympathetic fibers), often needed.
and diplopia.63
Transverse venous sinus stenting is a surgical intervention to consider in ● Optic nerve sheath
fenestration is effective in
patients who have transverse venous sinus stenoses with pressure gradients relieving pressure on the
(>8 mm Hg) across the stenoses and increased venous pressures in the optic nerve, thereby
superior sagittal sinus and venous sinuses proximal to the stenoses.22,50 It reducing papilledema and
has been proposed that stenting will reduce cerebral venous hypertension, improving visual function.
Complications can include
resulting in increased CSF absorption, reduced intracranial hypertension, and, vision loss, tonic pupil,
thus, improved symptoms and signs. Accordingly, several retrospective and diplopia.
and prospective studies have reported improvement in symptoms, signs,
visual function, and intracranial pressure.22,50,66 Potential complications of
transverse venous sinus stenting include in-stent thrombosis and subdural

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IDIOPATHIC INTRACRANIAL HYPERTENSION
KEY POINTS
● Transverse venous sinus
stenting has been reported
to improve symptoms, signs,
visual function, and
intracranial pressure.
Complications can include
in-stent thrombosis,
subdural hemorrhage, and
development of new
stenoses proximal to
the stent.
● The indications for
surgical intervention in
idiopathic intracranial
hypertension, the timing and
choice of surgical
intervention, and long-term
outcomes remain unclear.
● The main goals of
treatment of idiopathic
intracranial hypertension are
to preserve vision and
alleviate symptoms. Thus,
the management is tailored
depending on the severity of
vision loss, papilledema,
and symptoms as well as the
patient’s response to
medical therapy and ability
to tolerate medical therapy.
● Patients with idiopathic
intracranial hypertension
with minimal to mild vision
loss can usually be managed
with weight loss and medical
therapy, whereas patients
with moderate to severe
vision loss often need a
combination of weight loss,
aggressive medical therapy,
and, occasionally, surgical
intervention.
● Patients with idiopathic
intracranial hypertension
should be managed in
coordination with an
ophthalmologist or
neuro-ophthalmologist,
since formal perimetry and
monitoring of papilledema
severity is needed to guide
management.
1306
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hemorrhage as well as development of recurrent stenoses immediately
proximal to the stent.22,50,63
The choice of surgical intervention remains controversial and often varies
depending on local resources or practices. However, the patient’s symptoms
and signs should be considered in the decision-making process. For example, a
patient who has papilledema and vision loss without other symptoms and signs
of increased intracranial pressure might be best treated with an optic nerve
sheath fenestration, whereas a patient with severe symptoms (eg, headache),
papilledema with vision loss, and other signs (eg, sixth nerve palsy) might be
best treated with CSF shunting. Given the controversy with regard to choice
and timing of surgical intervention for IIH, the National Eye Institute of the
National Institutes of Health (NIH) has sponsored a multicenter, randomized,
single-masked clinical trial comparing maximal medical therapy versus maximal
medical therapy plus optic nerve sheath fenestration versus maximal medical
therapy plus CSF shunting for management of patients with IIH and moderate to
severe vision loss at initial presentation.67 This trial, called the SIGHT (Surgical
Idiopathic Intracranial Hypertension Treatment) trial, will evaluate short- and
long-term outcomes of these therapies, with the primary outcome being change
in mean deviation on automated perimetry. Other outcomes will include time to
treatment failure and change in CSF opening pressure, papilledema grade,
quality of life, and headache disability.67
Summary of Management Approach
The management approach for individual patients with IIH depends on the
severity of their vision loss based on formal perimetry, severity of papilledema
based on Frisén grade, severity of symptoms, response to medical therapy, and
ability to tolerate medical therapy. Patients with minimal vision loss (mean
deviation better than –3 dB) can often be managed with weight loss alone
(low-calorie and low-sodium diet plus exercise), although medical therapy can
be added depending on the severity of symptoms and response to weight-loss
attempts. Patients with mild vision loss (mean deviation of –3 dB to –7 dB)
can usually be managed with weight loss plus medical therapy. Patients with
moderate vision loss (mean deviation of –7 dB to –15 dB) can often be managed
with weight loss plus more aggressive medical therapy, although surgical
intervention could be considered depending on the response to weight loss and
medical therapy. Patients with severe vision loss (mean deviation worse than
–15 dB) often require a combination of weight loss plus aggressive medical
therapy plus surgical intervention, although the timing and choice of surgical
intervention remains controversial.
Patients with IIH require long-term monitoring, since this is a chronic disease
prone to relapses in association with weight gain. The severity of vision loss,
papilledema, and symptoms influence treatment decisions. Comanagement with
an ophthalmologist or neuro-ophthalmologist is crucial, with the timing of
follow-up tailored according to the severity of symptoms and signs at
presentation, response to treatment, and subsequent clinical course.
CONCLUSION
IIH is a syndrome of increased intracranial pressure of unclear etiology that
most often occurs in obese women of childbearing age. Recent studies have
OCTOBER 2019
found that the annual incidence of IIH is increasing in parallel with obesity
rates. Common symptoms of IIH include headache, transient visual obscurations,
and pulse-synchronous (pulsatile) tinnitus. Papilledema is the most common and
important clinical sign. If untreated, it can result in progressive and irreversible
vision loss with optic atrophy, underscoring the importance of funduscopic
examination and formal visual field testing (perimetry) for the monitoring of IIH.
Management options include weight loss, medical therapy (eg, acetazolamide or
topiramate), and surgical interventions (eg, CSF shunting, optic nerve sheath
fenestration, or transverse venous sinus stenting). The management approach
should be tailored for each patient according to the severity of vision loss,
severity of papilledema, severity of symptoms, response to medical therapy,
and ability to tolerate medical therapy.

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