Cerebral aneurysmal arteriopathy in childhood AIDS

Objective: To identify cerebral aneurysmal arteriopathy in children with longstanding AIDS. Background Five cases are described from the authors' experience, and eight additional cases are reviewed from the literature. Details are presented in regard to the clinical picture as well as brain imaging with cerebral angiography and magnetic resonance angiography in some cases. Autopsy information is available in four cases, including one of the authors' patients. Results: Seven patients acquired HIV infection perinatally, five patients were infected by blood transfusions, and one patient had both risk factors. Ln the four postmortem patients, the vascular pathology was similar, showing ectasia and aneurysmal dilatation confined to the large arteries of the circle of Willis. Medial fibrosis and loss of muscularis with destruction of the internal elastic lamina and intimal hyperplasia was common. The latency period following infection varied from 2 to 11 years. Once a diagnosis of aneurysmal arteriopathy was made, the patients deteriorated rapidly, with death occurring in less than 6 months. Conclusions: The development of cerebral aneurysmal arteriopathy in childhood AIDS tends to occur after a prolonged delay and is usually followed by death in a short period of time. The etiology for the vasculitis is unknown. Varicella tester virus may be the etiology in some of the eases because of its potential to cause this pathology and the striking unilateral arterial involvement found in Case 5. HIV vasculitis is also a possibility, as suggested by the detection of HIV protein or genomic material in two of the four autopsy cases.