Radiological Case of the Month | Allergy and Clinical Immunology | JAMA Pediatrics | JAMA Network
[Skip to Navigation]
Sign In
Special Feature
July 2001

Radiological Case of the Month

Arch Pediatr Adolesc Med. 2001;155(7):850. doi:10.1001/archpedi.155.7.849

Denouement and Discussion: Aneurysmal Dilation of Cerebral Arteries Associated With HIV Infection

Figure 1, Figure 2, and Figure 3. Magnetic resonance image at age 8½ years. Adjacent proton-weighted axial sections show ectasia (aneurysmal dilation) beginning at the carotid siphon and extending into proximal middle cerebral artery.

Figure 4. Magnetic resonance angiogram at age 8½ years. Coronal reconstruction in the frontal plane shows a large fusiform aneurysm extending from the carotid siphon into the proximal middle and anterior cerebral arteries.

Neurologic abnormalities are documented as occurring frequently in HIV-infected infants and children.1 General symptoms range from developmental regression to hyperactivity and learning disabilities. Corticospinal tract degeneration may occur with clinical clonus and hyperactive stretch reflexes.2 Localized neurologic deficits may also occur, usually secondary to infection with pathogenic or opportunistic organisms or lymphoma. Progressive multifocal leukoencephaly occurs late and can cause localized or general symptoms.3 Vascular abnormalities are described in HIV-infected infants and children. Bleeding secondary to thrombocytopenia, thromboses secondary to bacterial or fungal infection, and cardiomyopathy with consequent embolization occur.3 Drug-induced vasculitis, Henoch-Schöenlein purpura, and varicella-induced vasculitis may be present.

Recently, a number of HIV-infected children were reported to have remarkable aneurysmal dilation of the large cerebral vessels, particularly the Circle of Willis.4-11 Children with this abnormality usually present with an acute intracerebral event. Their age is past infancy, and they are usually severely immunologically compromised.5 Only rarely is a neurologic event the initial symptom of HIV infection.

The cause of the aneurysmal changes of vessels is unknown, although the aneurysms may be congenital rather than inflammatory. Many patients, including our own, have normal findings from scans prior to the acute neurologic event. Postmortem studies show arterial medial fibrosis, destruction of internal elastic lamina, and intimal hyperplasia.5,6,10 Speculation suggests that bacterial or fungal infection plays a role in some cases. Varicella infection has been implicated,5 as has HIV, as a causative agent.7 Therapy has been problematic because of the crucial location and nature of the aneurysms.

Our patient showed progression of neurologic signs during the following 2 years despite a marked rise (and subsequent fall) in his CD4 cell count. He remains fully functional in activities of daily living. Because of the surgical risk, no intervention has been attempted. He continues to be monitored closely, and his HIV is treated aggressively pharmacologically.

Accepted for publication December 21, 1999.

Presented as a poster at Southern Society Pediatric Research, New Orleans, La, February 7, 1998.

Reprints: Dianne S. Elfenbein, MD, Department of Pediatrics, University of South Florida, 17 Davis Blvd, Suite 308, Tampa, FL 33606.

Lobato  MNCaldwell  MBNg  POxtoby  MJ Encephalopathy in children with perinatally acquired HIV infection.  J Pediatr. 1995;126710- 715Google ScholarCrossref
Dickson  DWBelman  ALKim  TSHoroupian  DSRubinstein  A Spinal cord pathology in pediatric AIDS.  Neurology. 1989;39227- 235Google ScholarCrossref
Zukerman  GBSanchez  JLConway  EE Neurologic complications of HIV infections in children.  Pediatr Ann. 1998;27636- 639Google Scholar
Fulmer  BDDillard  SCMusulman  EMPalmer  CAOakes  J Two cases of cerebral aneurysms in HIV+ children.  Pediatr Neurosurg. 1998;2831- 34Google ScholarCrossref
Dubrovsky  TCurless  RScott  G  et al.  Cerebral aneurysmal arteriopathy in childhood AIDS.  Neurology. 1998;51560- 565Google ScholarCrossref
Shah  SSZimmerman  RARorke  LBVezina  LG Cerebrovascular complications of HIV in children.  AJNR Am J Neuroradiol. 1996;171913- 1917Google Scholar
Moriarty  DMHaller  JOLoh  JPFikrig  S Cerebral infarction in pediatric acquired immunodeficiency syndrome.  Pediatr Radiol. 1994;24611- 612Google ScholarCrossref
Philippet  PBlanche  SSebag  GRodesch  GGriscelli  CTardieu  M Stroke and cerebral infarcts in children infected with human immunodeficiency virus.  Arch Pediatr Adolesc Med. 1994;148965- 970Google ScholarCrossref
Husson  RNSaini  RLewis  LLButler  KPatronas  NPizzo  PA Cerebral artery aneurysms in children infected with human immunodeficiency-deficiency-virus.  J Pediatr. 1992;121927- 930Google ScholarCrossref
Park  YDBelman  ALKim  T-S  et al.  Stroke in pediatric acquired immunodeficiency syndrome.  Ann Neurol. 1990;28303- 311Google ScholarCrossref
Frank  YLim  WKahn  EFarmer  PGorey  MPahwa  S Multiple ischemic infarcts in a child with AIDS, varicella zoster infection, and cerebral vasculitis.  Pediatr Neurol. 1989;564- 67Google ScholarCrossref