Takayasu's arteritis is an idiopathic inflammatory disease of the large elastic arteries occurring in the young resulting in occlusive or ectatic changes mainly in the aorta and its immediate branches as well as the pulmonary artery and its branches. The disease is common in women in the second and third decades of life. Stroke as the first manifestation of Takayasu's disease is relatively rare. However 10–20% patients with Takayasu's arteritis can have a primary cerebrovascular presentation with headaches, seizures, transient ischemic attacks, strokes or intra-cerebral hemorrhage. We report a case of a 39-year-old lady who developed a stroke and was diagnosed as Takayasu's arteritis. This patient had fulfilled three of six criteria for Takayasu's arteritis based on The American College of Rheumatology. She responded to steroids and immune suppressive therapy. Stroke as the first presentation of Takayasu's arteritis is relatively rare and only a few instances have been reported in the literature. Our patient had bilateral carotid occlusion and collaterals between the vertebral artery and external carotid arteries. She presented with right hemiplegia and after diagnosis she was promptly treated with prednisolone, methotrexate and other supportive measure. The patient had good clinical recovery.

1.
Str
roke as th
he first m
a
manifestati
arteritis
ion of Takkayasu’s

2. Case Report
Stroke as the first manifestation of Takayasu’s
arteritis
Pushpendra Renjen*, Laxmi Khanna, Cecilia Fernandes, Nadeem Khan
Department of Neurology, Indraprastha Apollo Hospital, India
a r t i c l e i n f o
Article history:
Received 17 July 2013
Accepted 8 August 2013
Available online 8 September 2013
Keywords:
Stroke
Neurology
Vasculitis
Rheumatology
a b s t r a c t
Takayasu’s arteritis is an idiopathic inflammatory disease of the large elastic arteries
occurring in the young resulting in occlusive or ectatic changes mainly in the aorta and its
immediate branches as well as the pulmonary artery and its branches. The disease is
common in women in the second and third decades of life. Stroke as the first manifestation
of Takayasu’s disease is relatively rare. However 10e20% patients with Takayasu’s arteritis
can have a primary cerebrovascular presentation with headaches, seizures, transient
ischemic attacks, strokes or intra-cerebral hemorrhage. We report a case of a 39-year-old
lady who developed a stroke and was diagnosed as Takayasu’s arteritis. This patient had
fulfilled three of six criteria for Takayasu’s arteritis based on The American College of
Rheumatology. She responded to steroids and immune suppressive therapy.
Stroke as the first presentation of Takayasu’s arteritis is relatively rare and only a few
instances have been reported in the literature. Our patient had bilateral carotid occlusion
and collaterals between the vertebral artery and external carotid arteries. She presented
with right hemiplegia and after diagnosis she was promptly treated with prednisolone,
methotrexate and other supportive measure. The patient had good clinical recovery.
Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved.
1. Case presentation
Our patient was a 39-year-old housewife who developed
sudden onset right sided weakness with inability to speak.
She had no past history of coronary artery disease, valvular
heart disease, strokes, irregular fever, joint pains or other
systemic features. She was a hypertensive for the last two
years on irregular medication.
Examination revealed middle-aged lady, afebrile, no pallor,
no lymphadenopathy or pedal edema. All peripheral pulses
(radial, brachial, superficial temporal, femoral, popliteal and
posterior tibial) except the carotid artery pulsations were
palpable. Blood pressure in the right upper limb was 200/
110 mm Hg, in the left upper limb was 200/110 mm Hg and in
the right lower limb was 220/210 mm Hg. There was no bruit
heardover the carotidarteriesandtherewerenorenal vascular
bruits. Cardiovascular examination showed a grade II ejection
systolic murmur over the aortic area. Central nervous system
examination showed a normal higher mental status, optic
fundi did not reveal any abnormality; she had motor aphasia
with a dense right hemiplegia (Power by Medical Research
Council grade 1/5 in the right upper and right lower limb).
2. Investigations
Hematological evaluation revealed an ESR 110 mm/h, total
leukocyte counts of 12,000 and C-reactive protein was
* Corresponding author.
E-mail address: pnrenjen@hotmail.com (P. Renjen).
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/apme
a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 2 5 1 e2 5 3
0976-0016/$ e see front matter Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.apme.2013.08.018

3. 90.2 ng. X-ray chest and ECG were normal. Rheumatoid
factor, anti-nuclear antibody, anti Ds-DNA, lupus anticoag-
ulant, anti-cardiolipin antibodies, anti-thrombin III were
absent. Protein C, protein S and Factor V Leiden were
normal. Echocardiography revealed mitral valve thickening,
mild mitral regurgitation, thick aortic valve, moderate AR,
no AS, basal posterolateral wall hypokinesia and an ejection
fraction of 45%. Carotid Doppler showed bilateral carotid
artery block, normal subclavian arteries, prominent right
and left vertebral arteries. CT angiography showed multiple
vessels involvement with numerous collaterals indicating
long-standing occlusion. Magnetic resonance imaging of the
brain showed infarction in the left middle cerebral artery
territory. Digital subtraction angiography revealed complete
occlusion of right and left common carotid arteries in the
neck (Fig. 1A), bilateral vertebral arteries were dilated,
muscular collaterals till the external carotid artery retro-
gradely descending up to the bifurcation and ascending
upwards (Fig. 1B). Irregular luminal narrowing of the
thoracic aorta and lower half of the abdominal aorta,
irregular narrowing of the left renal artery near origin
(Fig. 2). Thoracic, abdominal and right renal arteries were
normal.
3. Treatment
The patient was treated with steroids, anticoagulants,
immunosuppressive drugs and regular physiotherapy.
4. Outcome and follow-up
The patient was followed up regularly after discharge. There
was gradual improvement in her motor power; by the end of
three months there was complete motor recovery.
5. Discussion
Aortoarteritis has multivessel involvement with frequent
involvement of the arch of the aorta and its branches at their
points of origin.1
Affected arteries being the subclavian (90%),
carotid (45%), vertebral (25%), and renal (20%).1
Occlusion of
the vertebral or carotid arteries may cause ischemic stroke.2
Takayasu’s arteritis characteristically involves the subcla-
vian arteries as by the 1990 criteria of The American College of
Rheumatology.3
But common carotid artery occlusion is also
known to occur.4,5
The natural history of the illness has been
described in three phases.1
The early and pulseless phase with
systemic symptoms followed by the phase of active vascular
inflammation and finally the chronic phase with fibrotic and
stenotic lesions.1
Neurological symptoms like headache,
depression, syncope, hemiplegia and visual disturbances
occur in the chronic phase of the illness.2,4,6
10e20% of pa-
tients with Takayasu’s arteritis patients can present with
ischemic stroke due to thrombosis or embolism.2,4
The in-
flammatory disease primarily affects the media or the
adventitia of the vessel walls resulting in luminal abnormal-
ities like stenosis, occlusion or aneurysm formation.4
Often
the arteries are affected over long segments on both sides.4
Intracranial stenosis in Takayasu’s arteritis could be due to
vasculitic involvement or due to prior embolization in to the
vessel.2,4
By using transcranial Doppler sonography, Kumral
et al have detected microembolic signals in the middle
Fig. 1 e Digital subtraction angiography showing (A)
complete occlusion of both common carotid arteries in the
neck, (B) dilated right vertebral artery. 271 3 155 mm
(72 3 72 DPI).
Fig. 2 e Digital subtraction angiography showing irregular
narrowing of left renal artery at its origin.
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4. cerebral arteries.7
Involvement of the cardiac valves and
proximal arteries may provide a source of embolism in
Takayasu’s arteritis.4
Other postulated mechanisms for stroke
in Takayasu’s arteritis are stenoocclusive extra cranial ves-
sels, hypertension and premature atherosclerosis.2
The cere-
bral hemodynamics and metabolism in patients of Takayasu’s
arteritis with neurological symptoms are altered.2
Using duplex ultrasonography, it was shown that the
subclavian arteries were involved bilaterally in 33% and on
either side in 67%, and the carotid system involvement was
69%.5
The carotid lesion in Takayasu’s arteritis can be seen
clearly by B mode ultrasonography.5
The vascular lesions are
often homogenous in density, long segment involvement
with concentric or circumferential thickening and these are
located in the proximal to middle segment of the common
carotid artery.5
In transverse section, the circumferentially
thickened intima media complex is termed as macaroni sign.
In Takayasu’s arteritis, the artery undergoing partial occlusion
will acquire a new large and organized lumen, called the
‘vesseleinvessel’ phenomenon, which is not present in sys-
temic arteritis.5
In a young patient, affection of the aorta,
subclavian or common carotid artery is suggestive of
Takayasu’s arteritis.1,4
Isolated common carotid artery oc-
clusion with reversed external carotid artery flow to the pat-
ent internal carotid artery is not an infrequent finding in
Takayasu’s arteritis.5
Our patient had bilateral complete occlusion of right and
left common carotid arteries in the neck, dilated bilateral
vertebral arteries with muscular collaterals extending till the
external carotid artery retrogradely, then descending up to the
bifurcation and ascending upwards (Fig. 1A & B). The inade-
quate cerebral circulation contributed to ischemic stroke. She
also had left renal artery stenosis (Fig. 2) leading to renovas-
cular hypertension. The management of Takayasu’s arteritis
consists of glucocorticoids in high doses which were tapered
to maintenance doses with the addition of immunosuppres-
sant drugs like cyclophosphamide, azathioprine or metho-
trexate.2,5,8
Surgical treatment includes angioplasty with
stenting to maintain a patent lumen or a renal artery bypass
(revascularization).8
Our patient responded to prednisolone,
methotrexate and antihypertensives.
The gold standard for diagnosis is angiography.1,6
Ultra-
sound, computerized tomography and magnetic resonance
angiography (MRA) may be used for diagnosis of Takayasu’s
arteritis.4,6
Neurological involvement in Takayasu’s arteritis
can include dizziness, headache, syncope, cranial nerve
palsies and mental decline.2
Stroke as the first presentation of Takayasu’s arteritis is
relatively rare and only a few instances have been reported in
the literature. Our patient had bilateral carotid occlusion and
collaterals between the vertebral artery and external carotid
arteries. She had involvement of the aortic branches, the
thoracic aorta, lower half of the abdominal aorta and the left
renal artery suggestive of a Type III disease.9
She presented
with right hemiplegia and after investigations she was treated
as a case of Takayasu’s arteritis with steroids, immune sup-
pressants and other supportive therapy. Patient had a good
clinical recovery. Takayasu’s arteritis is a treatable cause of
stroke in the young and if diagnosed early serious complica-
tions can be prevented.
6. Learning points/take home messages
, One should suspect vasculitis in any young patient with
stroke.
, Takayasu’s arteritis is a treatable cause of stroke.
, If diagnosed early, serious complications can be prevented
and patients may recover completely.
Conflicts of interest
All authors have none to declare.
r e f e r e n c e s
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2. Vidhate M, Garg RK, Yadav R, et al. An unusual case of
Takayasu’s arteritis: evaluation by CT angiography. Ann Indian
Acad Neurol. 2011;14:304e306.
3. Arrend WP, Micheal BA, Bloch DA, et al. The American College
of Rheumatology 1990 criteria for the classification of
Takayasu arteritis. Arthritis Rheum. 1990;33:1129e1134.
4. Ringleb PA, Strittmatte EI, Loewer M, et al. Cerebrovascular
manifestations of Takayasu arteritis in Europe. Rheumatology
(Oxford). 2005;44:1012e1015.
5. Sun Yu, Yip PK, Jeng JS, et al. Ultrasonographic study and long-
term follow-up of Takayasu’s arteritis. Stroke.
1996;27:2178e2182.
6. Cantu C, Pineda C, Barinagarrementeria F, et al. Noninvasive
cerebrovascular assessment of Takayasu arteritis. Stroke.
2000;31:2197e2202.
7. Kumral E, Evyapan D, Aksu K, et al. Microembolus detection in
patients with Takayasu’s arteritis. Stroke. 2002;33:712e716.
8. Panja M, Mondal PC. Current status of aortoarteritis in India.
J Assoc Physicians India. 2004;52:48e52.
9. Ueno A, Awane Y, Wakabayashi A, et al. Successfully operated
obliterative brachiocephalic arteritis (Takayasu) associated
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