Abstract
The central nervous system (CNS) may be involved by a variety of inflammatory diseases of blood vessels. These include primary angiitis of the central nervous system (PACNS), a rare disorder specifically targeting the CNS vasculature, and the systemic vasculitides which may affect the CNS among other organs and systems. Both situations are severe and convey a guarded prognosis. PACNS usually presents with headache and cognitive impairment. Focal symptoms are infrequent at disease onset but are common in more advanced stages. The diagnosis of PACNS is difficult because, although magnetic resonance imaging is almost invariably abnormal, findings are non specific. Angiography has limited sensitivity and specificity. Brain and leptomeningeal biopsy may provide a definitive diagnosis when disclosing blood vessel inflammation and are also useful to exclude other conditions presenting with similar findings. However, since lesions are segmental, a normal biopsy does not completely exclude PACNS. Secondary CNS involvement by systemic vasculitis occurs in less than one fifth of patients but may be devastating. A prompt recognition and aggressive treatment is crucial to avoid permanent damage and dysfunction. Glucocorticoids and cyclophosphamide are recommended for patients with PACNS and for patients with secondary CNS involvement by small-medium-sized systemic vasculitis. CNS involvement in large-vessel vasculitis is usually managed with high-dose glucocorticoids (giant-cell arteritis) or glucocorticoids and immunosuppressive agents (Takayasu’s disease). However, in large vessel vasculitis, where CNS symptoms are usually due to involvement of extracranial arteries (Takayasu’s disease) or proximal portions of intracranial arteries (giant-cell arteritis), revascularization procedures may also have an important role.
Highlights
The central nervous system (CNS) vasculature may be targeted by an heterogeneous group of inflammatory diseases
Glucocorticoids and cyclophosphamide are recommended for patients with primary angiitis of the central nervous system (PACNS) and for patients with secondary CNS involvement by small-medium-sized systemic vasculitis
CNS involvement in large-vessel vasculitis is usually managed with high-dose glucocorticoids or glucocorticoids and immunosuppressive agents (Takayasu’s disease)
Summary
The central nervous system (CNS) vasculature may be targeted by an heterogeneous group of inflammatory diseases. Primary form, angiitis of the CNS (PACNS) is a rare form of vasculitis of unknown etiology primarily affecting small and medium sized vessels supplying the brain parenchyma, spinal cord and leptomeninges [13]. PACNS results in signs and symptoms of CNS dysfunction with no clinically apparent participation of other organs. The CNS may be targeted, among other territories, by systemic vasculitides [4, 5]. This review will focus on diagnostic and therapeutic aspects of PACNS and secondary CNS involvement by systemic vasculitides in adulthood. Primary and secondary CNS vasculitis in childhood have been addressed in excellent recent reviews [6,7,8]
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