@article {Bhattacharyya195, author = {Shamik Bhattacharyya and Aaron L Berkowitz}, title = {Primary angiitis of the central nervous system: avoiding misdiagnosis and missed diagnosis of a rare disease}, volume = {16}, number = {3}, pages = {195--200}, year = {2016}, doi = {10.1136/practneurol-2015-001332}, publisher = {BMJ Publishing Group Ltd}, 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.}, issn = {1474-7758}, URL = {https://pn.bmj.com/content/16/3/195}, eprint = {https://pn.bmj.com/content/16/3/195.full.pdf}, journal = {Practical Neurology} }