Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis involving the central nervous system: case report and review of the literature.

OBJECTIVES: To report a case of biopsy-proven, ANCA-associated vasculitis (AAV) involving the central nervous system (CNS) and to review the relevant literature.
METHODS: Descriptive case report of one patient with AAV-related CNS vasculitis and review of the relevant literature (PubMed search from 1966 to February 2010).
RESULTS: A 61-year-old female patient with AAV developed cognitive impairment. Cerebrospinal fluid analysis was unremarkable, while magnetic resonance (MR) imaging showed multiple left hemisphere infarctions and MR angiography revealed multiple stenoses of the distal branches of the left median cerebral artery. Treatment with glucocorticoids, cyclophosphamide, and intravenous immunoglobulins led to improvement. CNS vasculitis often arises when vasculitis is active elsewhere. There is no clear preponderance of gender or of age of onset. Both ANCA-positive and -negative cases of CNS vasculitis are documented. The diagnosis is usually based on clinical CNS manifestations and multiple ischaemic (sometimes haemorrhagic) MR lesions mainly affecting the white matter. Angiography is often negative. Treatment with glucocorticoids and cyclophosphamide, sometimes with adjunctive intravenous immunoglobulins, usually improves clinical features and MR lesions.
CONCLUSIONS: AAV rarely involves the CNS. CNS vasculitis should be suspected if patients have neurological manifestations consistent with CNS involvement, particularly if they have evidence of disease activity elsewhere, and if MR shows multiple ischaemic (sometimes haemorrhagic) lesions mainly affecting the white matter. Sepsis, coagulation disorders, and severe hypertension must be ruled out. Awareness of this rare but severe complication can allow early recognition and prompt treatment.