Primary angiitis of the CNS (PACNS)

Primary angiitis of the CNS (PACNS) is a rare inflammatory disease affectin g middle-aged patients. The angiitis is focal and segmental in distribution , involving small and medium-sized leptomeningeal and intracranial vessels. The most frequent presenting symptoms are headaches, focal neurologic defi cits, and confusion. In addition, aphasia, neuropsychological deficits, and seizures may occur. The paper presents a review of the literature. In addi tion,a typical case of PACNS is presented. This case demonstrates that diag nosis of PACNS is difficult and brain biopsy is essential to confirm it.