title: Moyamoya Syndrome in South African Children With HIV-1 Infection.
abstract: A national multicenter study identified 17 South African children with vertically acquired HIV-1 infection and HIV-associated vasculopathy. Five of the children (all indigenous African ancestry) had progressive vascular disease, consistent with moyamoya syndrome. Median presentation age 5.8 years (range 2.2-11). The children with moyamoya syndrome presented with abnormal CD4 counts and raised viral loads. Clinical features included motor deficits, neuroregression, and intellectual disability. Neuroimaging supported progressive vascular disease with preceding clinically silent disease course. Neurologic recovery occurred in 1 patient with improved CD4 counts. Four of the 5 children presented during the era when access to antiretroviral therapy was limited, suggesting that with improved management of HIV-1, progressive vasculopathy is less prevalent. However the insidious disease course illustrated indicates that the syndrome can progress "silently," and manifest with misleading phenotypes such as cognitive delay or regression. Sub-Saharan Africa has limited access to neuroimaging and affected children may be underdiagnosed.
authors.affiliation: Department of Paediatric Neurology, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
authors.forename: Charles K
authors.initials: CK
authors.lastname: Hammond
authors.affiliation: Department of Paediatric Neurology, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
authors.forename: Alexander
authors.initials: A
authors.lastname: Shapson-Coe
authors.affiliation: Department of Paediatric Neurology, University of Kwa-Zulu Natal, Inkosi Albert Luthuli Central Hospital, Durban, South Africa.
authors.forename: Rajeshree
authors.initials: R
authors.lastname: Govender
authors.affiliation: Department of Paediatric Neurology, Department of Paediatrics and Child health, Stellenbosch University, Cape Town, South Africa.
authors.forename: Ronald
authors.initials: R
authors.lastname: van Toorn
authors.affiliation: Department of Paediatric Neurology, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
authors.forename: Alvin
authors.initials: A
authors.lastname: Ndondo
authors.affiliation: Department Paediatric Radiology, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
authors.forename: Nicky
authors.initials: N
authors.lastname: Wieselthaler
authors.affiliation: Paediatric Infectious Diseases Unit, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
authors.forename: Brian
authors.initials: B
authors.lastname: Eley
authors.affiliation: Department of Paediatric Neurology, University of Kwa-Zulu Natal, Inkosi Albert Luthuli Central Hospital, Durban, South Africa.
authors.forename: Lawrence
authors.initials: L
authors.lastname: Mubaiwa
authors.affiliation: Department of Paediatric Neurology, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa jo.wilmshurst@uct.ac.za.
authors.forename: Jo M
authors.initials: JM
authors.lastname: Wilmshurst
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging; D018791:CD4 Lymphocyte Count; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D015658:HIV Infections / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009072:Moyamoya Disease / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000628:therapy; D012189:Retrospective Studies; D013019:South Africa; D019562:Viral Load
keywords: Africa; HIV infection; HIV vasculopathy; cerebrovascular disease; children; moyamoya syndrome