It may produce a wide range of neurological symptoms, such as headache, skin rashes, feeling very tired, joint pains, difficulty moving or coordinating part of the body, changes in sensation, and alterations in perception, thought or behavior, as well as the phenomena of a mass lesion in the brain leading to coma and herniation.

[3] The most frequent presenting signs and symptoms of primary CNS vasculitis were focal neurological deficits (seen in 63% of cases), headaches (51%) and cognitive impairment (41%).

[4] Other presenting symptoms include aphasia or other difficulties with speech (35-43%), ataxia, visual field deficits, acute or subacute encephalopathy (which may progress to coma), and seizures (16-33%).

[3] Primary CNS vasculitis has an incidence of 2.4 cases per 1 million people, with an associated mortality of 8-23% and a 25% risk of severe disability.

[5] Diagnosis of primary CNS vasculitis may be made with brain imaging and biopsy of the affected blood vessel.

[5] Typical findings on angiography include smooth wall segmental stenosis of multiple arteriessometimes with post-stenosis dilation or bleeding.

[5] European guidelines recommend performing a biopsy in suspected cases of small vessel disease with normal angiography.

[4] High resolution MRI may show hyperenhancement of the blood vessel walls in primary CNS vasculitis and may help to distinguish the lesions form atherosclerotic plaques or reversible cerebral vasoconstriction syndrome which have similar morphology on angiography.

And analysis of the cerebrospinal fluid shows a non-specific pattern with mildly elevated leukocytes and increased protein, especially in small vessel disease.