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J R Soc Med 2006;99:527-528
doi:10.1258/jrsm.99.10.527
© 2006 Royal Society of Medicine

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J R Soc Med 2006;99:527-528
© 2006 The Royal Society of Medicine

Grand Round

A young stroke: the need for vigilance

Rowan Miller1 Colette Griffin1 Jagit Shah2 Amrish Metha2   Pankaj Sharma1

1 Senior House Officer (Neurology), SpR Neurology, and Consultant Neurologist & Reader in Cerebrovascular Biology, Hammersmith Hospitals Acute Stroke Unit, Department of Clinical Neuroscience, Imperial College, London W6 8RF, UK
2 SpR Neurology and Consultant Neuroradiology, Department of Neuroradiology, Hammersmith Hospitals, London W6 8RF, UK

Correspondence to: Dr P Sharma E-mail: psharma{at}imperial.ac.uk

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.

CASE HISTORY

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.


Figure 1
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Figure 1. Computerized imaging angiogram. Axial images of aortic arch and origin of great vessels demonstrate an artery-centric process with a soft tissue mass surrounding the origins of the brachiocephalic artery (B) and the left common carotid artery (C). The latter is markedly reduced in calibre. Note also the presence of small lymph nodes in the pre-aortic space (E). See the contrast within the superior vena cava (A) and the uninvolved origin of the left subclavian artery (D)

 
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).


Figure 2
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Figure 2. Whole body positron emission tomography isotope study. Increased concentric tracer uptake at the aortic root and ascending aorta is shown on the axial slice images. Maximum SUV 2.4. No abnormal uptake demonstrated in the remainder of the aorta, the common carotid vessels or the common iliac vessels. The features are consistent with a large vessel aortitis

 
COMMENT

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.

Footnotes

Competing interests None declared.

REFERENCES

  1. Bevan H, Sharma K, Bradley W. Stroke in young adults. Stroke 1990; 21: 382-6[Abstract/Free Full Text]

  2. Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol2002; 55:481 -6[Abstract/Free Full Text]

  3. Ringleb PA, Strittmatter EI, Loewer M, et al. Cerebrovascular manifestations of Takayasu arteritis in Europe. Rheumatology (Oxford)2005; 44:1012 -15

  4. Moriwaki R, Noda M, Yajima M, Sharma BK, Numano F. Clinical manifestations of Takayasu arteritis in India and Japan—new classification of angiographic findings. Angiology1997; 48:369 -79[Medline]


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