Cerebral vasculitis presenting with dementia

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By John V Bowler MD

Cerebral vasculitis, particularly primary angiitis of the central nervous system, can be of subtle onset, the manifestations protean, and the investigations often negative; consequently, the diagnosis can be very difficult to make. In this clinical article, Dr. John Bowler reviews the principal features of those vasculitides that may present with cognitive impairment and outlines the extent to which investigations may be falsely negative in primary angiitis of the central nervous system. An illustrative case history demonstrates many of these points. In addition, several other primary vasculitides are outlined.

Key Points

  • Catheter angiography has high false positive and false negative rates in diagnosing primary angiitis of the central nervous system.
  • As the disease is patchy, biopsy may be falsely negative.
  • A diagnosis must be made using all available information; it is the overall pattern and not usually just one investigation that secures an accurate diagnosis.
  • Treatment requires steroids plus a potent immunosuppressant.