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Stroke is a very common clinical presentation and young patients (<45 years old) may present without any apparent risk factors. It is therefore important to exclude all aetiological causes in order to reduce their chances for a subsequent event.
A 29-year-old right-handed Australian Caucasian woman was admitted following a sudden onset of an expressive dysphasia but no other neurological symptoms. She had no relevant past or family medical history and her only regular medication was the oral contraceptive pill. She consumed 14 units of alcohol per week, smoked 10 cigarettes per day and admitted to occasionally smoking cannabis but denied any other recreational drug abuse. On examination she was afebrile, in sinus rhythm and had a blood pressure of 135/60 mm Hg in the right arm and 120/65 mm Hg in the left arm. Cardiac and respiratory systems were unremarkable with no evidence of cardiac murmurs, all peripheral pulses were present. Examination confirmed an expressive dysphasia and no other neurological signs. The patient was commenced on 300 mg aspirin and admitted for further investigations. Her dysphasia rapidly resolved. However, the following morning she awoke with a dense right hemiplegia and complete aphasia.
The following investigations were normal or nondiagnostic: full blood count, renal, liver and thyroid function, syphilis serology, coagulation screen, vitamin B12, red cell folic acid, homocysteine, serum glucose, proteins C and S, activated protein C resistance, antibody to thrombin III, autoantibody screen, lupus anticoagulant and factor V Leiden. Her ESR was 66 mm/h, C-reactive protein 31 mg/L and total cholesterol was 6.5 mM/L. Electrocardiogram, Doppler scan and bubble echocardiogram was normal with no evidence of a patent foramen ovale. An initial computerized tomography brain scan showed a left middle cerebral artery infarct. A magnetic resonance imaging brain scan confirmed a large left middle cerebral artery infarct territory infarct with a large clot in the left carotid artery. A computerized tomography angiogram demonstrated a large rind of abnormal soft tissue which surrounded the aortic arch extending cranially along both the left common carotid and to a lesser extent the brachiocephalic artery (Figure 1). The degree of soft tissue around the left common carotid artery was such that the lumen of the vessel was severely narrowed.
A diagnosis of Takayasu's arteritis was made. This was confirmed by a positron emission tomography scan performed at a later date when the patient was on steroids which demonstrated increased uptake in the aortic root and ascending aorta in keeping with large vessel aortitis (Figure 2).
Although a rare cause of cerebrovascular disease, such is the prevalence of stroke that it is important to consider the diagnosis in young patients (<45 years), who make up approximately 8.5% of all stroke victims.1 In this patient the discrepancy in blood pressures in the upper limbs and the raised electron spin resonance pointed towards an inflammatory arteritis that was subsequently demonstrated using positron emission tomography imaging.
Takayasu's arteritis is a rare form of chronic inflammatory arteritis affecting large vessels, predominately the aorta and its main branches. Inflammatory infiltrates cause thickening of the affected arteries with narrowing and thrombosis. Dilation and aneurysm formation may also occur. Takayasu's is rare, with an annual incidence of 2.6 per million in North America. The disease has a higher incidence in females and usually presents in the second or third decade. Although it is rarely seen in Caucasians,2 the fact that our patient originated from Australia may be of clinical importance. Presentation varies from asymptomatic pulselessness to catastrophic neurological events. Clinical manifestations due to end organ ischaemia are dependent on the vessels involved with neurological involvement in 10%-20%3 of cases and may be ethnically dependent.4 Takayasu's is treated using steroids and immunosuppression and has a variable course.
Our case clearly demonstrates the need to be ever vigilant in the aetiology of the common stroke syndrome.
Competing interests None declared.