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Primary Central Nervous System Angiitis

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Systemic Vasculitis

Part of the book series: Medical Radiology ((Med Radiol Diagn Imaging))

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Abstract

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.

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Authors and Affiliations

  1. Department of Medical Imaging, Medical School of Tunis, Interior Security Forces Teaching Hospital, 2070, La Marsa, Tunisia

    M. Habib Bouhaouala & Dorra Ghorbel

  2. Department of Internal Medicine Organization, Medical School of Tunis, Interior Security Forces Teaching Hospital, 2070, La Marsa, Tunisia

    Akil Mestiri

  3. Department of Morphological and Functional Imaging, Medical School of Paris-Descartes, INSERM UMR 894, Sainte-Anne University Hospital, 1 rue Cabanis, 75674, Paris Cedex 14, France

    Olivier Naggara & J. François Meder

  4. Department of Medical Imaging and Imaging Guided Interventions, Medical School of Tunis, Mongi Slim University Hospital, 2046, La Marsa, Sidi Daoud, Tunisia

    Lotfi Hendaoui

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  1. M. Habib Bouhaouala
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  2. Olivier Naggara
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Correspondence to M. Habib Bouhaouala .

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  1. Medical School of Tunis, Department of Medical Imaging, Hôpital Mongi Slim, La Marsa, 2046, Tunisia

    Lotfi Hendaoui

  2. Mayo Graduate School of Medicine, Gonda Vascular Center, Mayo Clinic, Rochester, 55902, Minnesota, USA

    Anthony W. Stanson

  3. Medical School of Tunis, Department of Medical Imaging, Interior Security Forces Teaching Hospit, La Marsa, 2070, Tunisia

    M. Habib Bouhaouala

  4. Medical School of Toulouse University, Department of Medical Imaging, Rangueil Hospital, Toulouse CX 9, 31059, France

    Francis Joffre

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Bouhaouala, M.H., Naggara, O., Ghorbel, D., Mestiri, A., Meder, J.F., Hendaoui, L. (2011). Primary Central Nervous System Angiitis. In: Hendaoui, L., Stanson, A., Bouhaouala, M., Joffre, F. (eds) Systemic Vasculitis. Medical Radiology(). Springer, Berlin, Heidelberg. https://doi.org/10.1007/174_2011_235

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