🦠 A viral-induced vasculopathy with vascular inflammation due to either initial chickenpox infection or later reactivation of VZV.
🧠 Can lead to ischaemic stroke, spinal cord infarction, aneurysm formation, subarachnoid or intracerebral haemorrhage, and carotid dissection.
🦵 Rarely, it causes peripheral arterial disease. It may occur without rash, with diagnosis confirmed by detecting Anti-VZV IgG in CSF.

⚡ Key teaching point: VZV vasculopathy may present without skin lesions ("zoster sine herpete") - don’t exclude it just because there’s no rash.

🧬 Aetiology

👶 Primary VZV infection = chickenpox. Virus then lies dormant in dorsal root ganglia.
🔄 Reactivation later in life/immunosuppression → shingles (herpes zoster).
🧬 Inflammation affects large & small arteries; granulomatous angiitis can occur.
📆 Vasculopathy can appear up to 6 months after infection → evidence of chronic arterial injury.
👩‍⚕️ Occurs in both immunocompetent and immunocompromised patients, often multifocal.
🚫 No proven association between VZV and giant cell arteritis.

🩺 Clinical Features

🤕 Headache, fever, malaise → followed by focal neurological deficits (stroke/myelopathy).
👁️ Monocular visual loss (central retinal artery occlusion).
🧠 Complications: retinal necrosis, cerebellitis, post-herpetic neuralgia.
❌ Rash may be absent (zoster sine herpete).
🧲 Ipsilateral infarcts can accompany zoster infection.
🦵 VZV myelitis may cause long tract signs (UMN features).

📝 Exam Pearl: Think of VZV vasculopathy in a patient with stroke + recent shingles (especially ophthalmic distribution).

🔎 Investigations

🧪 Anti-VZV IgM: Active infection marker.
🖥️ Brain Imaging: Usually abnormal. Ovoid lesions at grey-white junction typical.
🩻 Angiography: Stenosis, occlusion, “string-of-beads” narrowing, post-stenotic dilatation. Aneurysms in HIV patients.
🧲 CEMRA: Vessel wall thickening & contrast enhancement.
💉 CSF:
- Mild pleocytosis (10–100 WCC/µL).
- Oligoclonal bands with anti-VZV IgG.
- Anti-VZV IgG more reliable than VZV DNA PCR.
- ❌ Negative anti-VZV IgG = rules out VZV vasculopathy.

💊 Management

🏥 Standard stroke care (ABCs, admit to HASU).
💉 IV Aciclovir 10 mg/kg TDS × 14 days (minimum).
💊 Add Prednisolone 1 mg/kg × 5 days to reduce inflammation.

🌟 Clinical takeaway: Early antiviral therapy is crucial. Delayed diagnosis risks multifocal infarcts and poor outcome.

References

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Varicella Cerebral Vasculopathy

Introduction

🧬 Aetiology

🩺 Clinical Features

🔎 Investigations

💊 Management

References

Categories