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Is Pseudotumor Cerebri An Unusual Expression of Chiari Syndrome? A Case

Report and Review of the Literature

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Neuro and Spine Surgery

SURGICAL TECHNOLOGY INTERNATIONAL Volume 30

Is Pseudotumor Cerebri An Unusual

Expression of Chiari Syndrome? A Case
Report and Review of the Literature
PAOLO PACCA, MD DIEGO GARBOSSA, MD
NEUROSURGEON NEUROSURGEON
DIVISION OF NEUROSURGERY DIVISION OF NEUROSURGERY
DEPARTMENT OF NEUROSCIENCE DEPARTMENT OF NEUROSCIENCE
UNIVERSITY OF TORINO UNIVERSITY OF TORINO
TORINO, ITALY TORINO, ITALY

ROBERTO ALTIERI, MD ALESSANDRO DUCATI, MD

RESIDENT PROFESSOR OF NEUROSURGERY
DIVISION OF NEUROSURGERY DIRECTOR OF NEUROSURGERY UNIT
DEPARTMENT OF NEUROSCIENCE DIVISION OF NEUROSURGERY
UNIVERSITY OF TORINO DEPARTMENT OF NEUROSCIENCE
UNIVERSITY OF TORINO
TORINO, ITALY
TORINO, ITALY
FRANCESCO ZENGA, MD MICHELE LANOTTE, MD
NEUROSURGEON PROFESSOR OF NEUROSURGERY
DIVISION OF NEUROSURGERY DIVISION OF NEUROSURGERY
DEPARTMENT OF NEUROSCIENCE DEPARTMENT OF NEUROSCIENCE
UNIVERSITY OF TORINO UNIVERSITY OF TORINO
TORINO, ITALY TORINO, ITALY

ABSTRACT
he Chiari I malformation (CM-I) is a developmental alteration of the posterior cranial fossa (PCF), radi-

T ographically defined as the descent of the cerebellar tonsils ≥ 5 mm below the foramen magnum (FM)

inside the cervical canal. Headache is the most frequent symptom associated with CM-I. The association

of CM-I and neurological symptoms configures with Chiari syndrome. A rare symptom associated with

Chiari syndrome is intracranial hypertension syndrome with cephalea and papilloedema—the typical find-

ings of pseudotumor cerebri (PTC).

PTC is a syndrome characterized by signs and symptoms of increased intracranial pressure (ICP) in the

absence of space-occupying masses and/or obstruction of the ventricular system detectable by

neuroimaging. The most common symptoms are headache and visual disturbances.

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Is Pseudotumor Cerebri An Unusual Expression of Chiari Syndrome? A Case Report and Review of the Literature
PACCA/ALTIERI/ZENGA/GARBOSSA/DUCATI/LANOTTE

Literature reports that the association between CM-I and PTC has a prevalence of 2–6%. More recently, a

prevalence of 11% has been described in a specific subset of obese or overweight female patients between 20

and 40 years old.

Here we report the case of a 38-year-old woman who came to our observation with a clinical picture and

neuroradiological examinations compatible with both CM-I and PTC. We discuss the clinical case and the

significant improvement after surgical occipito-cervical decompression.

is a syndrome characterized by signs and diagnostic and therapeutic approach in

INTRODUCTION
The Chiari I malformation (CM-I) is
an alteration of posterior cranial fossa
(PCF) development, radiographically
defined as the descent of the cerebellar
tonsils ≥ 5 mm below the foramen mag-
num (FM) inside the cervical canal.1-5
Cephalea, the most frequent symptom
associated with CM-I,3 has specific fea-
tures, according to the diagnostic crite-
ria (Table I). The association of CM-I
and neurological symptoms configures
with Chiari syndrome. Intracranial
hypertension syndrome (IHS) with
cephalea and papilloedema, which are
the typical features of pseudotumor
cerebri (PTC), is rarely reported.2-6
Idiopathic IHS, also known as PTC,
symptoms of increased intracranial
pressure. 7-11 The diagnostic criteria
(Table II) are well known.
PTC may be unusually present in
patients with CM-I, and whether its
presence is random or related to
increased intracranial pressure is not yet
entirely clear.12 A study by Banik et al.
in 2006 13 showed that 8/68 PTC
patients also had CM-I and they were all
obese females between 20 and 40 years
old.
PTC and CM-I share some common-
alities, such as the higher incidence in
females and symptoms such as cephalea
and papilledema.2,3,13
Here we present a new case of an
association of CM-I and PTC in a young
female patient. We discuss the clinical-
light of a critical review of the case
studies in the literature.
Case Report
CASE REPORT
A 38-year-old female patient,
170 cm tall and 131 kg weight, came to
our attention in 2015. She suffered
from ever-worsening cephalea during
the previous year and showed papil-
loedema as well as paresthesia at the
arms and upper limbs.
Therefore, she underwent a neurora-
diological workout that showed evidence
of a 9 mm cerebellar tonsils descent
(Fig. 1a) and radiological signs compati-
ble with intracranial hypertension. The

Table I
Headache attributed to Chiari malformation type I: diagnostic criteria

A. Headache characterised by at least one of the following and fulfilling criterion D:

- precipitated by cough and/or Valsalva manoeuvre
- protracted (hours to days) occipital and/or sub-occipital headache
- associated with symptoms and/or signs of brainstem, cerebellar, and/or cervical cord dysfunction
B. Cerebellar tonsillar herniation as defined by one of the following on craniocervical MRI:
1. ≥5 mm caudal descent of the cerebellar tonsils
2. ≥3 mm caudal descent of the cerebellar tonsils plus at least one of the following indicators of crowding of the subarach-
noid space in the area of the craniocervical junction:
a) compression of the CSF spaces posterior and lateral to the cerebellum
b) reduced height of the supraocciput
c) increased slope of the tentorium
d) kinking of the medulla oblongata
C. Evidence of posterior fossa dysfunction, based on at least two of the following:
1. otoneurological symptoms and/or signs (e.g., dizziness, disequilibrium, sensations of alteration in ear pressure, hypacu-
sia or hyperacusia, vertigo, down-beat nystagmus, oscillopsia)
2. transient visual symptoms (spark photopsias, visual blurring, diplopia, or transient visual field deficits)
3. demonstration of clinical signs relevant to cervical cord, brainstem, or lower cranial nerves, or of ataxia or dysmetria
D. Headache resolves within three months after successful treatment of the Chiari malformation

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Neuro and Spine Surgery

SURGICAL TECHNOLOGY INTERNATIONAL Volume 30

Table II
Headache attributed to idiopathic intracranial hypertension: diagnostic criteria
A. Progressive headache with at least one of the following characteristics and fulfilling criteria C and D:
1. daily occurrence
2. diffuse and/or constant (non-pulsating) pain
3. aggravated by coughing or straining
B. Intracranial hypertension fulfilling the following criteria:
1. alert patient with neurological examination that either is normal or demonstrates any of the following abnormalities:
a) papilloedema
b) enlarged blind spot
c) visual field defect (progressive if untreated)
d) sixth nerve palsy
2. increased CSF pressure (>200mm H2O in the non-obese, >250mm H2O in the obese) measured by lumbar puncture in
the recumbent position or by epidural or intraventricular pressure monitoring
3. normal CSF chemistry (low CSF protein is acceptable) and cellularity
4. intracranial diseases (including venous sinus thrombosis) ruled out by appropriate investigations
5. no metabolic, toxic, or hormonal cause of intracranial hypertension
C. Headache develops in close temporal relationship to increased intracranial pressure
D. Headache improves after withdrawal of CSF to reduce pressure to 120–170mm H2O and resolves within 72 hours of per-
sistent normalisation of intracranial pressure

eye examination revealed bilateral papil- time, there was a 9 mm descent of the The relationship between CM-I and
loedema with peripapillary signs of con- cerebellar tonsils, confirming a radio- PTC is still not yet entirely clear; par-
gestion. logical clinical picture of CM-I, with ticularly the cause and the effect. Ulti-
The patient started therapy with a symptoms and signs related to Chiari mately, is there a causal relationship
high dose of thiazide diuretics (acetazo- syndrome (cephalea, dizziness, and between PTC and CM-I?
lamide 750 mg per day), but experi- paresthesia). The MRI was compatible It is known that in patients with PTC,
enced no clinical improvement. with both PTC and CM-I. increased ICP results from alterations of
The cephalea, during the last month,
became continuous and lasted through-
out the whole day. As a result, the
patient took non-steroidal anti-inflam-
matory drugs, but had no significant
improvement.
Accordingly, with these findings, we
diagnosed a Chiari syndrome and per-
formed a decompressive suboccipital
craniectomy with optimal clinical and
radiological outcome (Fig. 1b).
Six months after surgical therapy, the
patient reported clinical improvement
in cephalea symptoms, vomiting, and
visual acuity; the neurological examina-
tion was unchanged.

DiscussionDISCUSSION

Our patient presented with signs and

symptoms of PTC (cephalea, nausea,
vomiting, and visual disturbance with
papilloedema) shown by MRI (disten-
a b

sion of the lamina of the optic nerves

Figure 1. a) Magnetic resonance of the brain, weighted T1 sagittal images showing a 9 mm dislocation of

and a partial empty sella). At the same

the cerebellar tonsils below the foramen magnum. b) Six-month post-operative magnetic resonance of the
brain, weighted T1 sagittal images showing good surgical decompression and outcome.

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Is Pseudotumor Cerebri An Unusual Expression of Chiari Syndrome? A Case Report and Review of the Literature
PACCA/ALTIERI/ZENGA/GARBOSSA/DUCATI/LANOTTE
CSF absorption, likely from obstruction
of outflow at the level of the arachnoid
villi. In CM-I, several different mecha-
nisms have been implicated in the pro-
duction of increased ICP.1,2,13-15
It can be considered correct that a
patient with the clinical picture of PTC
and evidence of CM-I may have prima-
ry PTC with CM-I being secondary to
increased ICP.13 However, the opposite
is also true, pre-existing CM-I may
have caused increased ICP from
obstruction of outflow, thus producing
the clinical picture of PTC. In the first
situation, CM-I is an acquired compli-
cation of PTC, whereas in the second
one, the CM-I is a congenital malfor-
mation that produces secondary PTC.13
Several studies1,2,13-15 have tried to
assess the association and prevalence,
with results ranging from 2–11%. The
two diseases have common signs and
symptoms (i.e., cephalea, vomiting,
and papilledema).2,3,13 The class with
the highest risk, common to both
pathologies, is women between 20 and
40 years of age.8,10,11 There are, howev-
er, significant differences: obesity is
present only in cases of PTC 2,13; the
therapeutic approach, first medical
(e.g., acetazolamide) in PTC, surgery
in symptomatic CM-I (S. Chiari), with
decompression of the PCF.13,15
The combination of PTC and CM-I
is reported with higher prevalence
(11%) in the study by Banik et al.,13
which describes a homogeneous series
of patients with PTC and CM-I, all
women aged 20 to 40 years and obese.
Assuming that the pseudo-tumor was
secondary to the presence of Chiari
malformation, based on previous expe-
rience of patients with papilledema
and CM-I treated surgically with suc-
cess (resolution of papilledema),2 the
authors have suggested surgical therapy
by occipital decompression to restore
normal CSF flow.
Whilst one might consider CM-I
and PTC to be two different manifesta-
tions of the same spectrum of disease
(i.e., the alteration of intracranial pres-
sure), Bejjani put forward a different
pathogenetic hypothesis. He postulated
that the presence of cranio-cephalic
disproportion could lead to intracra-
nial hypertension, with possible herni-
ation of the cerebellar tonsils in the
foramen magnum.15
These two studies addressed the
problem from different points of view:
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#854 Pacca FINAL
the first 13 considers PTC because of
CM-I, whilst the second 15 addresses
the two diseases as a result of a com-
mon problem.
It is important to consider CM-I as
a “cofactor” that can lead to PTC when
associated with certain predisposing
factors (obesity, female, aged between
20 and 40).
In a case study by Milhorat et al.,3 on
a large series of 364 patients with a
symptomatic CM-I, only nine had
papilledema (2%). Therefore, papillede-
ma is not a common sign in CM-I
because the malformation, although
altering the homeostasis of the brain,
generally does not result in a frame-
work of intracranial hypertension. In
fact, even assuming that the causal
mechanism of increased intracranial
pressure in patients with CM-I is the
obstruction of CSF at the FM level,
only in susceptible individuals, or with
risk factors for developing intracranial
hypertension, could the presence of
CM-I decompensate the situation,
leading to intracranial hypertension. In
obese women between 20 and 40
years, associated with CM-I, papillede-
ma might evolve into a more complex
clinical picture of PTC.
Based on these data, we can say that
CM-I alone infrequently leads to a
framework for papilledema and that
there must be an association to other
factors, as aforementioned, to fulfil a
PTC framework in the presence of
CM-I. The subject may benefit from
occipital decompression surgery per-
formed to restore normal cerebrospinal
fluid pressure,2 should drug and diet
not resolve the issue.
Conclusions
CONCLUSIONS
A patient with the clinical appearan-
ce of PTC and evidence of CM-I may
have true primary PTC with CM-I
being secondary to increased ICP.
However, pre-existing CM-I may have
caused increased ICP from obstruction
of outflow, thus producing the clinical
picture of PTC.
In the presence of clinical PTC, it
would be correct to perform an MRI to
evaluate the possible presence of descent
of the cerebellar tonsils that would diag-
nose a context of CM, which could bene-
fit from occipital-cervical decompression
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surgery after failure of medical therapy, as
it happened in our case. STI
Authors’ Disclosures
AUTHORS’ DISCLOSURES
The authors have no conflicts of
interest to disclose.
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