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Stroke as the first manifestation of Takayasu's arteritis

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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 …

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.

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  • 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. 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 3252
  • 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 1. Chaubal N, Dighe M, Shah M. Sonographic and color Doppler findings in aortoarteritis (Takayasu arteritis). J Ultrasound Med. 2004;23:937e944. 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 with the elongated coarctation. Jpn Heart J. 1967;8:538e544. 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 3 253
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