Benign angiopathy of the central nervous system.
Apparent central nervous system vasculitis could have a benign course in a subset of patients and is referred to as benign angiopathy of the central nervous system (BACNS). This is mostly is seen in young women and presents with acute onset of neurologic symptoms, usually in the form of severe headache and/or a focal neurologic event. Cerebrospinal fluid analysis could be normal or nearly normal. Brain angiogram typically shows segmental narrowing, ectasia, or beading in multiple vascular territories on brain angiogram. Like in all cases of CNS vasculitis, exclusion of other systemic diseases or its mimickers associated with cerebral involvement is important.BACNS could respond favorably to treatment with corticosteroids alone or in combination with a calcium channel blocker, thereby avoiding unnecessary and prolonged treatment with other immunosuppressants like cyclophosphamide with their side effects. Little is published in the literature about BACNS, but is a rapidly emerging concept. It is thus important to recognize. We report a patient with classic presentation of BACNS to highlight the significance of distinguishing benign angiopathy from the more aggressive form of CNS vasculitis, because the treatment and response varies in these 2 entities.
Jolly
Meenakshi
M
Section of Rheumatology, Department of Medicine, Advocate Christ Medical Center, Oak Lawn, Illinois 60453, USA. Meenakshi.Jolly-MD@advocatehealth.com
Curran
James J
JJ
Ellman
Michael
M