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Primary angiitis of the central nervous system: avoiding misdiagnosis and missed diagnosis of a rare disease
  1. Shamik Bhattacharyya,
  2. Aaron L Berkowitz
  1. Department of Neurology, Brigham and Women's Hospital, Boston, Massachusetts, USA
  1. Corresponding to Dr Shamik Bhattacharyya, Department of Neurology, Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115, USA; sbhattacharyya3{at}partners.org

Abstract

Primary angiitis of the central nervous system (PACNS) is an extremely rare condition, defined as a vasculitis limited to the CNS with no identifiable cause. Its presentation is non-specific and includes headache, cognitive dysfunction and focal neurological signs. Laboratory studies, neuroimaging and angiography are neither sufficiently sensitive nor specific for diagnosis; a definitive diagnosis requires brain biopsy. As a result, PACNS is commonly misdiagnosed. Here, we review its clinical, laboratory and radiological features, and focus on avoiding common diagnostic pitfalls.

  • VASCULITIS
  • CEREBROVASCULAR DISEASE

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