Severe cerebral vaso-occlusive disease in macrophage activation syndrome

Mohamad K El-Bitar; Samar A Muwakkit; Rola Hourani; Miguel R Abboud; Raya Saab; Omar Dabbagh

doi:10.1016/j.pediatrneurol.2009.11.007

Severe cerebral vaso-occlusive disease in macrophage activation syndrome

Pediatr Neurol. 2010 Apr;42(4):283-6. doi: 10.1016/j.pediatrneurol.2009.11.007.

Authors

Mohamad K El-Bitar¹, Samar A Muwakkit, Rola Hourani, Miguel R Abboud, Raya Saab, Omar Dabbagh

Affiliation

¹ Division of Pediatric Neurology and Pediatric Epilepsy Program, Department of Pediatrics, American University of Beirut Medical Center, 2020l Beirut, Lebanon.

PMID: 20304334
DOI: 10.1016/j.pediatrneurol.2009.11.007

Abstract

A 14-year-old girl was diagnosed with macrophage activation syndrome, based on clinical presentation, laboratory tests, and bone marrow aspirate findings. She developed severe central nervous system involvement in the form of seizure disorder and severe diffuse occlusive cerebral vasculopathy, with extensive collateral circulation consistent with moyamoya disease. To our knowledge, this description is the first of these findings in association with macrophage activation syndrome.

Publication types

Case Reports

MeSH terms

Adolescent
Bone Marrow / pathology
Brain / blood supply
Brain / pathology
Cerebral Angiography
Cerebrovascular Disorders / complications*
Cerebrovascular Disorders / pathology
Female
Humans
Macrophage Activation Syndrome / complications*
Macrophage Activation Syndrome / diagnosis
Macrophage Activation Syndrome / pathology
Magnetic Resonance Angiography
Magnetic Resonance Imaging
Moyamoya Disease / complications
Moyamoya Disease / pathology
Seizures / complications
Seizures / pathology
Severity of Illness Index