19. A Diagnosis of Exclusion

  1. James C. Pile4,
  2. Thomas E. Baudendistel5 and
  3. Brian J. Harte6
  1. Iris O. Yung1,
  2. Thomas E. Baudendistel5 and
  3. Gurpreet Dhaliwal2,3

Published Online: 21 MAR 2013

DOI: 10.1002/9781118483206.ch19

Clinical Care Conundrums: Challenging Diagnoses in Hospital Medicine

Clinical Care Conundrums: Challenging Diagnoses in Hospital Medicine

How to Cite

Yung, I. O., Baudendistel, T. E. and Dhaliwal, G. (2013) A Diagnosis of Exclusion, in Clinical Care Conundrums: Challenging Diagnoses in Hospital Medicine (eds J. C. Pile, T. E. Baudendistel and B. J. Harte), John Wiley & Sons, Inc., Hoboken, NJ, USA. doi: 10.1002/9781118483206.ch19

Editor Information

  1. 4

    Departments of Hospital Medicine and Infectious Diseases, Cleveland Clinic, Cleveland, Ohio, USA

  2. 5

    Department of Medicine, Kaiser Permanente Medical Center, Oakland, California, USA

  3. 6

    South Pointe Hospital, Cleveland Clinic Health System, Warrensville Heights, Ohio, USA

Author Information

  1. 1

    Department of Medicine, California Pacific Medical Center, San Francisco, California, USA

  2. 2

    Department of Medicine, University of California, San Francisco, California, USA

  3. 3

    Medical Service, San Francisco VA Medical Center, San Francisco, California, USA

  4. 5

    Department of Medicine, Kaiser Permanente Medical Center, Oakland, California, USA

Publication History

  1. Published Online: 21 MAR 2013
  2. Published Print: 15 MAR 2013

Book Series:

  1. Hospital Medicine: Current Concepts

Book Series Editors:

  1. Scott A. Flanders and
  2. Sanjay Saint

ISBN Information

Print ISBN: 9780470905654

Online ISBN: 9781118483206

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Keywords:

  • brain biopsy;
  • central nervous system (CNS);
  • clinical diagnosis;
  • meninges;
  • primary angiitis of central nervous system (PACNS);
  • vasculitis

Summary

Serious disorders that may present with central nervous system (CNS) abnormalities and nondiagnostic abnormal findings on lumbar puncture, brain magnetic resonance imaging (MRI), and cerebral angiography include intravascular lymphoma, prion diseases, cerebritis, paraneoplastic syndromes, and CNS vasculitis. Primary angiitis of the central nervous system (PACNS) is a challenging diagnosis with varied clinical features and often normal angiographic findings. In particular, the specificity of brain MR angiography (MRA) and cerebral angiography is low. Although PACNS is rare, it should be on the differential diagnosis in patients with unexplained neurologic deficits referable to the CNS alone, as the condition is fatal without prompt treatment. A diagnosis of PACNS is made only after excluding secondary causes of CNS vasculitis such as infections, malignancies, autoimmune conditions, reversible cerebral vasoconstrictive disease, and medications. The diagnosis is confirmed with a biopsy of the brain and meninges.