Table 1

Characteristics of patients with fusiform intracranial vasculopathy and HIV infection.

StudyAge (yrs), SexCD4 (cells/mm3) Viral Load (copies/ml)HistoryPresentationLaboratory FindingsImagingTherapyClinical Outcome
Our Patient38 FCD4 < 1, viral load 79,074NoneRight hemiparesisSee text@Head CT: ischemia of bilateral basal ganglia, hemorrhage of right frontal and occipital lobes.
Angiogram: multiple intracranial fusiform and saccular aneurysms in anterior and posterior circulation
Steroids, started ARV, anti-CMV therapy for retinitisNew hemorrhage on day 39.
Followup angiogram showed resolution. No new events at 18 mo followup
Modi937 MCD4 = 164NoneSevere cognitive impairmentCSF: protein 1.06 g/1, glucose 2.7 mmol/1, 3 neutrophils/ml, 41 lymphocytes/ml. CSF India ink negative, adenosine deaminase normal, syphilis serology negative, MTB culture negativeAngiogram: fusiform aneurysmal dilatation of bilateral ACA and MCATreated for presumed TB meningitis. No ARVLost to followup
43 MCD4 = 172On therapy for pulmonary TBHeadache, meningismus, confusionHead CT: SAH. Angiogram/surgery: Bilateral MCA fusiform aneurysmsUnable to repair right MCA aneurysm. No ARVDied of renal failure
43 MCD4 = 17On therapy for pulmonary TBGeneralized tonic-clonic seizuresCSF: protein 0.82 g/1, glucose 2.0 mmol/1, no cells. CSF adenosine deaminase normal. Serum ESR 130 mm/hCT angiogram: multiple fusiform intracranial aneurysms. + aneurysmal dilatation of ascending aortaStarted ARVLost to followup
Hamilton1234 MCD4 = 66, viral load “undetectable”Endstage renal diseaseHeadache, fever left hemiparesisHead CT: diffuse SAH. CT angiography: diffuse vasculopathy of all major cerebral vessels with multiple fusiform and giant saccular aneurysmsUnclear treatmentDischarged after 7 days
Ake11CD4 = 15, VL 191,429Recurrent VZV infectionsCognitive and language deficitsCSF: WBC 44 cells/mm3 (86% neutrophil ,11% lymphocyte, 1 % monocyte, 2% eosinophils), RBC 305,000 cells/mm3, glucose 24 mg/dl, Negative CSF bacterial culture, fungal culture, AFB culture. Negative CSF DNA PCR for CMV, Epstein-Barr virus, human herpes virus-6 and -8, herpes simplex virus, VZV. CSF HIV RNA <30 copies/ml. CSF VDRL and cryptococcal antigen negative. ESR 38 mm/hHead CT: hemorrhage in Sylvian fissure, lateral ventricle; SAH. Angiogram: Diffuse fusiform aneurysms and stenoses in anterior and posterior circulationsContinued noncompliance with ARVFatal subarachnoid hemorrhage 3 weeks after presentation
Tipping527 FCD4 = 14NoneRight hemiparesisCSF: 7 lymphocytes/mm3 3 polymorphs/mm3, protein 1.0 g/1, glucose 41 mg/dl. Negative CSF cryptococcal antigen, rapid plasma reagin, and cultures for bacteria, fungi, and tuberculosisCT: left basal ganglia infarct and fusiform dilation of L MCA, L ACA and distal basilar artery_Died of pneumonia 25 days after presentation
Kossorotoff623 MCD4 = 4962 previous MCA strokesRecurrent left MCA strokeCSF normalMRI: recurrent L MCA infarct. Angiogram: large L terminal ICA aneurysm; multiple ectasias alternating w/stenotic lesions on medium-size arteriesContinued ARVAlive at 9 yrs
32CD4 = 338Recurrent VZV infectionsChronic headacheCSF: WBC 13/mm3, protein 1.09 g/dl, glucose 2.1 mmol/1. CSF PCR VZV negative. Serologies positive for Epstein-Barr virus, human herpes virus-8, and CMVMRI: R MCA infarct Angiogram: multiple ectasia and focal stenoses on medium and small cerebral arteriesAspirin. Continued ARVAlive at 1 year. Followup MRA at 1 yr unchanged
O’Charoen736 MCD4 = 43, VL 298,000Polycystic kidney diseaseDysarthria, right-side weaknessCSF: WBC 1/mm3, RBC 8/mm3, protein 65 mg/dl, glucose 49 mg/dl. Negative CSF India ink, bacterial culture, VDRL, cryptococcus agglutination, CMV DNA PCR, VZV DNA PCR. ESR 95 mm/h, mg/dl. CRP 8.2 mg/dlMRI: L internal capsule .lateral thalamus infarct. MRA: fusiform aneurysmal dilation in A2 segments of bilateral ACA, MCA, postcerebral and basilar arteriesDid not receive
Berkefeld837 MMCA infarct 3 mo prior to presentationProgressive L hemiparesis and visual field disturbancesCSF: 7 WBC/μ1 protein 0.85 g/1. Microbiology examinations of CSF and serum normal. ESR 65 mm/h, CRP 0.9 mg/dlMRA: RICA and MCA showed moderate arterial dilation, thickening, and contrast enhancement of walls. MRI: R MCA infarctPenicillin G, corticosteroids (dose not given), azathioprine. Started ARVAlive at 3 mo
31 MSerum VZV antibody elevated, serum VZV PCR positiveRecurrent TIA, aphasia, right hemiparesisCSF: WBC 9/μ1. Serum VZV antibody elevation and positive serum VZV PCRMRI: L MCA infarcts. MCA aneurysmal dilation, thickening, and contrast enhancement of arterial wallImproved with acyclovir and corticosteroids (decortine 1000 mg for 4 days). Started ARVAlive at 4 mo
  • Dash denotes information not given. CT: computed tomography; CMV: cytomegalovirus; CSF: cerebrospinal fluid; MCA: middle cerebral artery; ARV: antiretroviral; TB: tuberculosis; ESR: erythrocyte sedimentation rate; SAH: subarachnoid hemorrhage; PCR: polymerase chain reaction; HIV: human immunodeficiency virus; CRP: C-reactive protein; ACA: anterior cerebral artery; MRI: magnetic resonance imaging; MRA: magnetic resonance angiogram; WBC: white blood cells; RBC: red blood cells; ICA: internal carotid artery; VZV: varicella zoster virus; TIA: transient ischemic attack; MTB: mycobacterium tuberculosis.