Takayasu's Arteritis Complicated With SAH and Hematomyelia

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Neurol Med Chir (Tokyo) 51, 119¿122, 2011

Takayasu's Arteritis Complicated With
Subarachnoid Hemorrhage and Hematomyelia
—Case Report—
Seung-Jae HYUN,1 Sung-Nam HWANG,2 Taek-Kyun NAM,2
Seung-Won PARK,2 and Jun-Soo BYUN3
1Department of Neurosurgery, Seoul National University Bundang Hospital,

Seoul National University College of Medicine, Seongnam, ROK;
Departments of 2Neurosurgery and 3Radiology, Chung-Ang University Yongsan Hospital,
Chung-Ang University College of Medicine, Seoul, ROK

Abstract
A 44-year-old woman presented with severe headache, drowsy mentality, and right hemiparesis. Brain
computed tomography and magnetic resonance angiography revealed non-aneurysmal subarachnoid
hemorrhage (SAH). Thoraco-abdominal and pelvic computed tomography angiography showed multiple steno-occlusive lesions involving the aorta and its large branches suggesting Takayasu's arteritis.
Spine magnetic resonance imaging was taken because of prominent right hand muscle atrophy on the
14th hospital day, which showed subacute stage of hematomyelia in the cervical cord and conus medullaris. Aneurysmal or non-aneurysmal SAH is rare in patients with Takayasu's arteritis but SAH with
coincidental hematomyelia is even more unusual. This case emphasizes the rarity of the coincidental
spinal hematomyelia and its importance in the differential diagnosis.
Key words:

Takayasu's arteritis,

subarachnoid hemorrhage,

Introduction
Takayasu's arteritis is an inflammatory vasculitis of
unknown etiology that involves the aorta and its major
branches, the pulmonary arteries and coronary arteries.
Vascular inflammation may cause arterial stenosis, occlusion, thrombosis, dilatation, or aneurysms.2,12) Takayasu's
arteritis affects young people, generally Asian women of
reproductive age. Clinical manifestations vary depending
Received

April 1, 2010;

Accepted

July 5, 2010

Neurol Med Chir (Tokyo) 51, February, 2011

aneurysm,

hematomyelia,

diagnosis

on the site of the blood vessels affected, organs involved,
and collateral circulation. Cerebrovascular accident may
be an important and predictive complication for the prognosis in such patients.2,4) We describe a woman who
presented with non-aneurysmal subarachnoid hemorrhage (SAH) with coincidental hematomyelia and occlusion of the carotid or vertebral artery associated with
Takayasu's arteritis.

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120

Fig. 1 Axial
computed
tomography
scans
showing
subarachnoid hemorrhage in the prepontine and perimedullary
cisterns.

Fig. 3 Brain and neck magnetic resonance angiograms showing the occluded right brachiocephalic and left subclavian arteries with multiple collateral vessels (arrows), and partial filling
of the right distal common carotid artery (double arrow).
Fig. 2 Computed tomography angiograms showing decreased
blood flow in the right petrous portion of the right internal
carotid artery (arrow). No intracranial aneurysm is noted.

Case Report
A 44-year-old woman presented with sudden onset of severe headache and drowsy mentality while she was
playing cards. She had suffered a mitral valve prolapse
and had been taking antihypertensive medications over
the last 5 years. On admission, she was drowsy with grade
2 right hemiparesis. Pain, temperature, and touch sensations were decreased on the right side of her body. Left
foot and leg were deformed, and muscles were atrophic
due to an accidental injury in her early life. Brain computed tomography (CT) demonstrated a small amount of SAH
in the prepontine and basal cisterns (Fig. 1). Brain CT and
magnetic resonance (MR) angiography showed significant
stenosis of the basilar artery and bulbous dilatation of the
left internal carotid artery, but no saccular aneurysmal lesion (Figs. 2 and 3). Our impression was basilar artery dissection. Lipid profile, coagulation study, hepatitis markers, cardiac markers, and electrocardiography were all
within normal limits. Erythrocyte sedimentation rate
(ESR) and C-reactive protein (CRP) were initially normal
but later constantly elevated: ESR (À30 mm/hr) and CRP
(À1 mg/dl), and mild leukocytosis (10,000–15,000/ml) was
noted.
On the 2nd hospital day, she began to complain of abdominal and flank pain. Thoraco-abdominal and pelvic
CT angiography showed segmental luminal narrowing of
the distal thoracic aorta, marked stenosis of the celiac
axis, and near total occlusion of the right brachiocephalic
and left subclavian arteries with distal filling by collateral
supply. The distal thoracic aorta showed focal stenosis

Fig. 4 Sagittal T1-weighted (A) and T2-weighted (B) magnetic
resonance (MR) images showing subacute stage hematomyelia
in the cervical spinal cord (arrows). Axial T2-weighted (C) and
gradient-recalled echo T2-weighted (D) MR images depicting
hematomyelia in the right anterior and posterior gray column
(arrows).

Neurol Med Chir (Tokyo) 51, February, 2011

Takayasu's Arteritis Complicated With SAH and Hematomyelia

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status was stable until discharge on the 66th hospital day.

Discussion

Fig. 5 Sagittal T1-weighted (A) and T2-weighted (B) magnetic
resonance (MR) images showing subacute stage hematomyelia
in the conus medullaris (arrows) and intradural-extramedullary
space (double arrows). Axial T1-weighted (C) and T2-weighted
(D) MR images at the conus medullaris level depicting rupture
of hematomyelia into the subarachnoid space (arrows).

with prominent collaterals such as bronchial or intercostal
arteries. Bilateral pleural effusion and dependent atelectasis were also noted. Her carotid and femoral pulsations
were very weakly palpated bilaterally. Her brachial blood
pressure was 114/66 mmHg on the right and 121/69 mmHg
on the left. Her pedial blood pressure was 80/57 mmHg on
the right and 95/68 mmHg on the left.
Under the impression of Takayasu's arteritis and SAH,
she underwent percutaneous stent insertion into the distal
thoracic aorta. However, stent insertion into the right
brachiocephalic artery failed because of marked tortuosity
of the artery. Steroid therapy was started and the motor
power of her right arm was improved to grade 4, but sensations and right leg weakness were unchanged. On the
14th hospital day, her prominent right hand intrinsic muscle atrophy led us to perform spinal MR imaging, which
showed subacute stage of hematomyelia in the cervical
cord (C2–C7) and conus medullaris (T12-L1) (Figs. 4 and
5). The hematoma in the conus medullaris had ruptured
out into the spinal subarachnoid space. Her neurological

Neurol Med Chir (Tokyo) 51, February, 2011

Patients with Takayasu's arteritis usually have constitutional symptoms such as fever, general malaise, and
weight loss before the development of various ischemic
symptoms. ESR and CRP are usually elevated in the acute
phase but remain normal in one third of patients.8) As the
disease progresses, the subclavian, innominate, carotid,
vertebral, coronary, renal, and other arteries are progressively occluded. As a result, ischemic vascular symptoms
are most common depending on the affected arteries.
Acute or chronic abdominal pain may be the presenting
symptom due to involvement of the mesenteric artery.8,13)
The most common symptoms are claudication of the legs,
and weakness and coldness of the arms. If the renal arteries are involved, hypertension may develop, and if the
carotid and vertebral arteries are involved, neurological
signs and symptoms may develop.
Most neurological symptoms are due to occlusion of the
cervical carotid and/or vertebral arteries, but are rarely
reported,8) possibly due to the abundant cerebral arterial
collateral vessels. Takayasu's arteritis rarely presents with
aneurysmal or non-aneurysmal SAH.2,5) The relationship
between cerebral aneurysm formation and Takayasu's arteritis has not yet been clearly defined. Hemodynamic
stress on the cerebral arteries, immune destruction and
necrosis of the elastic lamina, and hypertension may be
causes of aneurysm formation in Takayasu's arteritis.5,13)
However, aneurysmal SAH may be an incidental event unrelated with Takayasu's arteritis because the incidence of
cerebral aneurysm in Takayasu's arteritis is not different
from the natural incidence and microscopic studies of the
aneurysms have shown no vascular inflammation.6)
If pathological processes of the intracranial arterial wall
are involved in aneurysm formation in Takayasu's arteritis, dissecting aneurysm may also be formed and result in
SAH. Our patient had SAH mainly in the prepontine
cistern and her basilar artery was very stenotic compared
with the dilated vertebral arteries. Some patients with
Takayasu's arteritis present with aneurysmal or nonaneurysmal SAH, so our impression on admission was dissecting basilar artery or non-aneurysmal SAH. However,
the spinal MR imaging, taken 2 weeks later, showed
hematomyelia in the cervical cord and conus medullaris.
The immediate right hemiparesis and subsequent right
hand muscle atrophy were thought to be due to cervical
hematomyelia. Spontaneous onset of hematomyelia is usually due to vascular malformation in the spinal cord, intramedullary cavernoma, intrasyringal hemorrhage,
hematologic disorders, or anticoagulant therapy.1,9)
However, none of these were present in our patient. SAH
with coincidental hematomyelia is extremely unusual,
even compared to the rarity of aneurysmal or nonaneurysmal SAH in patients with Takayasu's arteritis.
Our patient immediately developed dense right hemiparesis despite the small amount of SAH, so the cause of
hemiparesis was thought to be cervical hematomyelia.
Since the hematomyelia of the conus medullaris ruptured

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into the spinal subarachnoid space without visible
cerebral saccular aneurysm, the cranial SAH might be due
to basilar artery dissection or ruptured hematoma from
the conus medullaris into the spinal subarachnoid space.
The subarachnoid blood was mainly located in the prepontine cistern, supporting the possibility of basilar artery dissection. Whether the bleeding in the cervical cord and conus medullaris occurred simultaneously with intracranial
SAH is still unknown due to the lack of initial spinal CT or
MR imaging.
Based on our speculation of hematomyelia and
Takayasu's arteritis, we suggest several plausible causes of
hematomyelia as follows: hemorrhagic infarction due to
embolism; hemorrhagic conversion secondary to spinal
cord infarction; and systemic vasculitis affecting small
vessels such as the radiculomedullary arteries. From the
neurosurgical point of view, Takayasu's arteritis is associated with cerebral and spinal cord infarctions. In a previous longitudinal study of 16 patients with Takayasu's arteritis, two patients experienced cerebral infarction but
none had spinal infarction.8) Another series included one
patient (5%) who suffered paraplegia due to spinal infarction.7)
However, whether the hematomyelia resulted from systemic pathologic processes in the arterial walls associated
with Takayasu's arteritis remains unclear. Systemic vasculitis can present as a tumor-like lesion although not usual.
Granulomatous vasculitis of tumor-like lesion associated
with spinal cord compression has been reported in
patients with Takayasu's arteritis, sarcoidosis, giant cell
arteritis, and Wegener's granulomatosis.3,10,11) Takayasu's
arteritis is now known to affect small vessels as well,
although radiculomedullary vessel involvement has not
been reported.12) Therefore, we suggest that the multi-centric hematomyelia of our patient probably developed from
inflammation associated with Takayasu's arteritis.
Aneurysmal or non-aneurysmal SAH occurs rarely in
the patients with Takayasu's arteritis, but this case emphasizes the rarity of the coincidental spinal hematomyelia
and its importance in the differential diagnosis.

Declaration of Interest
None of the authors have any financial interest in the subject under discussion in this paper.

References
1)

2)

3)

4)

5)

6)

7)

8)

9)

10)

11)

12)

13)

Aulakh R, Panigrahi I, Naranje K, Sharda S, Marwaha RK:
Spontaneous hematomyelia in a child with hemophilia A: a
case report. J Pediatr Hematol Oncol 31: 766–767, 2009
Kim DS, Kim JK, Yoo DS, Huh PW, Cho KS, Kang JK:
Takayasu's arteritis presented with subarachnoid hemorrhage: report of two cases. J Korean Med Sci 17: 695–698,
2002
Kim HA, Kim JH, Won JH, Suh CH: An unusual clinical
manifestation of Takayasu's arteritis: spinal cord compression. Joint Bone Spine 76: 209–212, 2009
Koz OG, Ates A, Numan Alp M, Gultan E, Karaaslan Y,
Kural G: Bilateral ocular ischemic syndrome as an initial
manifestation of Takayasu's arteritis associated with carotid
steal syndrome. Rheumatol Int 27: 299–302, 2007
Magge SN, Chen HI, Stiefel MF, Ernst L, Cahill AM, Hurst
R, Storm PB: Multiple ruptured cerebral aneurysms in a
child with Takayasu arteritis. J Neurosurg Pediatr 1: 83–87,
2008
Masuzawa T, Shimabukuro H, Sato F, Furuse M, Fukushima
K: The development of intracranial aneurysms associated
with pulseless disease. Surg Neurol 17: 132–136, 1982
Matsuura K, Ogino H, Matsuda H, Minatoya K, Sasaki H,
Yagihara T, Kitamura S: Surgical outcome of aortic arch
repair for patients with Takayasu arteritis. Ann Thorac Surg
81: 178–182, 2006
Petrovic-Rackov L, Pejnovic N, Jevtic M, Damjanov N: Longitudinal study of 16 patients with Takayasu's arteritis: clinical features and therapeutic management. Clin Rheumatol
28: 179–185, 2009
Pullarkat VA, Kalapura T, Pincus M, Baskharoun R: Intraspinal hemorrhage complicating oral anticoagulant therapy: an unusual case of cervical hematomyelia and a review
of the literature. Arch Intern Med 160: 237–240, 2000
Sailler LJ, Porte L, Ollier SM, Astudillo LM, Couret BG,
Catalaa I, Le Guellec S, Uro-Coste EV, Massip P, Arlet PM:
Giant cell arteritis and spinal cord compression: an overlap
syndrome? Mayo Clin Proc 81: 89–91, 2006
Schapiro JM, Shpitzer S, Pinkhas J, Sidi Y, Arber N: Sarcoidosis as the initial manifestation of Takayasu's arteritis. J
Med 25: 121–128, 1994
Skaria AM, Ruffieux P, Piletta P, Chavaz P, Saurat JH, Borradori L: Takayasu arteritis and cutaneous necrotizing vasculitis. Dermatology 200: 139–143, 2000
Weyand CM, Goronzy JJ: Medium- and large-vessel vasculitis. N Engl J Med 349: 160–169, 2003

Address reprint requests to: Sung-Nam Hwang, MD, PhD, Department of Neurosurgery, Chung-Ang University Yongsan
Hospital, Hangangno 3–ga, Yongsan–gu, Seoul 140–757,
ROK.
e-mail: tarheelk＠hanmail.net

Neurol Med Chir (Tokyo) 51, February, 2011