014. A RARE CAUSE OF STROKE: PRIMARY ANGIITIS OF THE CENTRAL NERVOUS SYSTEM

Abstract

Background: Primary angiitis of the central nervous system is a rare type of cerebral vasculitis which only affects the central nervous system. It has an incidence of 1-2 per million population compared with 40 per million population for systemic vasculitis. Methods: We present a 41 year old Caucasian female admitted with right-sided weakness which was associated with bilateral paraesthesia and blurred vision. There was no significant medical or medication history, or illicit drug abuse. On examination she had severe rightsided weakness and sensory deficit. Blood tests including inflammatory markers, ECG, chest X-ray and CT of the brain were all unremarkable. A subsequent diffusion-weight MRI of the brain demonstrated multiple acute infarcts affecting the frontal and parietal lobes, especially on the left side. A simultaneous MR-angiogram noted multiple strictures in the right internal carotid artery and circle of Willis with appearances suggestive of vasculitis. This was confirmed on CTangiogram. Virology screen, Lyme disease serology, TB-antigen and autoimmune screen including ANCA and ANA were all negative. A transthoracic echocardiogram was also normal. A lumbar puncture was unremarkable except for marked protein levels (0.81 g/l). Within 24-hours of admission the patient developed confusion, worsening right-sided weakness and expressive dysphasia despite high-dose aspirin. She was therefore commenced on high-dose prednisolone 60mg but later switched to intravenous methylprednisolone 1 g, as her neurological signs continued to deteriorate. Despite steroid treatment her neurology continued to fluctuate from complete resolution to paralysis. She was therefore commenced on intravenous cyclophosphamide 750 mg. Gradually, with immunosuppressant treatment as well as multidisciplinary rehabilitation her weakness improved and her confusion and dysphasia resolved. She underwent six cycles of cyclophosphamide treatment and will commence azathioprine as maintenance. Results: The patient was discussed between the neurology, rheumatology, neuroradiology and neurosurgical teams. All were in agreement that her symptoms were consistent with cerebral vasculitis. Despite extensive investigations, there was no evidence of systemic vasculitis. She was therefore diagnosed with primary angiitis of the central nervous system. Although a biopsy would confirm the diagnosis, this was not pursued given her presentation, imaging findings and excellent response to immunosuppressive treatment. Conclusion: Rheumatological disease such as vasculitis should be considered in any patient presenting with stroke at a young age. Vasculitis is typically a multi-systemic disease which can be associated with raised serum inflammatory markers and positive serum ANCA. However, it rarely can exclusively affect the brain and spinal cord and can only be diagnosed through lumbar puncture, imaging techniques and biopsy. Finally, inflammatory conditions such as vasculitis, SLE and rheumatoid arthritis are associated with increased risk of ischaemic heart disease and stroke. Appropriate management of these rheumatological conditions is therefore essential in preventing cardiovascular disease.