PROFOUND CEREBROSPINAL-FLUID PLEOCYTOSIS AND FROINS-SYNDROME SECONDARY TO WIDESPREAD NECROTIZING VASCULITIS IN AN HIV-POSITIVE PATIENT WITHVARICELLA-ZOSTER VIRUS ENCEPHALOMYELITIS

Demonstration of the direct involvement of cranial blood Vessels by va ricella tester virus (VZV) is facilitated by immunohistochemistry (IHC ), in situ hybridization (ISH) and polymerase chain reaction (PCR) tec hniques. The extent to which an inflammatory vasculitis serves as the pathogenic mechanism for VZV encephalomyelitis (VZVE) is still, howeve r, debated. Most VZVE patients are immuno-compromised and show little inflammation, either pre-mortem in cerebrospinal fluid (CSF) or at aut opsy. We describe an HIV-positive patient with a moderately depressed CD4 count (304) who presented with massively elevated CSF protein (180 0 mg/dl), bloody CSF and pleocytosis (1300 white blood cells (WBC)/mm( 3)). His CSF was positive for VZV DNA by PCR. He was treated with acyc lovir and foscarnet, but died. At autopsy, an unusually widespread, in flammatory, transmural vasculitis caused by VZV affected meningeal ves sels at virtually all brain stem and spinal cord levels, causing multi ple subpial hemorrhages and necrosis. Virus DNA in multiple areas of b rain, brainstem and spinal cord was readily revealed by PCR, but not b y the presence of viral inclusions, IHC or ISH. This case, with a clin ically confusing presentation for VZVE, illustrates the extensive, alb eit infrequent, degree of necrotizing vasculitis and CSF abnormalities that VZV is capable of producing. Antiviral therapy may have inhibite d VZV genome replication and subsequent antigen production, resulting in negative ISH and IHC studies, but generated increased VZV genomic f ragments that were detectable by the more sensitive PCR technique. (C) 1998 Elsevier Science B.V. All rights reserved.