Primary angiitis of the central nervous system: experience of a Victorian tertiary‐referral hospital. Issue 6 (9th June 2013)

• Record Type: Journal Article
• Authors: Oon, S.; Roberts, C.; Gorelik, A.; Wicks, I.; Brand, C.
• Is Part Of: Internal medicine journal. Volume 43:Issue 6(2013)
• Page Start: 685
• Page End: 692
• Publication Date: 2013-06-09
• Subjects: Medicine -- Periodicals; 616
• DOI: 10.1111/imj.12038
• Languages: English
• ISSNs: 1444-0903

Abstract

<abstract abstract-type="main"> <title>Abstract</title> <sec id="imj12038-sec-0001" sec-type="section"> <title>Background</title> <p>Primary angiitis of the central nervous system is a rare condition, which is often difficult to diagnose and is associated with significant morbidity and mortality. There is no standardised treatment protocol or randomised clinical trial evidence to guide management.</p> </sec> <sec id="imj12038-sec-5001" sec-type="section"> <title>Aim</title> <p>To describe the clinical features, diagnostic modalities, treatment and outcomes in an Australian hospital population‐based series of primary cerebral angiitis.</p> </sec> <sec id="imj12038-sec-0002" sec-type="section"> <title>Methods</title> <p>Data were collected via retrospective medical record review of patients with primary angiitis of the central nervous system for the period 1 July 1998 to 30 June 2009, using previously published diagnostic criteria. Eligible patient records were identified in two ways; from routinely collected hospital episode data, coded using the ICD‐10‐AM coding standard and by review of cerebral biopsy data.</p> </sec> <sec id="imj12038-sec-0003" sec-type="section"> <title>Results</title> <p>Ten of 12 included patients had a positive cerebral biopsy, with two patients diagnosed by angiography. Mean age at diagnosis was 47.2 years (range 18–73 years), with a female predominance of 5:1. Headache was the most common symptom experienced. Seventy per cent of the biopsy specimens showed a lymphocytic vasculitis. All patients received treatment with either steroids alone or a combination of steroids and cyclophosphamide, the latter treatment being initiated for those with a higher modified Rankin score of disability. Nine (75%) responded to treatment. There was one in‐hospital death, and two patients had no documented response to treatment.</p> </sec> <sec id="imj12038-sec-0004" sec-type="section"> <title>Conclusion</title> <p>This study presents the first Australian case series data of primary cerebral angiitis. Better understanding of management and outcomes of this rare condition would be gained through multicentre studies.</p> </sec> </abstract>