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.
Subject(s)
Child , Female , Humans , Brain , Central Nervous System , Cerebrospinal Fluid , Consciousness , Cranial Nerve Diseases , Headache , Magnetic Resonance Imaging , Nervous System , Neurologic Manifestations , Occipital Lobe , Paresis , Pons , Posterior Cerebral Artery , Reference Values , Systemic Vasculitis , Temporal Lobe , Tomography, X-Ray Computed , VasculitisABSTRACT
Clinical observation was carried out for 147 patients with mucocutaneous lymph node syndrome (MCLS) who were admitted to the Department of Pediatrics, Wonju Christian Hospital during the period from June, 1983 to June, 1992. A spinal tap was performed on 114 of these patients and the following results were obtained. 1) The incidence of aseptic meningitis in patients with MCLS was 52.6%. (60 cases of the 114 cases who had spinal taps (52.6%).) 2) The predominent age group for aseptic meningitis was 6 months to 1 year of age (35%). 3) For the majority, the value of glucose and protein in the CSF were within normal limit or only mildly elevated. 4) The neurologic manifestations associated with aseptic meningitis in patients with MCLS were irritability (78.3%), vomiting (25.0%), nuchal rigidity (11.7%), convulsion (5.0%) and facial nerve palsy (1.7%) in that order.