Syncopal Seizure: A Rare Manifestation of Takayasu Arteritis

Dear Editor, Takayasu’s arteritis is a chronic vasculitis affecting large- and medium-sized vessels.[1] It is mostly seen in woman less than 40 years of age.[2] Most common vessels involved are renal arteries, subclavian arteries and branches of arch of aorta, superior mesenteric artery, femoral and iliac arteries.[2] A 15 years old girl with no comorbidities presented to us with history of multiple episodes of transient loss of consciousness lasting for about few minutes over six months. The patient was evaluated by multiple clinicians and patient’s relatives described the semiology as loss of postural control followed by fall. For above-mentioned complaints, she was receiving antiseizure medications (ASM) without any significant improvement. The doses of ASMs were increased in every follow-up and at the end of six months, she was on four ASMs at optimal doses and good compliance (Levetiracetam 40 mg/kg/day, sodium valproate 20 mg/kg/day, Topiramate 6 mg/kg/day; all in two divided doses and clobazam 0.25 mg/kg/day once daily at evening). She also had a history of recurrent vertiginous sensation while writing during examination along with tingling and numbness over the right upper limb. Similar vertiginous sensation was also noted while using her left hand as well. She had history of significant weight loss over the last six months. On eliciting history further, she mentioned blackout and palpitation preceding the loss of consciousness. There were few episodes of bladder incontinence without frothing from mouth or tongue bite. Events resolved within 3-5 minutes with post-ictal headache without any residual focal neurological deficit, but associated with significant perspiration. Most of the events were during day while she was active doing some work or in standing position. There were no events reported during sleep. On examination, bilateral radial pulsation was absent. Right lower limb pulses were feeble. Bruits noted over right carotid and right renal artery. Blood pressure was not recordable over bilateral upper limbs and was 170/90 in left lower limb. There was no organomegaly on palpation and no focal neurological deficit. Routine blood examination revealed mild anaemia with haemoglobin 10.2 gm/dl with elevated erythrocyte sedimentation rate 36 mm/hour and elevated C-reactive protein level 10.82 mg/dl. Other biochemical parameters including liver and renal function tests were normal. Cardiac workup including ECG, 2d Echocardiography, and holter study was normal. Autonomic function tests were avoided due to significant compromised vascular status (both cranial and appendicular) on examination itself. Electroencephalography revealed normal study. His magnetic resonance imaging (MRI) brain was essentially normal. In view of significant bruits in carotid and renal artery along with pulseless radial arteries, computer tomography (CT) angiography of aorta and its major vessels was done which showed complete stenosis of right common carotid artery and both subclavian arteries at their origin [Figure 1a and b]. Extensive collaterals were also present. Infrarenal aorta was also narrowed with up to 60% stenosis. The superior mesenteric artery and right renal artery were also stenosed [Figure 1c].Figure 1: Computer tomography angiogram (CTA) done showing significant stenosis of both left and right common carotid artery proximally, with bilateral subclavian complete stenosis with extensive collaterals (Figure a, black double head arrow and figure b white arrow). There was arrowing in right common iliac and internal iliac artery, abdominal aorta narrowing infrarenal (D9-L2) luminal stenosis around 60% (figure a black arrow and figure c white arrow head). Final diagnosis was made as Type V Takayasu arteritis. In postpercutaneous CTA, a stent is seen in the origin of right renal artery with adequate contrast flow beyond the stent (figure d, white arrow). An another stent noted from right innominate to vertebral artery stenting with adequate contrast flow distal to right vertebral artery (figure d, black arrow). Right common carotid not visualised in proximal 2 cm with good contrast opacification beyond it via multiple collateral channels (Figure d)She was diagnosed as Takayasu arteritis grade V as per 2022 ACR/EULAR classification criteria for Takayasu arteritis[3] with syncopal atonic seizure. She was started with pulse Methylprednisolone 1 gram intravenous daily for 5 days followed by oral prednisolone 1 mg/kg/day. She was also started with methotrexate 7.5 mg orally once weekly which was stepped up to 25 mg once weekly along with folate rescue. She also received antihypertensive medication cilnidipine 10 mg twice daily and Clonidine 0.1 mg three times a day, with regular blood pressure monitoring at lower limb. Gradually all antiseizure medications were tapered off and stopped. Patient was also explained about her disease and explained about the risk of syncopal events during sudden neck movement. No new events recorded in 6 months of follow-up. Two month back, patient received balloon angioplasty along with stent placement of right renal artery [Figure 1d]. Post-procedure the requirement of antihypertensive drugs was reduced significantly to cilnidipine 10 mg once daily. After discussion with family members, she underwent carotid angioplasty and right innominate to vertebral artery stenting, by interventional radiologist [Figure 1d]. Patient received dual antiplatelet (Aspirin with clopidogrel) before start of percutaneous intervention for 6 weeks, which was reduced to single antiplatelet (Aspirin 75 mg/day). Till last follow-up patient was stable and no new syncopal events were recorded. Discussion Transient loss of consciousness often causes diagnostic dilemma to the physicians as well as neurologist. Cerebral hypoperfusion can presented as wide range of different neurological manifestations-like transient ischemic attack, stroke, seizure or syncope. Sometimes syncope can mimic seizure and they are often considered as medically refractory epilepsy.[4] Examination of carotid and peripheral pulses is very informative during clinical examination of such patients. Subclavian steal syndrome is characterised by the vertebrobasilar insufficiency brought about by diversion of blood from these vessels to the arm muscles during their exertion to meet the metabolic demands. This can explain the tingling and numbness of right upper limbs after using it for some time due to vascular claudication.[5] Most common causes include atherosclerosis, Takayasu’s arteritis, cervical ribs and thoracic outlet compression. Mostly the syndrome is unilateral and that too left sided.[6] Subclavian steal syndrome is a rare complication of Takayasu arteritis and the bilateral presentation as in our case is rarer.[7] About 2.5% of secondary hypertension are due to renal artery stenosis.[8] Our patient had hypertension which was due to right renal artery osteal stenosis. Takayasu arteritis is an important cause of hypertension in young. She had involvement of right common iliac artery and femoral artery that result in intermittent claudication in lower limbs. Similar type of lower limbs claudication were reported in literature in around 13% and 20% by involvement of common iliac and femoral artery, respectively.[2] The convulsive syncope was previously also reported in young patients of Takayasu arteritis aged 17–19 years of Asian and African origin.[4,9] In current case the percutaneous intervention was done for right renal artery, right carotid artery and right innominate to vertebral artery. Percutaneous interventions are feasible and standard intervention of choice for peripheral as well as carotid artery stenosis management in Takayasu arteritis as per recent literature.[10] Conclusion Examination of peripheral pulses is extremely important in any patient who present with history of recurrent transient loss of consciousness. It can help in diagnosing serious underlying pathology-like Takayasu arteritis causing syncope which may mimic seizure-like events sometimes labelled as refractory epilepsy. Percutaneous angioplasty of carotid as well as peripheral arteries can be offered in selected cases of significant Takayasu arteritis stenosis. Syncope due to cerebral hypoperfusion can manifest as seizure which remain medically refractory unless the primary cause is treated adequately like Takayasu arteritis in the present case. 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. Ethical approval Ethical approval for this study was not required, as per institute ethical policy for the single case report. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.