Primary angiitis of the central nervous system: experience of a Victorian tertiary-referral hospital

Background: 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. Aim: To describe the clinical features, diagnostic modalities, treatment and outcomes in an Australian hospital population-based series of primary cerebral angiitis. Methods: 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. Results: 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. Conclusion: 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.