||Rapid Contralateral Progression of Focal Cerebral Arteriopathy Distinguished from RNF213-related moyamoya disease and fibromuscular dysplasia
Yoshio, Araki ,
Yasushi, Takagi ,
Yohei, Mineharu ,
Hatasu, Kobayashi ,
Susumu, MiyamotoToshihiko, Wakabayashi
1409 , 2017-08 , Springer
Background Focal cerebral arteriopathy includes unifocal ormultifocal lesions that are unilateral or bilateral. Large- and/ormedium-sized vessels are involved and can be visualized onangiography.Case report We report a case of cerebral infarction in a 9-yearoldJapanese female who presented with a transient ischemicattack. Steno-occlusion involving the distal part of the internalcarotid artery, proximal middle cerebral artery, and anterior cerebralartery was observed. Digital subtraction angiography demonstrateda beaded appearance in the cervical portion of the diseasedinternal carotid artery. Revascularization surgery was performed45 days after the onset. A new infarction appeared on theother side of the anterior cerebral artery territory 7 months afterthe first onset. Antiplatelets and vasodilators were administered,and no progression was observed during 18 months of follow-up.Genetic analysis did not show ring finger protein 213 (RNF213)-related moyamoya disease, and pathological examination revealedno characteristics of fibromuscular dysplasia.Conclusion The radiological and genetic features coincidedwith focal cerebral arteriopathy, which is a distinct entity fromfibromuscular dysplasia and RNF213-related moyamoyadisease.