Primary and Secondary Vasculitis of the Central Nervous System

  • V. Martinelli
  • A. Manfredi
  • L. Moiola
  • M. G. Sabbadini
  • G. Comi
Part of the Topics in Neuroscience book series (TOPNEURO)


Vasculitis is characterised by inflammation of the arterial wall, with possible involvement of adjacent tissues. Besides tissue injury due to ischaemia, sustained inflammation sometimes compromises vessel integrity. The central nervous system (CNS) is the target organ of isolated, or primary, vasculitis (primary angiitis of the CNS, or PACNS). Far more frequently, the involvement of the CNS is part of a more complex scenario involving other organs or systems (systemic vasculitis) [1]. Understanding the immunopathological processes underlying vasculitis, as well as the role of both local and distal regulatory control on vascular inflammation, remains an intriguing field of interest.
Table 1

Possible causes of opsoclonus-myoclonus syndrome


Mumps (paramyoviridae) [46]

Epstein-Barr virus [47, 48]

Coxsackie B3 [49]


Neuroblastoma [2, 5, 6 , 50, 51, 52]

Hepatoblastoma [5]

Ganglioneuroma [6]

Other agents known to cause symptoms of opsoclonus











Systemic Lupus Erythematosus Giant Cell Arteritis Polyarteritis Nodosa Temporal Arteritis Hypersensitivity Vasculitis 
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 Italia 2002

Authors and Affiliations

  • V. Martinelli
    • 1
  • A. Manfredi
    • 2
  • L. Moiola
    • 1
  • M. G. Sabbadini
    • 2
  • G. Comi
    • 1
  1. 1.Department of NeuroscienceSan Raffaele Scientific InstituteMilanItaly
  2. 2.Department of MedicineSan Raffaele Scientific InstituteMilanItaly

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