Paroxysmal nocturnal hemoglobinuria presenting as moyamoya syndrome.
We report an 11-year-old girl who has paroxysmal nocturnal hemoglobinuria (PNH) and was admitted because of recurrent cerebrovascular accidents (CVA) and intermittent hemoglobinuria. Internal carotid angiography revealed bilateral typical moyamoya patterns. Although CVA due to arterial thrombosis may occur in PNH, the basal moyamoya vessels were never mentioned in case reports yet. The moyamoya syndrome has been reported in a variety of diseases and represents the nonspecific response to an impairment of arterial flow at specific sites in the brain. Our case discloses that PNH may present as moyamoya syndrome.
Lin
H C
HC
Department of Pediatrics, National Taiwan University Hospital, Taipei.
Chen
R L
RL
Wang
P J
PJ
D002533:Cerebral Angiography; D002561:Cerebrovascular Disorders / Q000097:blood / Q000175:diagnosis / Q000473:pathology; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D009072:Moyamoya Disease / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D014057:Tomography, X-Ray Computed