A practical approach to the diagnosis and management of primary angiitis of the central nervous system

Abstract

Vasculitides present a rare and heterogeneous group of diseases that affect small, middle-sized and/or large arteries. Most of them are systemic diseases, however, they can also present as an isolated (primary) angiitis of the central nervous system. Among the most common presenting symptoms are: headache, cognitive deficits, and encephalopathy, but they frequently present with ischaemic and/or haemorrhagic stroke, and transitory ischaemic attack too. In the cerebrospinal fluid, lymphocytic pleocytosis and/or elevated proteins can be seen, with acute phase proteins and other laboratory findings (rheumatology and microbiological tests, cytology) being commonly negative. The findings on magnetic resonance imaging of the brain are non-specific in 90%. With negative magnetic resonance imaging and cerebrospinal fluid findings, the diagnosis of primary angiitis of the central nervous system is highly implausible. On angiography, in approximately 40% of patients the vascular beading, a non-specific finding of dilated areas alternating with narrowing of the blood vessels can be seen. Biopsy rpesents the golden standard, although it is false negative in 25% of cases due to its segmental distribution. Most studies suggest corticosteroids and cyclophosphamide as an induction therapy, folowed by a maintenance therapy (azathioprine or mycophenolate mofetil) in 6–12 months. Primary angiitis of the central nervous system still presents a diagnostic challenge with early therapeutic intervention being crucial for a better outcome.