Varicella-zoster virus vasculopathy. A review description of a new case with multifocal brain hemorrhage.

BACKGROUND

The varicella zoster virus (VZV) is a highly neurotropic virus that, after the primary infection, remains latent in the nerve cells and can reactivate many years later, resulting in various conditions affecting the central nervous system, such as vasculopathy and stroke.

METHODS

We report on a review of the published literature that included all case reports identified via PubMed and an additional unpublished case of VZV vasculopathy. All epidemiological, clinical, laboratory, imaging, virologic, treatment and outcome data collected are described.

RESULTS

Of the 62 patients, 41.6% were immunocompromised. Ischemic stroke occurred in 77.2% of the patients, comprising cases of isolated (37.1%) and multifocal stroke (17.7%). Multifocal, ischemic and hemorrhagic stroke was only described in the newly reported case. The magnetic resonance imaging results were normal in 2.9% of the cases. The vascular studies (angiography and magnetic resonance angiography [MRA]) revealed signs of angiitis in 74.4% of the cases; the small arteries were involved in 38.5% of the cases, large arteries in 17.7% and mixed in 43.5%. For 95.2% of the patients, the cerebrospinal fluid (CSF) was positive for VZV IgG antibodies, and for 46.1% of the patients, the CSF was positive for polymerase chain reaction (PCR); however, the diagnosis was confirmed in only 3 of 6 biopsies.

DISCUSSION

VZV vasculopathy can occur in both immunocompetent and immunosuppressed patients. Neuroimaging can reveal stroke and angiitis, and the detection of VZV-specific IgG antibodies in the CSF is a reliable and highly sensitive diagnostic tool. The multifocal nature of VZV vasculopathy makes biopsy a test with low sensitivity and high morbidity.