Severe Cerebral Vaso-Occlusive Disease in Macrophage Activation Syndrome

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...

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Bibliographic Details
Published in:Pediatric neurology 2010-04, Vol.42 (4), p.283-286
Main Authors: El-Bitar, Mohamad K., MD, Muwakkit, Samar A., MD, Hourani, Rola, MD, Abboud, Miguel R., MD, Saab, Raya, MD, Dabbagh, Omar, MD
Format: Article
Language:English
Subjects:
Adolescent
Biological and medical sciences
Bone Marrow - pathology
Brain - blood supply
Brain - pathology
Cerebral Angiography
Cerebrovascular Disorders - complications
Cerebrovascular Disorders - pathology
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Humans
Macrophage Activation Syndrome - complications
Macrophage Activation Syndrome - diagnosis
Macrophage Activation Syndrome - pathology
Magnetic Resonance Angiography
Magnetic Resonance Imaging
Medical sciences
Moyamoya Disease - complications
Moyamoya Disease - pathology
Neurology
Pediatrics
Seizures - complications
Seizures - pathology
Severity of Illness Index
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Description
Summary: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.
ISSN:0887-8994
1873-5150
DOI:10.1016/j.pediatrneurol.2009.11.007