J Neurosurg 118:1058–1062, 2013
©AANS, 2013

Neurological sequelae from brachiocephalic vein stenosis
Report of 2 cases
David W. Herzig, M.D.,1 Andrew B. Stemer, M.D., 2 Randy S. Bell, M.D., 3
Ai-Hsi Liu, M.D., 2 Rocco A. Armonda, M.D., 3 and William O. Bank, M.D. 2
Department of Neurosurgery, Georgetown University Hospital; 2Department of Radiology, Washington
Hospital Center, Washington, DC; and 3Department of Neurosurgery, Walter Reed National Military
Medical Center, Bethesda, Maryland
1

Stenosis of central veins (brachiocephalic vein [BCV] and superior vena cava) occurs in 30% of hemodialysis
patients, rarely producing intracranial pathology. The authors present the first cases of BCV stenosis causing perimesencephalic subarachnoid hemorrhage and myoclonic epilepsy.
In the first case, a 73-year-old man on hemodialysis presented with headache and blurry vision, and was admitted with presumed idiopathic intracranial hypertension after negative CT studies and confirmatory lumbar puncture.
The patient mildly improved until hospital Day 3, when he experienced a seizure; emergency CT scans showed
perimesencephalic subarachnoid hemorrhage. Cerebral angiography failed to find any vascular abnormality, but demonstrated venous congestion. A fistulogram found left BCV occlusion with jugular reflux. The occlusion could not
be reopened percutaneously and required open fistula ligation. Postoperatively, symptoms resolved and the patient
remained intact at 7-month follow-up.
In the second case, a 67-year-old woman on hemodialysis presented with right arm weakness and myoclonic
jerks. Admission MRI revealed subcortical edema and a possible dural arteriovenous fistula. Cerebral angiography
showed venous engorgement, but no vascular malformation. A fistulogram found left BCV stenosis with jugular
reflux, which was immediately reversed with angioplasty and stent placement. Postprocedure the patient was seizure
free, and her strength improved. Seven months later the patient presented in myoclonic status epilepticus, and a fistulogram revealed stent occlusion. Angioplasty successfully reopened the stent and she returned to baseline; she was
seizure free at 4-month follow-up.
Central venous stenosis is common with hemodialysis, but rarely presents with neurological findings. Prompt
recognition and endovascular intervention can restore normal venous drainage and resolve symptoms.
(http://thejns.org/doi/abs/10.3171/2013.1.JNS121529)

Key Words
•
central venous stenosis
•
jugular venous reflux
•
idiopathic perimesencephalic subarachnoid hemorrhage •
myoclonic epilepsy
vascular disorders

N

sequelae from abnormal cerebral
venous drainage are predominantly caused by intracranial venous thrombosis or a vascular malformation. Competency of the IJV valve prevents an alteration in the extracranial venous system from affecting
the cerebral circulation.4,14 Anatomical studies have found
that chronic venous hypertension will make the IJV valve
dysfunctional.4,15 When the IJV valve is incompetent, the
resulting jugular venous reflux can lead to intracranial
pathological conditions.2,3,5,6,12,20
Venous hypertension is a significant problem in patients with chronic hemodialysis. Approximately 30% of
eurological

Abbreviations used in this paper: AVF = arteriovenous fistula;
BCV = brachiocephalic vein; IJV = internal jugular vein; PSAH =
perimesencephalic subarachnoid hemorrhage; SVC = superior vena
cava.

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•

patients receiving hemodialysis will develop stenosis of
the central veins (BCV or SVC) attributable to proximal
venous catheterization.1,10,18 Of this subset, half will develop symptomatic venous hypertension from venous engorgement, most commonly presenting as ipsilateral chest
and upper-extremity edema.1
We present 2 cases of BCV stenosis producing extracranial venous hypertension and different neurological findings. These are the first reports of central venous
stenosis associated with idiopathic PSAH and recurrent
myoclonic epilepsy.
Case 1

Case Reports

History and Examination. This 73-year-old hypertenJ Neurosurg / Volume 118 / May 2013

Neurological sequelae from brachiocephalic vein stenosis
sive African-American man on hemodialysis, who had a
history of a pacemaker placement and idiopathic intracranial hypertension, presented with 2 days of disorientation,
perseveration, headache, and blurry vision. On admission,
a CT/CT angiography study of the head showed no acute
findings or vascular abnormalities, and evaluation by an
ophthalmologist was remarkable for papilledema. A lumbar puncture recorded an opening pressure of 35 cm H2O
with 15 ml of CSF drained off. The patient’s mental status mildly improved following the lumbar puncture and
he was started on acetazolamide. His symptoms were attributed to idiopathic intracranial hypertension and side
effects of topiramate started the day prior to admission
for headaches.
Operation. On hospital Day 3, the patient complained
of a severe headache prior to having a witnessed grand
mal seizure. Emergency head CT revealed SAH in the
basilar cisterns (Fig. 1). Cerebral angiography failed to
find an aneurysm or vascular malformation, but demonstrated extremely slow cerebral circulation time with
marked cortical venous dilation. A fistulogram of the left
upper-extremity AVF showed left BCV occlusion with
left jugular venous reflux (Fig. 2). An attempt to recannulate the left BCV was unsuccessful and the patient was
taken to the operating room for emergency fistula ligation.

Postoperative Course. Postoperatively, all of his
symptoms quickly improved. Repeat cerebral angiogram
on postoperative Day 1 showed normal arterial-venous
transit time, resolution of cortical venous distention, and
antegrade jugular flow. The patient returned to his baseline neurological status within 2 days, was discharged
home, and remained neurologically intact at 6-month
follow-up.
Case 2
History and Examination. This 67-year-old hypertensive African-American woman on hemodialysis presented with 2 weeks of disorientation, progressively worsening right arm weakness, and “jerking” movements. In
the emergency room, a CT head scan demonstrated left
frontal lobe edema and an MRI study of the brain confirmed bilateral (left > right) subcortical white matter
edema (Fig. 3). Findings on an MR angiography study
were of concern for early venous visualization and possible dural AVF. The neurological evaluation showed that
her seizures were consistent with myoclonic epilepsy, and
levetiracetam was started.

Operation. Cerebral angiography demonstrated delayed drainage and venous engorgement, but did not
show any underlying vascular malformations (Fig. 4). A
fistulogram showed high-grade stenosis of the left BCV
with left jugular venous reflux (Fig. 5). The left BCV was
successfully treated with angioplasty and stent insertion,
leading to immediate antegrade flow through the left IJV
(Fig. 6).

Postoperative Course. Postprocedure the patient was
seizure free, her right arm strength improved greatly, and
J Neurosurg / Volume 118 / May 2013

Fig. 1. Case 1. Axial head CT scan showing SAH in the basilar cisterns.

she was discharged home. Seven months after discharge,
she developed problems with her hemodialysis access and
was scheduled for a fistulogram the following day. The
next morning, the patient was found by her family in status epilepticus, the first seizure since her original admission. An MRI study obtained on presentation noted new
bilateral thalamic edema and improved bilateral subcortical white matter edema when compared with the prior
admission. A fistulogram showed complete occlusion of
the left BCV stent with retrograde flow in the left IJV.
Angioplasty was again successful in reopening the left
BCV, with immediate resolution of jugular venous reflux.
The patient improved quickly to her neurological baseline
and remained seizure free at 4-month follow-up.

Discussion
Central Venous Stenosis

Central venous stenosis is prevalent in 16%–50% of
all patients with central venous catheters; higher rates are
associated with multiple catheter placements, indwelling catheters, and pacemaker wires,1,10,18 and it is usual­
ly asymptomatic in patients not receiving hemodialysis.
Oth­­er less common causes of central venous stenosis include radiation to the neck and chest, tumor compression
(SVC syndrome), and deep venous thrombosis of the upper extremity. With hemodialysis, central venous stenosis
is thought to be caused by high-pressure turbulent flow
stimulating intimal hyperplasia. The combination of venous stenosis and increased blood flow with hemodialysis results in venous engorgement and produces clinical
manifestations.2
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D. W. Herzig et al.

Fig. 2. Case 1. Left upper-extremity fistulogram showing BCV occlusion (arrow) with left IJV reflux. SC = subclavian vein.

Diagnosis of central venous stenosis can be elusive,
and the senior authors (W.O.B. and R.A.A.) advise that a
thorough evaluation must include a fistulogram (if present). The National Kidney Foundation guidelines rec­
ommend percutaneous transluminal angioplasty, with or
without stent placement, as the preferred approach to central venous stenosis.18 If the stenosis proves to be refractory to endovascular methods, surgical options include
revision or ligation of the AVF and bypass.

Fig. 3. Case 2. Bilateral subcortical white matter edema on FLAIR
imaging.

are also a source of debate. One proposal is that increased
venous pressure can lead to vessel engorgement and potentially rupture. This hypothesis is supported in the literature with reports on PSAH precipitated by straining and
physical exertion11,21 as well as publications highlighting
aberrant venous drainage patterns7,16,19,21 and underlying
venous pathologies.8,9,13 Our first case also corroborates

The IJV Valve and Functionality

The IJV valve was first described in the 16th century
and long thought to have no physiological significance.15
However, growing evidence from more recent investigations demonstrates that these valves are indeed functional
and serve to avert backflow into the IJVs.14,15,20 Competency of the IJV valve prevents alterations to the intracrani­
al circulation, which may be why reports of neurological
changes from jugular venous reflux are rare.
With long-standing venous hypertension, as with central venous stenosis, Fisher et al.4 found that the IJV valve
became dysfunctional in 10 (67%) of 15 patients. This
finding is corroborated by the fact that jugular venous reflux was present in our report and in all previous central
venous case reports.3,5,6,12
Venous Hypertension and PSAH

Since van Gijn et al.17 first described PSAH in 1985,
the exact cause remains unresolved. Most authors suggest
a venous source given its relatively benign clinical course
and low complication rate when compared with aneurysmal SAH.
Putative risk factors predisposing patients to PSAH

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Fig. 4. Case 2. Cerebral angiogram, venous phase, showing delayed drainage and venous engorgement.

J Neurosurg / Volume 118 / May 2013

Neurological sequelae from brachiocephalic vein stenosis
neurological findings. We share 2 cases of venous hypertension from BCV stenosis transmitted to the intracranial circulation and resulting in different neurological
sequelae. Prompt recognition and intervention can lead
to restoration of normal venous drainage and resolution
of symptoms.
Disclosure
This report had no financial support and all authors included do
not have a conflict of interest or any financial disclosures.
Author contributions to the study and manuscript preparation
include the following. Conception and design: Bank. Acquisition of
data: Herzig, Bell, Liu, Armonda, Bank. Analysis and interpretation
of data: Bank. Drafting the article: Herzig. Critically revising the
article: all authors. Reviewed submitted version of manuscript: all
authors. Approved the final version of the manuscript on behalf of
all authors: Herzig. Administrative/technical/material support: Bell,
Liu, Armonda, Bank.
References

Fig. 5. Case 2. Left upper-extremity fistulogram demonstrating highgrade stenosis (arrow) of the BCV with left IJV reflux.

the idea that increased venous pressure can lead to PSAH,
and to our knowledge this the first report on central venous hypertension being associated with PSAH.

Conclusions

Central venous stenosis is a common finding in patients receiving hemodialysis that rarely presents with

Fig. 6. Case 2. Angiogram obtained after angioplasty and stent
placement, with antegrade flow through the left IJV.

J Neurosurg / Volume 118 / May 2013

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Manuscript submitted August 5, 2012.
Accepted January 8, 2013.
Please include this information when citing this paper: published
online February 1, 2013; DOI: 10.3171/2013.1.JNS121529.
Address correspondence to: David W. Herzig, M.D., Department
of Neurosurgery, Georgetown University Hospital, 3800 Reservoir
Road NW, 7PHC, Washington, DC 20007. email: david.herzig@
gmail.com.

J Neurosurg / Volume 118 / May 2013