Cerebral vasculopathy associated with collateralization resembling moya moya phenomenon and with anti-Ro/SS-A and anti-La/SS-B antibodies.
We describe a 48-year-old, previously healthy, anti-Ro/SS-A and anti-La/SS-B antibody positive black woman with negative risk factors for atherosclerosis, who developed mental status and personality changes over a 6-12-month period, and progressive cortical blindness over a 2-week period. Angiographic and computed axial tomographic studies of the brain demonstrated multiple large areas of infarction correlating with stenosis and occlusions of the internal carotid and posterior cerebral arteries. Moya moya-like findings were prominent radiographically. Results of angiographic, computed tomographic, and magnetic resonance imaging studies were interpreted as being compatible with large, medium, and small vessel disease, most likely a vasculitis.
Provost
T T
TT
Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205.
Moses
H
H
Morris
E L
EL
Altman
J
J
Harley
J B
JB
Alexander
E
E
Reichlin
M
M
D000792:Angiography; D000974:Antibodies, Antinuclear / Q000032:analysis* / Q000097:blood / Q000134:cerebrospinal fluid; D001766:Blindness / Q000209:etiology; D002339:Carotid Arteries / Q000473:pathology; D002536:Cerebral Arteries / Q000473:pathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D009072:Moyamoya Disease / Q000150:complications* / Q000276:immunology / Q000473:pathology; D014057:Tomography, X-Ray Computed; D014657:Vasculitis / Q000150:complications* / Q000276:immunology / Q000473:pathology
D000974:Antibodies, Antinuclear; C035356:SS-A antibodies; C035357:SS-B antibodies