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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-
PTC is a syndrome characterized by signs and symptoms of increased intracranial pressure (ICP) in the
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
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
Table I
Headache attributed to Chiari malformation type I: diagnostic criteria
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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
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#854 Pacca FINAL
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 the first 13 considers PTC because of surgery after failure of medical therapy, as
of outflow at the level of the arachnoid CM-I, whilst the second 15 addresses it happened in our case. STI
villi. In CM-I, several different mecha- the two diseases as a result of a com-
nisms have been implicated in the pro- mon problem.
duction of increased ICP.1,2,13-15 It is important to consider CM-I as Authors’ Disclosures
It can be considered correct that a a “cofactor” that can lead to PTC when
AUTHORS’ DISCLOSURES
patient with the clinical picture of PTC associated with certain predisposing
and evidence of CM-I may have prima- factors (obesity, female, aged between The authors have no conflicts of
ry PTC with CM-I being secondary to 20 and 40). interest to disclose.
increased ICP.13 However, the opposite In a case study by Milhorat et al.,3 on
is also true, pre-existing CM-I may a large series of 364 patients with a
have caused increased ICP from symptomatic CM-I, only nine had References
obstruction of outflow, thus producing papilledema (2%). Therefore, papillede-
REFERENCES
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in symptomatic CM-I (S. Chiari), with Based on these data, we can say that intracranial volume in pediatric Chiari I mal-
decompression of the PCF.13,15 CM-I alone infrequently leads to a formation. Acta Neurochir 2010;152:221–7.
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CONCLUSIONS
Idiopathic Intracranial Hypertension. Acta
by occipital decompression to restore Neurol Scand 2010;121:71–82.
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