Learning objectives
|
Introduction
- 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.
Aetiology
- 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 affects both immunocompetent and immunocompromised patients and is often multifocal.
- There is no relationship between VZV and Giant cell arteritis
Clinical
- 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
Investigations
- 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 postste-nic 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.
Management
- 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
References
- Varicella zoster virus vasculopathies: diverse clinical manifestations, laboratory features, pathogenesis, and treatment. Lancet Neurol. 2009 August ; 8(8): 731. doi:10.1016/S1474-4422(09)70134-6.
- Herpes zoster and the risk of ischemic and hemorrhagic stroke: A systematic review and meta-analysis
| Note: The plan is to keep the website free through donations and advertisers that do not present any conflicts of interest. I am keen to advertise courses and conferences. If you have found the site useful or have any constructive comments please write to me at drokane (at) gmail.com. I keep a list of patrons to whom I am indebted who have contributed. If you would like to advertise a course or conference then please contact me directly for costs and to discuss a sponsored link from this site. |