Central Nervous System Vasculitis in Children

  • Kalthoum Tlili-Graiess
  • Nadia Mama-Larbi
  • Nadine Girard
  • Charles Raybaud
Part of the Medical Radiology book series (MEDRAD)


Central nervous system (CNS) vasculitis in children may develop as a primary condition, or secondary to an underlying systemic disease. Accurate and early diagnosis is mandatory in order to prevent severe neurological complications. Neuroimaging play an important role in advancing the diagnosis. Cerebral digital subtraction angiography (DSA), MRI, and MRA are the most useful examinations for vasculitis. MRI appears as the modality of choice for diagnosis and follow-up evaluation of cerebral involvement with vasculitis. MRA will be the first angiographic examination in the diagnostic workup of suspected cerebral vasculitis. DSA remains an indispensable tool in some cases for the investigation of medium and small brain artery stenosis. The infectious causes of vasculitis, including bacterial, viral, and fungal infections, are only a small component of the overall list of etiologies but among the more treatable causes if diagnosed early and treated effectively. Cerebral complications associated with systemic vasculitis can occur from hypertensive encephalopathy secondary to renovascular hypertension, or direct intracranial vessel involvement. Childhood primary CNS vasculitis (PVCNS) includes two distinct and actually well-recognized subtypes: angiography-positive PVCNS affecting large-and medium-sized vessels with a progressive and non-progressive forms, and angiography-negative PVCNS involving small-sized vessels. These subtypes are defined by clinical features, neuroimaging characteristics, and brain biopsy findings and each mandates a distinct therapeutic approach. Therefore the diseases that must be considered in children presenting with features suggesting angiography-positive PVCNS are distinctly different from those in suspected angiography-negative PVCNS.


Magnetic Resonance Angiography Digital Subtraction Angiography Kawasaki Disease Central Nervous System Involvement Takayasu Arteritis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2011

Authors and Affiliations

  • Kalthoum Tlili-Graiess
    • 1
  • Nadia Mama-Larbi
    • 1
  • Nadine Girard
    • 2
  • Charles Raybaud
    • 3
  1. 1.Medical school of Sousse, Department of Medical ImagingSahloul University HospitalSousseTunisia
  2. 2.Medical School of MarseilleLa Timone HospitalMarseilleFrance
  3. 3.Derek Harwood-Nash Chair in Medical Imaging, Department of Diagnostic Imaging, Hospital for Sick ChildrenUniversity of TorontoTorontoCanada

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