doi:10.1111/j.1468-2982.2007.01437.x

BRIEF REPORT

Spontaneous intracranial hypotension with isolated cortical vein
thrombosis and subarachnoid haemorrhage
Y-F Wang1,3, J-L Fuh1,3, J-F Lirng2,3, F-C Chang2,3 & S-J Wang1,3
1

Neurological Institute and 2Department of Radiology, Taipei Veterans General Hospital, 3National Yang-Ming University School of Medicine,
Taipei, Taiwan

Wang Y-F, Fuh J-L, Lirng J-F, Chang F-C & Wang S-J. Spontaneous intracranial
hypotension with isolated cortical vein thrombosis and subarachnoid haemorrhage. Cephalalgia 2007; 27:1413–1417. London. ISSN 0333-1024
Spontaneous intracranial hypotension (SIH) associated with subarachnoid
haemorrhage (SAH) has never been reported. Here, we report on a case of a
33-year-old woman with SIH, who developed simple partial sensory seizures
3 weeks later. Neuroimaging studies, including brain computed tomography and
angiography, were initially normal, but revealed an isolated cortical venous
thrombosis at 3 weeks. One week later, brain magnetic resonance imaging
showed SAH around the thrombosed cortical vein. We postulate that the decline
in the venous blood flow velocity due to SIH may have resulted in cortical
venous thrombosis, which in turn led to rupture of the vessel wall and SAH
in this patient. 䊐 Cortical venous thrombosis, headache, spontaneous intracranial
hypotension, subarachnoid haemorrhage
Dr Shuu-Jiun Wang, the Neurological Institute, Taipei-Veterans General Hospital,
Taipei, Taiwan, 112. Tel. + 886 2 2876 2522, fax + 886 2 2876 5215, e-mail
sjwang@vghtpe.gov.tw Received 26 January 2007, accepted 15 June 2007

Introduction
Spontaneous intracranial hypotension (SIH) is a
syndrome associated with low cerebrospinal fluid
(CSF) volume or decreased CSF pressure, and
usually presents with a postural headache (1). SIH
associated with isolated cortical venous thrombosis
(ICVT) rarely occurs (2), whereas SIH with subarachnoid haemorrhage (SAH) has never been
reported in the English literature. We present a case
of a woman with SIH who developed ICVT and
subsequently SAH, a case which may provide some
insight into the pathophysiology of SIH.

Case report
A 33-year-old woman, who did not have systemic
disease or take medications regularly, had experienced an abrupt onset of intense pain involving
the occipito-nuchal region in an attempt to stand
up 3 weeks prior to presentation. The symptom
© Blackwell Publishing Ltd Cephalalgia, 2007, 27, 1413–1417

had improved immediately after she lay down.
However, the headache worsened as time went by,
and nausea and vomiting followed at its extremes.
She was taken to the emergency department of
another hospital on the next day (day 2) and brain
computed tomography (CT) was normal. Some
bloody CSF was eventually obtained after several
unsuccessful spinal taps, which was attributed to
traumatic tapping. Cerebral angiography failed to
demonstrate any vascular anomaly (day 4). She was
discharged 3 days later after symptoms improved,
but her headache returned shortly thereafter. She
was then admitted to our hospital 2 weeks later due
to persistence of headache. A repeat brain CT on
day 20 had revealed a hyperdense tubular structure
in the left parasagittal parietal area (Fig. 1a). She
experienced an episode of numbness involving the
right leg, extending to the right hemibody and face
on day 21, which resolved within 10 min, and an
emergent brain CT failed to disclose significant
change compared with previous films. She reported
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Figure 1 (a) Axial view of the brain computed tomography (day 20): a hyperdense tubular structure in the left parietal
parasagittal area is seen (arrowheads). (b) Venous phase of cerebral angiogram of the right internal carotid artery (ICA)
(day 24): the right vein of Trolard was patent, as indicated by arrows. Venous phase of cerebral angiogram of the left ICA
(day 24): (c) lateral view shows segmental non-visualization of the left vein of Trolard (arrows), and engorgement of the
surrounding venules (*), suggestive of thrombosis of part of the vein of Trolard and redirection of blood flow through the
collateral venules; (d) anteroposterior view reveals smooth segmental narrowing of part of the transverse sinus (white
arrowheads) connecting the sinus confluence and the vein of Labbé, probably a normal anatomical variation.

two more attacks during the following week. All
these attacks were attributed to simple partial seizures. Neurological examination performed after
complete recovery was unremarkable, and electroencephalography (EEG) did not show any epileptiform discharge. A repeat cerebral angiography on
day 24 demonstrated segmental non-visualization

of the vein of Trolard in the left parietal parasagittal
area (Fig. 1c), which corresponded to the ‘cord sign’
on the previous brain CT films, and a diagnosis of
ICVT in addition to SIH was made. Smooth segmental narrowing of part of the transverse sinus
connecting the sinus confluence and the vein of
Labbé was also found (Fig. 1d); this could, however,
© Blackwell Publishing Ltd Cephalalgia, 2007, 27, 1413–1417

SIH with ICVT and SAH

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homocysteine, lupus anticoagulant, cardiolipin IgG
and IgM and liver functions) was unrevealing.
Lumbar puncture on day 31 revealed slightly
depressed intracranial pressure (78 mmH2O) and
some erythrocytes (73/mm3) in the CSF. Heavily
T2-weighted MR myelography on day 61 showed
abnormal CSF leakage at the high cervical retrospinal region and lower cervical spinal nerve roots
(Fig. 3a–c). As the response to conservative measures was unsatisfactory, she received epidural
blood patches (EBP) over the lower cervical region
on day 84, which resulted in prompt symptomatic
relief. The hyperdense tubular structure was not
seen on a follow-up brain CT. Brain MRI and MR
venography on day 113 documented resolution of
the SAH, as well as other typical SIH findings, and
partial recanalization of the previously thrombosed
cortical vein. The patient remained symptom-free at
6 months’ follow-up after EBP.

Discussion

Figure 2 Brain magnetic resonance imaging. (a)
Fluid-inversion recovery (FLAIR) (day 28) showed
hyperintensities within the cortical sulci (black arrows) in
the left parietal region, which were isointense to faintly
hyperintense (black arrows) on T1-weighted images
(T1WIs) (b), indicating subarachnoid blood.

have been a normal anatomical variation. Brain
magnetic resonance imaging (MRI) on day 28
showed SAH in the vicinity of the thrombosed
cortical vein (Fig. 2a,b) in addition to diffuse pachymeningeal gadolinium enhancement. The evaluation of thrombophilia (including prothrombin and
activated partial thromboplastin times; protein C,
protein S, antithrombin III, fibrinogen levels,
© Blackwell Publishing Ltd Cephalalgia, 2007, 27, 1413–1417

We present a unique case of SIH, complicated by
the development of ICVT and subsequently SAH.
Cerebral venous sinus thrombosis is an uncommon
complication in patients with SIH (3–5), and there
has been only one case report of ICVT in association
with SIH (2). According to the Monro-Kellie doctrine (6), one can hypothesize that reduced intracranial pressure and brain descent can result in
dilation of cerebral veins and distortion of venous
walls, respectively, both of which may contribute to
stagnancy and turbulence of blood flow in the
cerebral veins, leading to thrombosis (2). The anatomical variation of segmental narrowing of part of
the transverse sinus seen in our patient (Fig. 1d)
might result in inadequate drainage of the vein of
Labbé on the left side, which could be exaggerated
once blood flow volume is increased, as in the case
of intracranial hypotension. Such exaggeration as
seen in our patient could possibly have further
slowed the venous flow, and thus led to thrombus
formation in the vein of Trolard, one major collateral channel of which is provided by the vein of
Labbé (Fig. 4).
The majority of SAHs are of aneurysmal origin,
and non-traumatic cortical SAH is rare (7).
However, it is not unusual in patients with ICVT,
and there have been five patients associated with
SAH and ICVT reported in the literature (7–9). The
most frequent clinical manifestations are headache
and focal seizures. However, these symptoms are
also commonly seen in patients with ICVT and may
not be easily differentiated from those of SAH on

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Figure 3 Heavily T2-weighted magnetic resonance myelography shows cerebrospinal fluid leakage at the C1-2 retrospinal
region (white arrows) (a: sagittal plane, b: transverse plane) and along the C5-6 nerve roots (white arrows) (c).

clinical grounds alone. The exact mechanism of
SAH in patients with ICVT is unknown. When
venous thrombosis is limited to a single cortical
vein, blood flow could be diverted through collateral venules surrounding the lesion by reversing
the direction of flow. Blood and pressure accumulate backwards, namely in the subarachnoid
segment of the cortical vein, and could thus result
in rupture of the vessel wall, and hence SAH. On
the other hand, in patients with dural sinus thrombosis, several or most of the cortical veins are

involved, and access of blood flow to other cortical
veins via collaterals is limited, which may explain
why SAH seems to be less commonly associated
with dural sinus thrombosis than with ICVT. We
believe that the reduced CSF space pressure in our
patient could have further exaggerated the discrepancy between intra- and extraluminal pressure,
favouring rupture in the subarachnoid segment of
the cortical vein.
To our knowledge, this is the first case of SAH
complicating SIH in the English literature. The
© Blackwell Publishing Ltd Cephalalgia, 2007, 27, 1413–1417

SIH with ICVT and SAH
a

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clinical course and imaging findings of this patient
not only provide some insight into the pathophysiology of SIH, but also suggest a sequential relationship from SIH to ICVT and then to SAH.

Acknowledgements
The study was supported in part by a grant from the
Taipei-Veterans General Hospital (V96C1-041), Taipei,
Taiwan.

References

b

Figure 4 Postulated mechanism of isolated cortical venous
thrombosis. (a) The vein of Trolard (VT) is drained by the
superior sagittal sinus (SSS) in normal subjects, and its
terminal branches can also be drained by the vein of
Labbé (VL). In the presence of intracranial hypotension
however, as shown in (b), blood flow stagnation in the VT
caused by decreased flow velocity in the SSS and poor
collateral drainage of the VL due to anatomical variation
(arrowheads) might explain what happened in this patient.
The stasis and small diameter of the VT predisposed to
thrombus formation.

© Blackwell Publishing Ltd Cephalalgia, 2007, 27, 1413–1417

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