MOYAMOYA disease in an adolescent with HIV-1

Moyamoya disease has been reported in both children and adults with HIV-1. Most cases reported in children were found to have unsuppressed viral loads and low CD4 counts. Although the aetiology of the disease is largely unknown, a few studies have postulated cytokine imbalance and immune activation as possible causes. Intimal staining of the involved cerebral arteries have revealed transmembrane glycoprotein of HIV-gp 41. We present the case of an 18-year-old boy with congenitally acquired HIV-1 who presented with right hemiparesis at the age of 12 years and was found to have Moyamoya disease on neuroimaging. His CD4 count has always been low (<100 cells/cumm) in spite of being virally suppressed. He was started on anti-retroviral therapy at 5 and half years of age and he was continued on the same. He was treated conservatively and he continues to have residual right hemiparesis.