Cerebral vasculitis

The noninfectious, inflammatory vasculitides include giant cell arteritis, Takayasu disease, Churg-Strauss angiitis, Wegener disease, polyarteritis nodosa, microscopic polyangiitis, Buerger disease, amyloid-β-related angiitis, and isolated vasculitis of the central nervous system. While these disorders are relatively uncommon, they produce a variety of neurologic diseases including muscle disease, mononeuropathy multiplex, polyneuropathy, cranial nerve palsies, visual loss, seizures, an encephalopathy, venous thrombosis, ischemic stroke, and intracranial hemorrhage. The multisystem vasculitides often have stereotypical clinical findings that reflect disease of the kidney, sinuses, lungs, skin, joints, or cardiovascular system. These disorders also usually have abnormalities found on serologic testing. Isolated vasculitis of the central nervous system is more difficult to diagnose because the clinical and brain imaging findings are relatively nonspecific. Examination of the cerebrospinal fluid will demonstrate changes consistent with an inflammatory process. Arteriography often shows areas of segmental narrowing affecting multiple intracranial vessels and brain/meningeal biopsy may be required to establish the diagnosis. Management of patients with a multisystem vasculitis or isolated vasculitis of the central nervous system is centered on the administration of immunosuppressive agents. In many cases, corticosteroids remain the mainstay of medical treatment.