EARLY AND LATE-ONSET MANIFESTATIONS OF CEREBRAL VASCULITIS RELATED TOVARICELLA-ZOSTER

Varicella-zoster associated cerebral vasculitis (VZCV) as a cause of c erebral infarction has hitherto been considered a rare condition. Isch emic stroke in previously healthy children has occurred during recover y from chickenpox or has been attributed to virus reactivation among i mmunosuppressed patients. The clinical, radiologic and immunologic fin dings in four children with VZCV will be reported. Clinical manifestat ions included sudden onset of hemiparesis, motor aphasia and disturbed consciousness in previously healthy children. Only one child had a hi story of chickenpox six weeks prior to the onset of stroke, whereas a latency period of up to four years between chickenpox and the onset of stroke was found in the other three children. Diagnosis of VZCV was c onfirmed repeatedly by demonstrating intrathecal production of varicel la-zoster IgC antibodies in three children or a four-fold increase of varicella-zoster serum IgA-antibodies in one child. Intrathecal produc tion of antibodies against other latent viruses and borreliosis could be excluded. PCR for varicella on CSF, performed in two patients, rema ined negative. No intrathecal production of varicella-zoster antibodie s has been found in a control group of twenty clinically healthy child ren (age range from 2-18 years) with a previous varicella infection. D uring follow-up two children recovered completely whereas two children still suffer from serious neurological deficits. Immunological invest igations, performed in three children, showed circulating immune-compl exes with slightly lowered complement concentrations in two patients. In addition a lowered T-helper/T-suppressor cell ratio of unknown orig in was found in three children. These immunological findings will be d iscussed in the light of the pathophysiology of VZCV.