Isolated Angiitis of the Cnetral Nervous System

ABSTRACT

Isolated angiitis of the central nervous system is a rare clinicopathologic entity characterized by vasculitis restricted to the vessels of central nervous system without other apparent systemic vasculitis. It manifests headache, higher cortical dysfunction. focal neurologic dysfunction and cranial nerve palsies. We experienced a case of isolated angiitis of the central nervous system in 6 year-old girl who was admitted to out unit because of headache, hemiparesis and altered consciousness. The laboratory test for blood, urine, and cerebrospinal fluid are all within normal ranges. There was no evidence of systemic vasculitis. The contrast enhanced brain CT scan showed low densities along the left cerebellar hemisphere and posterior aspect of temporal lobe without enhancement, and left lateral internal carotid angiogram showed poorly contrast filling along the territory of left posterior cerebral artery due to narrowing or ischemic changes of the vessels. Axial T2WI (TR/TE, 2190/80) of magnetic resonance imaging showed multiple scattered high signal intensities at left pons with cerebellar hemisphere and diffuse high signal intensity along the left occipital lobe with enlarged surrounding gyral patterns, and axial T1WI(TR/TE, 665/25) showed intense gyral pattern contrast enhancement along the left occipital lobe. Steroid was tried with apparent benefit. We report a case of isolated angiitis of central nervous system with review of literature.