Unusual presentation of central nervous system manifestations of Varicella zoster virus vasculopathy in renal transplant recipients

Abstract

We describe 2 renal transplant recipients with severe but reversible neurological manifestations related to Varicella zoster virus (VZV) cerebral vasculopathy. To the best of our knowledge, this is the first description of cerebral VZV vasculopathy in solid organ transplant recipients. We review the published literature on the clinical presentation, diagnosis and treatment. In solid organ transplant recipients presenting with neurological signs and symptoms, a diagnosis of VZV-associated vasculopathy should be considered.

Varicella zoster virus encephalitis in solid organ transplant recipients: Case series and review of literature

“…Large vessel vasculopathy is associated with elderly immunocompetent patients and presents with acute neurologic deficits . Cases have been reported in SOT recipients . It follows an episode of trigeminal HZ and affects large arteries of anterior or posterior circulation .…”

“…Antivirals including vidarabine, which was discontinued in United States in 2001, foscarnet, cidofovir, and brincidofovir have been used for acyclovir‐resistant strains . Although one case reported steroid use to decrease the effects of vasculitis, use of corticosteroid remains controversial…”

“…3 Cases have been reported in SOT recipients. 19 It follows an episode of trigeminal HZ and affects large arteries of anterior or posterior circulation. 3 Diffuse inflammation of small cerebral vessels is seen in small vessel vasculopathy and results in more generalized symptoms.…”

“…Diagnosis is made with lumbar puncture and CSF analysis, which typically shows lymphocytic pleocytosis and viral culture, antibody, or PCR positive for VZV. 3,4,19,22,24 CSF PCR assays have led to earlier diagnosis; VZV PCR has sensitivity of 80%-95% in immunocompromised patients and specificity of >95%. 25 In VZV vasculopathy, there may be no CSF pleocytosis and detection of anti-VZV antibody in CSF may have higher sensitivity than VZV PCR.…”

Central Nervous System Infections

“…Immunocompromised hosts, particularly those with impaired cell-mediated immunity, are at the greatest risk of disease (72). CNS VZV infection most commonly manifests as encephalitis, but it can rarely present as varicella cerebellitis, meningitis, myelitis, or vasculopathy (73,74). CSF examination reveals a mononuclear pleocytosis, and diagnosis is confirmed by detection of VZV via PCR or identification of intrathecal antibodies (75).…”