Primary Central Nervous System Angiitis

  • M. Habib Bouhaouala
  • Olivier Naggara
  • Dorra Ghorbel
  • Akil Mestiri
  • J. François Meder
  • Lotfi Hendaoui
Part of the Medical Radiology book series (MEDRAD)


Primary central nervous system angiitis is an uncommon idiopathic vasculitis involving small and medium-sized artery walls. It requires a prompt diagnosis of certainty and an early initiation of treatment. The diagnostic approach represents a challenge because of the clinical polymorphism, the absence of specific laboratory tests and the imperfect specificity of currently available neuroimaging techniques. The absence of both systemic inflammation and specific auto antibodies in the presence of inflammatory signs in the cerebral spinal fluid (CSF) analysis constitute strong arguments of presumptive diagnosis. MRI evocative signs are cortical or deep multifocal ischemic lesions of variable age and size associated with petechial haemorrhages and cortico-meningeal contrast enhancement testifies to a diffuse inflammation. Conventional cerebral angiography (CCA) may demonstrate non-specific concomitant segmental stenosis and dilation of intracranial arteries. At present brain biopsy is still the gold standard for diagnostic certainty. CT and MR angiography have been constantly improving so that they are being used more often than CCA and have become important diagnostic tools for PCNSA. The most efficient treatment combines cyclophosphamide and glucocorticoids but the prognosis remains poor.


Magnetic Resonance Imaging Apparent Diffusion Coefficient Cerebral Spinal Fluid Cerebral Amyloid Angiopathy Brain Biopsy 
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

  • M. Habib Bouhaouala
    • 1
  • Olivier Naggara
    • 3
  • Dorra Ghorbel
    • 1
  • Akil Mestiri
    • 2
  • J. François Meder
    • 3
  • Lotfi Hendaoui
    • 4
  1. 1.Department of Medical ImagingMedical School of Tunis, Interior Security Forces Teaching HospitalLa MarsaTunisia
  2. 2.Department of Internal Medicine OrganizationMedical School of Tunis, Interior Security Forces Teaching HospitalLa MarsaTunisia
  3. 3.Department of Morphological and Functional ImagingMedical School of Paris-Descartes, INSERM UMR 894, Sainte-Anne University HospitalParis Cedex 14France
  4. 4.Department of Medical Imaging and Imaging Guided InterventionsMedical School of Tunis, Mongi Slim University HospitalLa MarsaTunisia

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