Neurol Sci (2011) 32 (Suppl 3):S291S294
DOI 10.1007/s10072-011-0698-x
CLINICS
Chronic daily headache in the adults: differential diagnosis
between symptomatic Chiari I malformation and spontaneous
intracranial hypotension
Eliana Mea Luisa Chiapparini Massimo Leone
Angelo Franzini Giuseppe Messina
Gennaro Bussone
Published online: 6 August 2011
Springer-Verlag 2011
Abstract This article briefly reviews the spectrum of
headaches associated with Chiari type I malformation
(CMI) and specifically analyzes the current data on the
possibility of this malformation as an etiology for some
cases of chronic daily headache (CDH). CMI is definitely
associated with cough headache and not with primary
episodic headaches, with the rare exception of basilar
migraine-like cases. With regard to CDH, there is no clear
evidence supporting an association with CMI. A magnetic
resonance imaging (MRI) study would be justified only in
patients showing either a Valsalva-aggravating component
or cervicogenic features. Hydrocephalus and low-intracra-
nial pressure syndrome should be ruled out in patients
showing tonsillar herniation in an MRI study and con-
sulting due to daily headache.
Keywords Chiari
CDH
IHS
Introduction
CMI is characterized by the downward displacement of the
cerebellar tonsils (the paraflocculus) through the foramen
magnum into the upper cervical spinal canal. CM types I,
II, and III represent different pathologic aspects of pos-
terior fossa hindbrain maldevelopment.
Due to the variable degrees of nervous tissue displace-
ment, the size of the foramen magnum, and the compressed
structures, clinical presentation of CMI are highly variable.
E. Mea (&)
L. Chiapparini
M. Leone
A. Franzini

G. Messina
G. Bussone
Fondazione IRCCS Istituto Neurologico C. Besta,
Via Celoria 11, 20133 Milan, Italy
e-mail: mea.e@istituto-besta.it
Clinical presentations of CMI are related to direct
compression syndromes (brainstem or spinal cord) or to
cerebrospinal fluid (CSF) disturbances (hydrocephalus,
SHM, endolymphatic hydrops, pseudotumor-like episodes,
and headache) [1].
Clinical manifestations, which typically begin in young
adulthood, can include headaches, visual disturbances,
neuro-otological complaints, lower cranial nerve dysfunc-
tion, and sleep apnea [1]. Among the manifestations of
CMI, headache is one of the most common symptoms,
occurring in 1598% of patients.
Chiari-distinctive headache is occipitalsuboccipital,
with a tendency for accentuation by Valsalva, head
dependency, sudden postural change and physical exertion
[24].
Management of CMI includes decompressive suboc-
cipital craniectomy, although opinions differ on the reasons
for operative intervention [5]. Conservative management
without surgery is generally used for headache rather than
for specific occipitalsuboccipital headache with signifi-
cant disability and concurrent cough headache.
Headaches associated with Chiari type I malformation
It is not rare in daily clinical practice to see patients with
episodic headaches, mainly migraine, who have received a
craniocervical MRI to rule out a CMI.
There is no evidence suggesting that CMI can induce
any kind of primary episodic headaches. First, the number
of reported cases combining both tension-type headache or
migraine and CMI is very low, clearly anecdotal [6, 7].
Second, in the series of patients with symptomatic CMI,
there was no increase in the prevalence of migraine or other
primary headaches [3].
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The prevalence of CMI is not precisely known, but it
was considered to be approximately one or two per 1000
births. Since the advent of MRI, the diagnosis of CMI has
also become easier and more frequent [8].
Therefore, the incidental coexistence of these two dis-
orders is an expected possibility, and currently there is no
indication for an MRI study in patients presenting with
primary headaches of any kind other than those associated
with Valsalva maneuvers.
In a retrospective review of 50 patients with CMI,
Pascual et al. [9] reported that 25 (50%) had a headache
disorder. Fourteen of 50 (28% of total), or 56% of those
with headache, had a specific, usually protracted, suboc-
cipitaloccipital pain of variable quality and duration that
was aggravated by Valsalva maneuver, effort, cough, or
postural changes; this typical headache was relieved by without headache, and between patients those with head-
occipitalsuboccipital craniectomy. Of the other 11
patients with headache, six had tension-type headache and
five had migraine (12 and 10% of total). The incidence of
migraine and tension-type headache is in accordance with
that reported in the general adult population.
Nohria and Oakes [10] reported the data of headache on
43 CMI patients. Twenty-nine of the 39 patients who
experienced symptoms were children. The most common
symptom was pain in 27 (69%), with the most common site
being the occipital and neck area (17 of 27; 63 or 44% of
total subjects).
Stovner [6] reported the symptoms of 34 patients with
CMI; 20 complained of headache (59%). Ten (50%) had
short-lasting attacks of cough headache that lasted for
\5 min. Fourteen of 20 (70%) had relatively long-last-
ing attacks ranging from 3 h to several days; eight
patients had continuous, fluctuating headaches. The rel-
atively long-lasting headaches were unilateral, without
described location, and were pulsating in 50% of the
patients, with side-shift in four of seven patients. Eleven
of 14 patients suffering from headache with long-lasting
attacks met the minimal IHS criteria for migraine with-
out aura.
Milhorat et al. [1] prospectively observed 364 CMI
patients; 296 (81%) had headache. This inferred that all
experienced suboccipital headaches were described as a
heavy, crushing, or pressure-like sensation that radiated to
the vertex, behind the eyes and inferiorly to the neck and
shoulders. Severe headaches were pounding, but non-
throbbing. A distinctive feature of the headaches was their
tendency to be accentuated by physical exertion, Valsalva
maneuvers, head dependency and sudden changes in
posture.
Other studies support the notion that headache and neck
pain are the frequent complaints, occurring in 6581% of
CMI patients; however, sufficient characteristics of head-
aches are lacking [1113].
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Neurol Sci (2011) 32 (Suppl 3):S291S294
Pathogenesis of CMI headaches
Initially, Chiari related the CMI changes to congenital
hydrocephalus. It was later noticed that the severity of the
hydrocephalus did not relate to the extent of the malfor-
mation and postulated that the defective growth of the skull
caused increased local pressure, presumably forcing the
descent of the hindbrain [14].
The pathophysiology of headaches in CMI remains
obscure [9]. The degree of tonsillar descent correlated with
the presence of cough headache in the series by Pascual
et al. [9]. The authors stated that tonsillar herniation, not
BOS, causes the specific occipitalsuboccipital headache.
In Stovners cases [6], tonsillar descent failed to differen-
tiate between patients those with headache and those
ache with and without short-lasting cough attacks.
Stovner [6] cites that the mechanism for cough headache
seems to be the transient pressure dissociation between the
intracranial and the intraspinal compartments. This proba-
bly induces further impaction of the cerebellar tonsils into
the foramen magnum, producing pain by traction and
pressure on pain-sensitive nerves, meninges and vessels.
Milhorat et al. [9] found decreased CSF volume in the
posterior cranial fossa and claims that newly formed CSF is
displaced into other available spaces, almost certainly
affecting CSF compliance and altering the normal damping
effect of an open CSF system.
Headache and tonsillar herniation: other possibilities
can be considered
There are at least two other possibilities to be considered in
the differential diagnosis of patients who complain of
continuous or subcontinuous headache and have tonsillar
herniation detected by an MRI. The first possibility is
headache due to intracranial hypertension secondary to
hydrocephalus. Ventricular dilation is a well-known com-
plication of CMI, and these patients usually show daily
headache. Hydrocephalus can be slight or even intermit-
tent, due to a valve-like mechanism, and can even be
missed in a conventional cranial CT scan study.
The second clinical possibility to be considered in
patients with daily headache and tonsillar descent is low-
pressure headache giving rise to pseudo-Chiari. Brain
descent, with tonsillar herniation similar to the one seen in
CMI, is a well-documented consequence of the syndrome
of intracranial hypotension. These patients usually com-
plain of posterior headache, frequently aggravated or
brought on by cough, and dizziness, which, together with
the herniation in the non-contrast MRI scan, can be mis-
interpreted as secondary to a true CMI.
Neurol Sci (2011) 32 (Suppl 3):S291S294 S293

There are several clues indicating a pseudo-Chiari due to IHS Diagnosis ICD-10
low-intracranial pressure. The first indication is a recent 7.7 Headache attributed to Chiari G44.82
lumbar puncture or epidural anesthesia procedure. How- malformation type I (CMI) [Q07.0]
ever, this antecedent is not always present, as some cases Diagnostic criteria:
occur after an effort (e.g., coitus) or even spontaneously 3. Demonstration of clinical signs relevant to cervical cord,
[4]. Second, in these cases headache appears or aggravates brainstem or lower cranial nerves or of ataxia or dysmetria
only on standing (orthostatic headache). Some of these D. Headache resolves within 3 months after successful treatment of
patients also have headache induced by cough. In addition, the Chiari malformation
the absence of posterior fossa dysplasia and the appearance
IHS Diagnosis ICD-10
of meningeal enhancement after gadolinium confirms this
7.2.3 Headache attributed to spontaneous G44.820
diagnosis. (or idiopathic) low-CSF pressure
Diagnostic criteria:

A. Diffuse and/or dull headache that worsens within 15 min after

Conclusions sitting or standing, with at least one of the following and fulfilling
criterion D:
CMI is definitely associated with cough headache. This 1. Neck stiffness
hindbrain malformation does not correlate with a higher 2. Tinnitus
incidence of primary episodic headaches. MRI is justified 3. Hypacusia
only in patients showing either a Valsalva aggravating 4. Photophobia
component or cervicogenic features. Headache due to 5. Nausea
hydrocephalus and low-pressure headache should be ruled B. At least one of the following:
out in patients exhibiting a tonsillar descent in an MRI 1. Evidence of low-CSF pressure on MRI (e.g., pachymeningeal
study and consulting due to daily headache. enhancement)
2. Evidence of CSF leakage on conventional myelography, CT
myelography or cisternography
IHS Diagnosis ICD-10 3. CSF opening pressure \60 mm H2O in sitting position
7.7 Headache attributed to Chiari G44.82 C. No history of dural puncture or other cause of CSF fistula
malformation type I (CMI) [Q07.0] D. Headache resolves within 72 h after epidural blood patching
Diagnostic criteria:

A. Headache characterized by at least one of the following and

fulfilling criterion D:
1. Precipitated by cough and/or Valsalva maneuvre
2. Protracted (hours to days) occipital and/or suboccipital headache
3. 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. C5 mm caudal descent of the cerebellar tonsils
2. C3 mm caudal descent of the cerebellar tonsils plus at least one
of the following indicators of crowding of the subarachnoid 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, hypacusia or
hyperacusia, vertigo, down-beat nystagmus, oscillopsia)
2. Transient visual symptoms (spark photopsias, visual blurring,
diplopia or transient visual field deficits)
Conflict of interest The authors declare that there is no actual or
potential conflict of interest in relation to this article.
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