Characteristics of patients with fusiform intracranial vasculopathy and HIV infection.
| Study | Age (yrs), Sex | CD4 (cells/mm3) Viral Load (copies/ml) | History | Presentation | Laboratory Findings | Imaging | Therapy | Clinical Outcome |
|---|---|---|---|---|---|---|---|---|
| Our Patient | 38 F | CD4 < 1, viral load 79,074 | None | Right hemiparesis | See 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 retinitis | New hemorrhage on day 39. Followup angiogram showed resolution. No new events at 18 mo followup |
| Modi9 | 37 M | CD4 = 164 | None | Severe cognitive impairment | CSF: 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 negative | Angiogram: fusiform aneurysmal dilatation of bilateral ACA and MCA | Treated for presumed TB meningitis. No ARV | Lost to followup |
| 43 M | CD4 = 172 | On therapy for pulmonary TB | Headache, meningismus, confusion | — | Head CT: SAH. Angiogram/surgery: Bilateral MCA fusiform aneurysms | Unable to repair right MCA aneurysm. No ARV | Died of renal failure | |
| 43 M | CD4 = 17 | On therapy for pulmonary TB | Generalized tonic-clonic seizures | CSF: protein 0.82 g/1, glucose 2.0 mmol/1, no cells. CSF adenosine deaminase normal. Serum ESR 130 mm/h | CT angiogram: multiple fusiform intracranial aneurysms. + aneurysmal dilatation of ascending aorta | Started ARV | Lost to followup | |
| Hamilton12 | 34 M | CD4 = 66, viral load “undetectable” | Endstage renal disease | Headache, fever left hemiparesis | — | Head CT: diffuse SAH. CT angiography: diffuse vasculopathy of all major cerebral vessels with multiple fusiform and giant saccular aneurysms | Unclear treatment | Discharged after 7 days |
| Ake11 | CD4 = 15, VL 191,429 | Recurrent VZV infections | Cognitive and language deficits | CSF: 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/h | Head CT: hemorrhage in Sylvian fissure, lateral ventricle; SAH. Angiogram: Diffuse fusiform aneurysms and stenoses in anterior and posterior circulations | Continued noncompliance with ARV | Fatal subarachnoid hemorrhage 3 weeks after presentation | |
| Tipping5 | 27 F | CD4 = 14 | None | Right hemiparesis | CSF: 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 tuberculosis | CT: left basal ganglia infarct and fusiform dilation of L MCA, L ACA and distal basilar artery | _ | Died of pneumonia 25 days after presentation |
| Kossorotoff6 | 23 M | CD4 = 496 | 2 previous MCA strokes | Recurrent left MCA stroke | CSF normal | MRI: recurrent L MCA infarct. Angiogram: large L terminal ICA aneurysm; multiple ectasias alternating w/stenotic lesions on medium-size arteries | Continued ARV | Alive at 9 yrs |
| 32 | CD4 = 338 | Recurrent VZV infections | Chronic headache | CSF: 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 CMV | MRI: R MCA infarct Angiogram: multiple ectasia and focal stenoses on medium and small cerebral arteries | Aspirin. Continued ARV | Alive at 1 year. Followup MRA at 1 yr unchanged | |
| O’Charoen7 | 36 M | CD4 = 43, VL 298,000 | Polycystic kidney disease | Dysarthria, right-side weakness | CSF: 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/dl | MRI: L internal capsule .lateral thalamus infarct. MRA: fusiform aneurysmal dilation in A2 segments of bilateral ACA, MCA, postcerebral and basilar arteries | Did not receive | — |
| Berkefeld8 | 37 M | — | MCA infarct 3 mo prior to presentation | Progressive L hemiparesis and visual field disturbances | CSF: 7 WBC/μ1 protein 0.85 g/1. Microbiology examinations of CSF and serum normal. ESR 65 mm/h, CRP 0.9 mg/dl | MRA: RICA and MCA showed moderate arterial dilation, thickening, and contrast enhancement of walls. MRI: R MCA infarct | Penicillin G, corticosteroids (dose not given), azathioprine. Started ARV | Alive at 3 mo |
| 31 M | — | Serum VZV antibody elevated, serum VZV PCR positive | Recurrent TIA, aphasia, right hemiparesis | CSF: WBC 9/μ1. Serum VZV antibody elevation and positive serum VZV PCR | MRI: L MCA infarcts. MCA aneurysmal dilation, thickening, and contrast enhancement of arterial wall | Improved with acyclovir and corticosteroids (decortine 1000 mg for 4 days). Started ARV | Alive 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.