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Primary Angiitis of the Central Nervous System

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MR Imaging in White Matter Diseases of the Brain and Spinal Cord

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

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Conclusion

Physicians are faced daily with the challenges of accurately diagnosing patients by employing the current diagnostic criteria, recommended structural neuroimaging techniques, and/or guidelines put forth by ad hoc committees, consensus groups, and data from large, multicenter, placebo-controlled studies. A comprehensive understanding of the approach to PACNS from the etiology to optimal treatment is currently not clearly defined. This is a rare disorder that represents a unique diagnostic challenge for the neurologist given the lack of uniformity with respect to the clinical presentation, radiological and histopathological findings, and the varying natural history of the disease. This condition is restricted to the CNS with no signs of systemic involvement and biopsy of the brain parenchyma and leptomeninges is paramount in the diagnosis. A delay in the diagnosis and proper treatment of the disorder may cause increased morbidity and death. Current treatment strategies should be initiated aggressively, but only after confirmation of the diagnosis has been made. The diagnosis should not be made based on any single study. Instead, historical, clinical, serological, radiological, and histopathological correlation is needed for an accurate diagnosis. It is only by this approach that appropriate treatment interventions can be instituted in a timely manner to increase the likelihood of a better outcome. Future studies may focus on the genetic predilection for this condition which may provide insight into its pathogenesis and varied clinical course as well as treatment response.

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Authors and Affiliations

  1. Barrow Neurological Institute, Division of Neurology, St. Joseph’s Hospital & Medical Center, 350 W. Thomas Road, Phoenix, Arizona, 85013, USA

    Darin T. Okuda MD & Timothy L. Vollmer MD

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  1. Darin T. Okuda MD
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Editors and Affiliations

  1. Neuroimaging Research Unit Department of Neurology Scientific Institute, University Ospedale San Raffaele, Via Olgettina, 60, 20132, Milan, Italy

    Massimo Filippi MD (Director) (Director)

  2. Department of Neurology & Behavioral Sciences, University of Siena, Viale Bracci 2, 53100, Siena, Italy

    Nicola De Stefano MD PhD (Associate Professor) (Associate Professor)

  3. Service de Neuroradiologie Diagnostique et Thérapeutique CHU Bordeaux Pellegrin and Laboratoire de Neurobiologie des Affections de la Myéline, Université Victor Segalen, Bourdeaux 2, 33076, Bordeaux, France

    Vincent Dousset MD, PhD (Professor) (Professor)

  4. Department of Electrical Engineering, United States Naval Academy, Maury Hall 227, Annapolis, MD, 21402-5025, USA

    Joseph C. McGowan PhD (Associate Professor of Electrical Engineering) (Associate Professor of Electrical Engineering)

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Okuda, D.T., Vollmer, T.L. (2005). Primary Angiitis of the Central Nervous System. In: Filippi, M., De Stefano, N., Dousset, V., McGowan, J.C. (eds) MR Imaging in White Matter Diseases of the Brain and Spinal Cord. Medical Radiology Diagnostic Imaging. Springer, Berlin, Heidelberg. https://doi.org/10.1007/3-540-27644-0_20

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