Moyamoya Syndrome in South African Children With HIV-1 Infection

Area of Science:

• Neurology
• Infectious Diseases
• Pediatrics

Background:

• HIV-1 infection can lead to serious complications in children.
• HIV-associated vasculopathy, including moyamoya syndrome, is a recognized but less understood complication.
• Limited access to healthcare in Sub-Saharan Africa may impact diagnosis and management.

Purpose of the Study:

• To describe the clinical characteristics and neuroimaging findings of vertically acquired HIV-1 infected children with moyamoya syndrome.
• To investigate the potential association between HIV-1 management, antiretroviral therapy access, and the prevalence of this condition.
• To highlight diagnostic challenges and the potential for silent disease progression.

Main Methods:

• A national multicenter study involving 17 South African children with vertically acquired HIV-1 infection and vasculopathy.
• Detailed clinical assessment, including CD4 counts and viral loads.
• Neuroimaging studies to evaluate vascular changes consistent with moyamoya syndrome.

Main Results:

• Five children (all of African ancestry) presented with progressive moyamoya syndrome, with a median age of 5.8 years.
• These children had abnormal CD4 counts and elevated viral loads, with symptoms including motor deficits and neuroregression.
• Neuroimaging confirmed progressive vascular disease, with evidence of a preceding silent phase. One patient showed recovery with improved CD4 counts.

Conclusions:

• Vertically acquired HIV-1 infection is associated with moyamoya syndrome in children, particularly in those with limited access to antiretroviral therapy.
• The syndrome can have an insidious, silent course, presenting with non-specific neurological symptoms like cognitive delay.
• Underdiagnosis may occur in resource-limited settings like Sub-Saharan Africa due to limited neuroimaging access.