title: Primary angiitis of the central nervous system: avoiding misdiagnosis and missed diagnosis of a rare disease.
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.
authors.affiliation: Department of Neurology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
authors.forename: Shamik
authors.identifier: http://orcid.org/0000-0001-8047-3622
authors.initials: S
authors.lastname: Bhattacharyya
authors.affiliation: Department of Neurology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
authors.forename: Aaron L
authors.initials: AL
authors.lastname: Berkowitz
mesh_terms: D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D006801:Humans; D035583:Rare Diseases; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000628:therapy
keywords: CEREBROVASCULAR DISEASE; VASCULITIS