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 Table of Contents  
Year : 2021  |  Volume : 9  |  Issue : 2  |  Page : 118-121

Thrombolysis beyond the time window in takayasu's arteritis: A challenging experience

1 Department of Neurology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India
2 Department of Radiology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India

Date of Submission25-Oct-2021
Date of Decision10-Jan-2022
Date of Acceptance14-Jan-2022
Date of Web Publication5-Apr-2022

Correspondence Address:
Dr. Madhavi Karri
Department of Neurology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcvs.jcvs_20_21

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Takayasu's arteritis (TA) is chronic inflammatory vasculopathy preferentially affecting the aorta and its major branches. It is one of the potential causes of stroke in young adults. Here, we report a young female who presented to us with an acute-onset right hemiparesis with motor aphasia after 10 h of symptoms onset. Her National Institute of Health Stroke Scale (NIHSS) score at admission was 12. The stroke imaging showed acute infarct in the left middle cerebral artery territory with arterial spin labelling perfusion sequence showing diffusion perfusion mismatch with good penumbra. She underwent intravenous thrombolysis with tenecteplase, based on imaging findings and despite being out of window period. She had an excellent clinical recovery and was discharged with oral anticoagulant and azathioprine. After 3 months of stroke and recovery, follow-up was unremarkable with minimal residual deficits with an NIHSS score of 4 and Modified Rankin Scale of 1. Hence, appropriate early interventions in acute stroke in TA individuals are highly beneficial and timely considered for a good outcome and better quality of life.

Keywords: Ischaemic stroke, Takayasu's arteritis, thrombolysis

How to cite this article:
Karri M, Ramasamy B, Swamiappan E, Perumal S, Kannan KT. Thrombolysis beyond the time window in takayasu's arteritis: A challenging experience. J Cerebrovasc Sci 2021;9:118-21

How to cite this URL:
Karri M, Ramasamy B, Swamiappan E, Perumal S, Kannan KT. Thrombolysis beyond the time window in takayasu's arteritis: A challenging experience. J Cerebrovasc Sci [serial online] 2021 [cited 2022 May 22];9:118-21. Available from: http://www.jcvs.com/text.asp?2021/9/2/118/342557

  Introduction Top

Takayasu's arteritis (TA) is an inflammatory disease involving large- and medium-sized arteries with a strong predilection to the aortic arch and its branches. It occurs more commonly in young females in their second or third decade.[1]. The estimated incidence was 2.6 cases per million per year. The disease is more common in Asian countries with the highest prevalence of 40 per million was estimated in Japan.[2] Neurological manifestations constitute about 10%–20% of patients with TA.[3] Thrombolysis in acute stroke presentation in TA beyond the time window has not been reported. Here, we present a young female with TA, who underwent thrombolysis beyond the window period of 4.5 h after presenting in the emergency department, 2-day post-ictus with a good outcome.

  Case Report Top

We are presented with a 23-year-old female to our emergency department with right hemiparesis with motor aphasia for 3 am on 8th May 2020 (10 h after the onset of symptoms). She was conscious, alert with a Glasgow Coma Scale of 10/15 (E4VAM5), obeying commands. Her pulse rate was 85 per min, with peripheral arterial pulses in the left upper limb being absent. Her blood pressure in the right upper limb was 100/50 mm of Hg, the lower limb was 90/50 mm of Hg, whereas the left upper limb pulse was feeble with a systolic blood pressure of 40 mm of Hg and left lower limb showed 80/40 mm of Hg. There was no bruit in the bilateral carotid artery. Neurological examination showed motor aphasia with weakness of left side extremities with the motor strength of 3 over 5 and positive left Babinski's sign. The rest of the examination was normal. National Institute of Health Stroke Scale score at admission was 12 out of 42.

She was diagnosed with TA by an outside physician in November 2019. Since then, she was started on steroids and azathioprine. Doppler of the left upper showed complete occlusion of left subclavian artery with reduced velocity in axillary, brachial, radial and ulnar arteries and normal flow in the left common carotid artery. No history of similar illness in the family. She presented after 10 h of symptoms onset. She was out of the window period. Magnetic resonance imaging (MRI) of the brain showed small core acute infarct in the left basal ganglia (left lentiform nucleus and caudate nucleus) extending to the left corona radiate with preserved left internal capsule [Figure 1]c. Magnetic Resonance (MR) intra cranial angiogram showed loss of flow signal in left internal carotid artery, left middle cerebral artery and its distal branches with no evidence of collateral supply to left middle cerebral artery territory region [Figure 1]i. MR angiogram of extracranial vessels showed diffuse wall thickening in the arch of the aorta suggestive of Takayasu's arteritis, along with thrombotic occlusion and absent flow in left subclavian, left common carotid, left external and internal carotid arteries indicating the presence of active inflammation [Figure 1]a and [Figure 1]b. MRI Aspects Score was 8 out of 10) [Figure 1]c and [Figure 1]h. MRI aspects score was 8 out of 10) (figure diffusion-weighted imaging [DWI]).
Figure 1: (a) Magnetic resonance extracranial angiogram showed concentric wall thickening of aortic arch suggestive of Takayasu arteritis. (b) The left common carotid and the extracranial segment left internal carotid artery were enlarged with absent flow suggestive of active inflammation. (c) Diffusion-weighted imaging revealed a small core infarct involving the left lentiform nucleus, caudate nucleus. (d) Prominent susceptibility weighted imaging signal in the cortical veins of the left middle cerebral artery territory, suggestive of viable but ischaemic cortical tissue – called “susceptibility-weighted imaging penumbra sign”. (e) Susceptibility-weighted imaging susceptibility-weighted imaging showed blooming involving the proximal left middle cerebral artery (M1 segment) – called “ Susceptibility weighted imaging blooming sign”. (f) No evidence of collateral supply to the left middle cerebral artery territory was noted. (g) Arterial spin labelling showed a large ischaemic territory and were compared with diffusion-weighted images (c and h) which revealed a small core infarct. (i) Magnetic resonance intracranial angiogram revealed complete loss of flow signal in the left internal carotid artery, left middle cerebral artery and its distal branches

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Considering clinical history, proximal occlusion was considered to be chronic in nature. Susceptibility weighted imaging (SWI) came to our rescue in this situation. SWI showed blooming in proximal left middle cerebral artery (M1 segment) – so called “SWI blooming sign” [Figure 1]d suggestive of acute thrombosis of the involved segment. We also noted prominent SWI signal in the cortical veins of the left middle cerebral artery territory, suggestive of viable but ischemic cortical tissue – so called “SWI Penumbra sign” [Figure 1]e and [Figure 1]f. Arterial spin labelling (ASL) perfusion sequence showed a large territory ischemia in left frontal, high parietal and left temporal lobes with diffusion perfusion mismatch – indicating a large penumbra of viable tissue with ongoing ischemia [Figure 1]g and [Figure 1]h. She was thrombolysed with tenecteplase (dose 16 mg) with a door to needle time of 30 min and stroke onset to needle time of 600 min. Mechanical thrombectomy was ruled out based on imaging as no access to the vessel was present. Following 2 days later, she improved dramatically with normal speech and the limb power of 2 out of 5 and was discharged in a wheelchair. Post-thrombolysis computed tomography showed temporal evolution of infarct without any progression or complications noted. After 3 months of follow-up, she was able to walk without support (power of 4 out of 5) with mild dysarthria.

  Discussion Top

TA, also known as pulseless disease, is a rare and potentially devastating arteriopathy. It mainly affects the large vessels, aorta and its branches in particular. Varied neurological manifestations observed in TA, include headache, visual disturbances, seizures, giddiness and multiple cranial nerve palsies. TA has an aetiological association with stroke in young. Transient ischaemic attacks, stroke and posterior reversible encephalopathy syndrome are known to occur, concerning to cerebrovascular pathology. There are two known pathophysiologies in the occurrence of ischaemic stroke. Thromboembolic events from a stenosed vessel or haemodynamic compromise are presumed to cause a stroke in patients with TA. Ischaemic strokes more commonly occur in the middle cerebral artery territory.[4] Infarcts in the internal carotid artery regions (cortical watershed region) occur after a prolonged period of decreased blood flow.[5] As TA includes young people, conventional atherosclerosis has no role in the underlying mechanism.

In out of window period ischaemic strokes, imaging helps in quantifying the infarct core (small versus large core) and also in evaluating the presence of salvageable brain tissue (penumbra). ASPECTS score (plain computed tomography [CT]) and DWI are good predictors for infarct core.[4] Penumbra is best identified using CT or ASL of non-contrast MRI perfusion.[6] The presence of acute red blood cell-rich clot in proximal middle cerebral artery M1 segment was indicated by the 'SWI clot sign'.[7]

Thrombolysis in TA with an acute stroke is not a much-described entity. Few cases have reported with acute stroke in TA treated with thrombolysis and mechanical embolectomy.[6],[7] Inflammation of the vessels predisposes them to stenosis, occlusion and thrombosis in the affected vessels. Although the pathophysiology is slightly different from atherosclerosis, the treating physicians, treatment with thrombolysis and mechanical thrombectomy need to be given a thought in an acute stroke in patients with TA. Based on imaging characteristics, vessel morphology and type of stroke, the management decision can be taken accordingly.

Here, we report the first case of out of window thrombolysis in TA patients. In our case, we preferred thrombolysis over antiplatelet therapy even beyond the time window, depending the on penumbra and salvageable brain tissue noted on brain imaging. Mechanical thrombectomy would be an ideal treatment option in this circumstance; however, accessibility to the occluded vessels to reach the clot excluded the possibility. Hence, based on the recent guidelines, intravenous thrombolysis was administered and turned out to have a very positive and successful outcome. Decision-making in acute scenarios along with appropriate immunosuppressive therapy is a valuable key in maintaining the disease in remission and prevent long-term complications.

  Conclusion Top

TA is a rare systemic disorder often is under-recognised or diagnosed late. Stroke is an acute neurological presentation, as an initial or as a disease consequence can be observed. Early diagnosis and active management are effectual with better morbidity and quality of life.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Numano F. The story of takayasu arteritis. Rheumatology (Oxford) 2002;41:103-6.  Back to cited text no. 1
Onen F, Akkoc N. Epidemiology of takayasu arteritis. Presse Med 2017;46:197-203.  Back to cited text no. 2
Klos K, Flemming KD, Petty GW, Luthra HS. Takayasu's arteritis with arteriographic evidence of intracranial vessel involvement. Neurology 2003;60:1550-1.  Back to cited text no. 3
Yong SW, Bang OY, Lee PH, Li WY. Internal and cortical border-zone infarction: Clinical and diffusion-weighted imaging features. Stroke 2006;37:841-6.  Back to cited text no. 4
Howard R, Trend P, Russell RW. Clinical features of ischemia in cerebral arterial border zones after periods of reduced cerebral blood flow. Arch Neurol 1987;44:934-40.  Back to cited text no. 5
Béjot Y, Couvreur G, Ricolfi F, Osseby GV, Cochet A, Giroud M. Acute cerebrovascular manifestation of takayasu arteritis. Am J Med 2011;124:5-6.  Back to cited text no. 6
Hedna VS, Patel A, Bidari S, Elder M, Hoh BL, Yachnis A, et al. Takayasu's arteritis: Is it a reversible disease? Case report and literature review. Surg Neurol Int 2012;3:132.  Back to cited text no. 7
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