- A viral induced vasculopathy with vascular inflammation due to active initial chicken pox infection or reactivation of the virus.
- The vasculopathy can lead to ischaemic infarction of the brain and spinal cord, as well as aneurysm formation, subarachnoid and cerebral haemorrhage, carotid dissection.
- Rarely can even cause peripheral arterial disease. It can occur without a rash and diagnosis is by Anti VZV IgG on CSF.
- Primary infection with VZV in childhood manifests as chickenpox, and then VZV enters a dormant period in the dorsal root ganglia
- Reactivation as cell mediated immunity falls later is seen in later life or when on immunosuppression
- Inflammatory changes of both large and small arteries. There is a granulomatous angiitis of the nervous system in patients with herpes zoster.
- The VZV vasculopathy occurring up to 6 months later suggesting some chronic arterial damage.
- It may affect both immunocompetent and immunocompromised patients and is often multifocal.
- There is no relationship between VZV and Giant cell arteritis
- Headache, fever, malaise and then focal neurological findings and can cause a vasculopathy or myelopathy.
- Retinal necrosis, and cerebellitis have been seen. Post herpetic neuralgia is also seen.
- There may be no or minimal rash (zoster sine herpete).
- There may also be simply zoster infection with ipsilateral infarcts.
- Headache may be seen.
- Monocular visual loss due to involvement of blood supply to retina with resultant CRAO.
- A myelitis due to VZV may occur with long tract signs
- Elevated anti-VZV IgM antibody, indicating active infection
- Brain Imaging: Is almost always abnormal. Ovoid rounded lesions can be superficial or deep. Classically lesions are seen on imaging at the grey-white matter junction.
- Angiography: can affect large and small arteries. May be presence of stenosis or occlusion. Can be segmental constriction, often with post stenotic dilatation. Aneurysm formation may be seen especially in those with HIV.
- CEMRA: Contrast-enhanced vessel wall imaging high-resolution MRI (HRMR) has revealed vessel wall thickening and enhancement in multiple intracranial vasculopathies, including varicella zoster virus (VZV) vasculopathy.
- CSF: Mildly raised WCC (< 10-100) and red cells may also be seen. Oligoclonal bands are commonly with IgG directed against VZV. The diagnostic value of detecting anti-VZV IgG is more effective than detecting VZV DNA. A negative PCR does not exclude the diagnosis. A negative anti-VZV IgG antibody tests in the can reliably exclude the diagnosis of VZV vasculopathy.
- Standard stroke care. ABC. HASU bed.
- IV Aciclovir 10 mg/kg TDS for at least 14 days is the standard therapy which may be combined with Prednisolone 1mg/kg for the first 5 days