Some patients presenting with primary angiitis of the central nervous system may have a nonprogressive and relatively benign course. Hajj-Ali et al refer to these patients as Benign Angiopathy of the Central Nervous System (BACNS).
Features of BACNS:
(1) Most patients are females.
(2) The onset is usually acute.
(3) Headache is the most common presenting complaint. Focal or diffuse neurologic symptoms may be present.
(4) All patients have markedly abnormal cerebral angiography consistent with vasculitis.
(5) Most patients show abnormalities on MRI.
(6) There are no or minor changes in the CSF protein concentration and leukocyte count.
(7) The condition is rapidly reversible with treatment.
(8) Most patients recover without disability. If disability is present it is usually mild.
Features of CNS vasculitis on angiography:
(1) involvement of >= 1 major branch of the anterior and posterior carotid arteries or subbranches
(2) areas of stenosis and dilatation which may alternate ("vascular beading")
Vasospasm is the presumed mechanism.
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