Case Report

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American Journal of Emergency Medicine

journal homepage: www. elsevier.com/ locate/ajem

American Journal of Emergency Medicine 32 (2014) 685.e3-685.e5

An unusual cause of stroke in a young female

Abstract

Takayasu arteritis is a granulomatous vasculitis of medium- to large-sized arteries. Initial presentation is usually with nonspecific constitutional symptoms, but unusually, it can present with acute ischemic features in the advanced disease. We present a case of a young Asian female who presented with acute stroke due to Takayasu arteritis. Involvement of proximal arteries was a source of embolism, which was the most likely cause of stroke in this patient. Patient made a good clinical recovery with a combination of medical treatment and rehabilitation. Takayasu arteritis should be considered as a possibility in all young patients presenting with stroke, as this is fairly responsive to medical management and associated with good clinical outcomes.

Ischemic arterial stroke in children and young adults occurs less commonly than in elderly, but its consequences can be quite devastating. In particular, issues of evaluation for causes and risk factors, short-term stroke treatments, rehabilitation, and long-term prevention of future strokes all play a very prominent role in stroke care for children and young adults. In addition, because stroke is a rare disorder in children and young adults with different Underlying etiology, optimal stroke care in Young people requires expertise from a variety of medical specialties, including hematology, neurology, radiology, rehabilitation, physiotherapy, and occupational therapy. Although some guidelines have been proposed for the evaluation and

treatment of stroke in children and young adults, the particular circumstances of each patient must also be considered.

We present case of a 28-year-old Asian woman who presented with acute left-sided weakness. On examination, her right-sided radial, brachial, and carotid pulses were not palpable. Blood pressure in right upper limb was not recordable, whereas it was 102/64 mm Hg in her left upper arm. Blood pressure in her both lower limbs was 180/ 90 mm Hg. She had a left upper motor neuron facial palsy with a left dense hemiplegia (power grade 0/5) on her left side.

A bruit was audible over the left carotid and subclavian arteries. Her electrocardiogram showed sinus rhythm, and echocardiogram was unremarkable. She was mildly anemic with hemoglobin level of 10.8 mg/ dL, a raised ESR of 110 mm/h, and her autoimmune profile was negative. Computed tomography head confirmed a large infarct in right frontal region. Carotid Doppler showed bilateral occlusive Common carotid artery thrombi and marked hypertrophy of left vertebral artery with contralateral Internal carotid artery deriving flow from it through circle of Willis. computed tomographic angiography (Fig. 1A and B) showed a total osteal occlusion with right and left common Carotid arteries appearing just before bifurcation. Right internal carotid artery was of small caliber and thick walled, whereas left internal carotid artery had total proximal occlusion. Left vertebral artery was markedly dilated, whereas right vertebral artery showed subtotal proximal occlusion and small caliber in the rest of its course. Circle of Willis was collateralized from vertebral and basilar arteries.

Fig. 1. A and B, Computed tomographic angiography showed a total osteal occlusion with right and left common carotid arteries appearing just before bifurcation. C, Computed tomography head confirmed a large infarct in the right frontal region. Carotid Doppler showed bilateral occlusive common carotid artery thrombi and marked hypertrophy of left vertebral artery with contralateral internal carotid artery deriving flow from it through circle of Willis.

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this phase is characterized by nonspecific inflammatory features in the affected arteries. This later on progresses into obstructing lesions within the lumen giving rise to the symptoms of organ ischemia in the territory of the affected arteries. The common clinical features during this phase include absence of peripheral pulses, limb claudication, visual symptoms secondary to ischemic retinopathy, renovascular hypertension, oliguric renal failure, coronary artery disease, stroke, and Raynaud’s phenomenon [2].

Takayasu arteritis is a very rare cause of stroke. Approximately 10% to 20% of patients with TA have been reported to have Neurologic manifestations [4,5]. These include dizziness, headaches, and strokes [2,6]. Acute stroke as the first presentation of TA has rarely been reported [7].

Transient ischemic attack or strokes may occur in TA because of intracranial stenosis secondary to vasculitis or the emboli originating from inflammatory regions of artery [5]. Imaging studies can provide the information about involvement of heart valves or proximal arteries as a source of embolism for acute stroke, as this was the most likely cause of stroke in our patient. Anticoagulation is necessary to prevent further episodes of strokes, which could be devastating. There is no diagnostic laboratory test for TA. Diagnosis mainly depends upon clinical features and imagining studies. Pulse difference in the extremities and hypertension due to involvement of renal artery or aorta are found in 96% and 772%, respectively. Ultrasound Doppler, computed tomogra- phy, and recently magnetic resonance angiography have improved the diagnosis of TA [7]. Steroids are the mainstay of treatment for TA. Steroid unresponsive patients can be treated with cytotoxic drugs including cyclophosphamide, azathioprine, and methotrexate [2]. Treatment goals include aims to control Disease progression with minimal long-term drug side effects. Indications for surgical interventions include severe stenosis of renal artery, limb claudication, symptomatic carotid artery disease, or evidence of coronary artery involvement.

Prognosis is fairly good, with a 10-year survival rate of around 90%. Upon immunosuppression, the disease takes a stable course in most of the patients [8]. Severe hypertension and cardiac failure are bad prognostic factors.

Takayasu arteritis should be considered as a possibility in all young patients presenting with stroke, as this is fairly responsive to medical management and associated with good clinical outcomes.

Takayasu arteritis should be considered as a possibility in all young patients (especially females) presenting with stroke, as this is fairly responsive to medical management and associated with good clinical outcomes.

Emphasize the need for thorough evaluation, including exam- ination of pulses and blood pressure in all 4 limbs, of young patients with stroke or secondary hypertension to establish the underlying etiology. Absence of peripheral pulses in upper limbs, carotid/renal bruits, and presence of hypertension can point toward the diagnosis.

Imaging of carotid and intracranial vessels in patients with TA

Fig. 2. A and B, Magnetic resonance angiography.

Magnetic resonance angiography (Fig. 2A and B) showed dilated vertebral arteries, multiple collaterals in the neck, and narrowed and concentrically thickened carotid vessels with unusual origin of main vessels arising from aortic arch.

The diagnosis of Takayasu arteritis (TA) was made according to the American College of Rheumatology criteria [3], and the patient was started on steroids. She made good clinical and functional recovery on standard treatment and neurorehabilitation. Currently, she is independent with her advanced daily living and taking 5 mg of prednisolone and warfarin.

Takayasu arteritis is panarteritis affecting medium- and large- sized arteries. It is more prevalent in Asian population [1]. Initial presentation is usually with nonspecific constitutional symptoms, such as fever, night sweats, and malaise and arthralgia. Histologically,

may be helpful for primary or secondary Prevention strategies.

The rarity of the disease and such a presentation can cause delay in the diagnosis and treatment.

Early diagnosis, evaluation, rehabilitation, and strategies for long-term prevention of future strokes all play a key role in stroke care for young adults.

Malik Asif Humayun, FCPS, MRCP

Royal Hampshire county hospital

Winchester, UK

Adnan Masood, FCPS Tariq Waseem, FCPS

Jinnah Hospital LahoreLahore

Pakistan

M.A. Humayun et al. / American Journal of Emergency Medicine 32 (2014) 685.e3-685.e5 685.e5

Farina Ahmad, MBBS DHQ Hospital Faisalabad Faisalabad, Punjab, Pakistan

Tanzeem Raza, MSc, FRCP

Royal Bournemouth Hospital

Bournemouth, UK

http://dx.doi.org/10.1016/j.ajem.2013.11.039

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