Moyamoya syndrome as a manifestation of varicella-associated cerebral vasculopathy-case report and review of literature.

Background: Varicella-associated cerebral vasculopathy (VACV) is a serious complication of Varicella zoster virus (VZV) infection. VACV has protean manifestations, with varying clinical, radiological features and prognosis.
Case Description: Moyamoya syndrome (MMS) with VACV is reported in few cases in the past. All the patients were in paediatric age group, presenting with multiple episodes of transient ischemic attacks (TIAs) and infarct. Our case was a 10-year-old Indian girl with ischemic stroke due to VACV who was treated with intravenous acyclovir. She presented 11 months later with multiple episodes of TIAs. Her angiogram showed bilateral moyamoya vasculature. Acetazolamide challenge study revealed areas of hypoperfusion. Previously reported such cases had been treated medically with steroids and antiplatelets. Most of these patients had resolution of motor symptoms after long follow-up; however, this period was marred by recurrent symptoms. Our patient underwent cerebral revascularisation procedure, following which her TIAs resolved, there was improvement in her limb power and, according to her parents, her performance in school has improved at 2-year follow-up.
Conclusion: MMS can be a manifestation of VACV and should be suspected in paediatric patient of non-east Asian population. These patients require treatment with intravenous acyclovir to inactivate the virus. Those with TIAs should undergo cerebral revascularisation procedures. Medical management should be reserved for patients with adequate collaterals.