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Case Report

Stroke as the first manifestation of Takayasu’sarteritis

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

* Corresponding author.E-mail address: pnrenjen@hotmail.com (

0976-0016/$ e see front matter Copyright ªhttp://dx.doi.org/10.1016/j.apme.2013.08.018

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 the right lower limb was 220/210 mm Hg. There was no bruit

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 revealedmiddle-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

P. Renjen).2013, Indraprastha Medic

heardover thecarotidarteriesand therewerenorenalvascular

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

al Corporation Ltd. All rights reserved.

Fig. 2 e Digital subtraction angiography showing irregular

narrowing of left renal artery at its origin.

a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 2 5 1e2 5 3252

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.

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).

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 phasewith

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

a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 2 5 1e2 5 3 253

cerebral arteries.7 Involvement of the cardiac valves and

proximal arteries may provide a source of embolism in

Takayasu’s arteritis.4 Other postulatedmechanisms for stroke

in Takayasu’s arteritis are stenoocclusive extra cranial ves-

sels, hypertension and premature atherosclerosis.2 The cere-

bral hemodynamics andmetabolism 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

1. Chaubal N, Dighe M, Shah M. Sonographic and color Dopplerfindings in aortoarteritis (Takayasu arteritis). J Ultrasound Med.2004;23:937e944.

2. Vidhate M, Garg RK, Yadav R, et al. An unusual case ofTakayasu’s arteritis: evaluation by CT angiography. Ann IndianAcad Neurol. 2011;14:304e306.

3. Arrend WP, Micheal BA, Bloch DA, et al. The American Collegeof Rheumatology 1990 criteria for the classification ofTakayasu arteritis. Arthritis Rheum. 1990;33:1129e1134.

4. Ringleb PA, Strittmatte EI, Loewer M, et al. Cerebrovascularmanifestations 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. Noninvasivecerebrovascular assessment of Takayasu arteritis. Stroke.2000;31:2197e2202.

7. Kumral E, Evyapan D, Aksu K, et al. Microembolus detection inpatients 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 operatedobliterative brachiocephalic arteritis (Takayasu) associatedwith the elongated coarctation. Jpn Heart J. 1967;8:538e544.

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