□ CASE REPORT □

Reversible Cerebral Vasoconstriction Syndrome in a Patient
with Takayasu’s Arteritis
Yuto Uchida 1, Noriyuki Matsukawa 1, Takuya Oguri 1, Keita Sakurai 2, Toshiyasu Miura 1,
Shiho Iwagaitsu 3, Taio Naniwa 3 and Kosei Ojika 1

Abstract
We herein present the first reported case of reversible cerebral vasoconstriction syndrome (RCVS) in
Takayasu’s arteritis (TA), in a patient with severe thunderclap headache and generalized tonic-clonic seizure.
Magnetic resonance imaging and angiography revealed posterior reversible encephalopathy syndrome, followed by severe vasoconstriction in multiple arteries. RCVS should be included in the differential diagnosis
of thunderclap headache attacks with radiologic intracranial vascular abnormalities in connective tissue diseases, including TA.
Key words: reversible cerebral vasoconstriction syndrome, Takayasu’s arteritis, intracranial vessel, magnetic
resonance angiography, posterior reversible encephalopathy syndrome
(Intern Med 50: 1611-1614, 2011)
(DOI: 10.2169/internalmedicine.50.5185)

Introduction
Reversible cerebral vasoconstriction syndrome (RCVS) is
characterized by prolonged but reversible vasoconstriction of
the cerebral arteries, usually associated with acute-onset, severe “thunderclap headache”, with or without additional
neurologic signs and symptoms. The “string of beads” appearance of the cerebral arteries on angiographic images can
resolve spontaneously in 1-3 months (1, 2). Previous reports
have described the most common clinical feature to be a severe acute headache, like a “clap of thunder”, similar to that
of a ruptured aneurysm. However, cases described as RCVS
could include a heterogeneous range of disorders. Furthermore, the underlying pathologic mechanism remains unknown (1).
Takayasu’s arteritis (TA), which is common in Asia and
Mexico, is a systematic inflammatory disease that affects the
aorta and its main branches (3, 4). The inflammatory processes cause narrowing, occlusion, and/or dilation of arteries,
resulting in a variety of symptoms. The involvement of the
extracranial portions of the carotid and vertebral arteries induces cerebrovascular manifestations, e.g., vertigo, syncope,

and convulsions, mainly caused by cerebral ischemia (3, 4).
However, the involvement of intracranial arteries in patients
with TA has rarely been reported (5). We herein report the
presence of RCVS, and associated clinical and arteriographic involvement of the intracranial arteries, in a patient
with TA. To our knowledge, this is the first case of RCVS
described in a TA patient.

Case Report
A 15-year-old girl with TA presented to our emergency
department complaining of acute-onset severe thunderclap
headache with impaired consciousness, followed by generalized tonic-clonic seizure lasting a few minutes. She had
been diagnosed with TA 3 months earlier, based on the
American College of Rheumatology criteria. On that occasion, she had complained of low-grade fever, fatigue, and
abdominal pain. Left radial arterial pulsations were diminished, and blood pressure in her left arm was reduced (right:
126/64 mmHg, left: 104/48 mmHg). Laboratory findings
were as follows: microcytic anemia (hemoglobin: 8.1 g/dL),
increased erythrocyte sedimentation rate (130 mm/hr) and
C-reactive protein (8.03 mg/dL). Contrast-enhanced com-

１

Department of Neurology, Nagoya City University, Japan, ２Department of Radiology, Nagoya City University, Japan and ３Department of Medical Oncology and Immunology, Nagoya City University, Japan
Received for publication January 20, 2011; Accepted for publication April 4, 2011
Correspondence to Dr. Noriyuki Matsukawa, norim@med.nagoya-cu.ac.jp

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DOI: 10.2169/internalmedicine.50.5185

Figure 1. Contrast-enhanced CT at diagnosis of Takayasu’s arteritis showed stenotic lesions of the
thoracic and abdominal aorta (A) and its main branches, the common carotid artery (B), and the bilateral renal arteries (C). Arrows indicate radiological stenotic lesions. A: coronal sequence of aorta.
B: axial sequence of right common carotid artery. C: coronal sequence of renal arteries. L: left, R:
right

puted tomography (CT) revealed stenosis of the bilateral renal arteries and thickening of the abdominal artery walls
(Fig. 1). When she presented at our outpatient clinic 1
month before the current headache event, the active inflammation of TA was suppressed and her medical condition was
stable under treatment with prednisolone at a dose of 30
mg/day.
On arrival at the emergency department, a physical examination showed normal temperature, and hypertension
with a bilateral difference in blood pressure (right: 181/78
mmHg, left 152/64 mmHg). She was alert, and focal neurologic signs were absent. The results of laboratory examinations, including inflammatory reaction, electrolytes and renal function, were normal. An initial cerebrospinal fluid
(CSF) examination was also normal. CT of her head showed
no parenchymal abnormalities, such as subarachnoid hemorrhage or cerebral hemorrhage.
Her hypertension had diminished the following day,
whereas her headache remained. No further seizures developed after admission. Brain magnetic resonance (MR) imaging using fluid-attenuated inversion recovery (FLAIR)
showed diffuse hyperintense bilateral cortical and subcortical
regions (Fig. 2A, B). MR angiography (MRA) also revealed

proximal narrowing of the right posterior cerebral artery
(Fig. 2C). We considered cerebral artery vasoconstriction to
be related to her acute hypertension, although angiitis of the
central nervous system could not be completely ruled out.
We observed her under continuous treatment with 30 mg
prednisolone, based on an absence of neurologic symptoms,
no fever, and normal CSF findings. On day 10 after her hospital admission, she had a recurrence of severe thunderclap
headache with increased blood pressure, while straining during a bowel movement (right: 220/124 mmHg). On day 13,
repeated MRA revealed an increase in the number of narrowed regions in the bilateral middle and posterior cerebral
arteries, although the overall high intensity on FLAIR had
disappeared (Fig. 2D, E, F). An ultrasound study of the internal carotid artery revealed no thickening of the vessel
wall, although the wall of the common carotid artery was
thickened on contrast-enhanced CT at TA diagnosis. Transcranial Doppler showed markedly increased blood flow velocity with turbulence in the right middle cerebral artery
(mean blood flow velocity: right 134 cm/s, left 82 cm/s).
Heparin was intravenously administered, and her systolic
blood pressure was controlled below 150 mmHg with nimodipine. Within 2 weeks of starting treatment, her head-

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DOI: 10.2169/internalmedicine.50.5185

Figure 2. Initial fluid-attenuated inversion recovery magnetic resonance (MR) image on day 3
showed hyperintense signals in the cortical and subcortical white matter, involving the bilateral cerebellar hemispheres, bilateral-occipital lobes and right frontal lobe (A, B). MR angiography (MRA)
showed proximal narrowing of the right posterior cerebral artery (arrow) (C). Repeated MRA on
day 13 revealed progressive luminal narrowing in multiple regions (arrows), although the overall
parenchymal abnormality had disappeared (D, E, F). The cerebral narrowing in these areas was improved on follow-up MRA on day 29 (G).

ache gradually improved. MRA was significantly improved
by day 29 after admission (Fig. 2G), and the blood flow velocity fell to normal (mean blood flow velocity: right 102
cm/s, left 81 cm/s). MRA revealed normal findings for all
intracranial vessels 3 months after onset.

Discussion
The present case can be summarized as follows: a teenage
girl receiving prednisolone therapy presented with thunderclap headache, decreased consciousness, convulsion and hypertension. Head MR imaging showed abnormal signals in
the brain parenchyma and narrowing of the cerebral arteries,
suggesting radiologic posterior reversible encephalopathy

syndrome (PRES) and RCVS. The narrowing of the cerebral
arteries progressed for 1 month, after which her symptoms
rapidly improved and the abnormal signal detected by MR
imaging diminished. To our knowledge, this is the first case
of RCVS presenting in a patient with TA.
The criteria for RCVS were initially proposed by
Calabrese et al (1) in 2007, as follows: i) transfemoral angiography or indirect CT angiography or MRA documenting
multifocal segmental cerebral artery vasoconstriction; ii) no
evidence of aneurysmal subarachnoid hemorrhage; iii) normal or near-normal CSF analysis; iv) severe, acute headache, with or without additional neurologic signs or symptoms; and v) reversibility of angiographic abnormalities
within 12 weeks after onset. The symptoms and clinical data

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DOI: 10.2169/internalmedicine.50.5185

in the current patient matched all five of these original criteria. However, these criteria were unable to completely exclude the narrowing of intracranial cerebral arteries caused
by primary angiitis-related TA in this case.
TA is an idiopathic granulomatous vasculitis that affects
the aorta and its main branches. Approximately 10-15% of
patients with TA have ischemic stroke or transient ischemic
attack. These strokes have mainly been attributed to stenotic
extracranial vessels, caused by granulomatous vasculitis,
whereas two cases of TA were reported as strokes involving
the intracranial arteries (4, 6). The present case presented
with stable symptomatic TA, with normal serological data
and CSF findings. Moreover, an ultrasound study of the internal carotid artery revealed no thickening of the vessel
wall. These observations suggested that the TA activity in
this case was adequately suppressed and clinically controlled. The radiologically-detected intracranial cerebral artery narrowing had disappeared within several weeks, possibly suggesting that it was not related to the inflammatory
reaction. This case was complicated by acute-onset headache
and seizure, with increased blood pressure; patients with
RCVS often complain of neurologic symptoms and a rapid
rise in blood pressure. Thus, RCVS, rather than angiitis related to TA, might have been involved in the narrowing of
the intracranial cerebral arteries in the current case.
PRES is a new clinicoradiologic entity characterized by
headache, confusion, visual disturbance, seizures and transient reversible changes on MR imaging in the cortical and
subcortical white matter of the parieto-occipital lobes (7, 8).
PRES has previously been described in several conditions,
including hypertensive encephalopathy, pre-eclampsia, eclampsia, infections, electrolyte imbalance, hypercalcemia
and use of several therapeutic agents (7). Vasogenic edema
based on endothelial dysfunction, occurring as a result of
acutely elevated blood pressure exceeding the autoregulatory
capacity of the brain vasculature, may be an essential factor
in the development of PRES. In the current case, the neuroradiological finding and clinical course were consistent with
the disease concept of PRES. The development of endothelial dysfunction in patients with TA might account for the
ischemic signals and high intensity on FLAIR images, although the involvement of anti-endothelial antibody in TA
remains unknown.

RCVS and PRES may have similar underlying mechanisms and may represent overlapping clinical entities, although they are cliniconeuroradiologically defined as distinct
entities, for descriptive purposes. The rapid increase in
blood pressure in the present case may, at least in part, have
also played a crucial role in triggering vasoconstriction. Interestingly, patients with TA may present a clinical course
similar to RCVS. An identical pathogenesis, inducing intracranial artery vasoconstriction, may also present different radiologic findings, with different regions of vasoconstriction
and degrees of ischemic signals in the brain parenchyma, as
in the two headache attacks in the present case.
In conclusion, we herein report a patient with TA complicated by RCVS. When a patient with TA presents with thunderclap headache with vascular abnormalities in intracranial
vessels, the transient reactive vascular abnormality, RCVS,
should be considered as a more likely differential diagnosis
than organic stenotic granulomatous vasculitis.
The authors state that they have no Conflict of Interest (COI).

References
1. Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern
Med 146: 34-44, 2007.
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Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 33: 1129-1134, 1990.
4. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann
Intern Med 120: 919-929, 1994.
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7. Fujita M, Komatsu K, Hatachi S, Yagita M. Reversible posterior
leukoencephalopathy syndrome in a patient with Takayasu arteritis. Mod Rheumatol 18: 623-629, 2008.
8. Hinchey J, Chaves C, Appignani B, et al. A reversible posterior
leukoencephalopathy syndrome. N Engl J Med 334: 494-500,
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Ⓒ 2011 The Japanese Society of Internal Medicine
http://www.naika.or.jp/imindex.html

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