Primary Central Nervous System Vasculitis

Excerpt

Primary angiitis of the central nervous system (PACNS), also known as central nervous system (CNS) vasculitis, is a rare inflammatory disorder of the blood vessels of the brain and spinal cord, without any evidence of systemic vasculitis or an inflammatory or connective tissue disease. PACNS was first described as a distinct clinical entity in 1959, and since then, multiple different clinical presentations have been reported. Manifestations of PACNS at the time of diagnosis are nonspecific, with a wide range of presenting symptoms. The most common presenting manifestations are focal neurologic complaints. These may include weakness, aphasia, and visual field defects. Severe, persistent headaches are also common, as are cognitive decline and encephalopathy.