Child's Nervous System

, Volume 33, Issue 8, pp 1405–1409 | Cite as

Rapid contralateral progression of focal cerebral arteriopathy distinguished from RNF213-related moyamoya disease and fibromuscular dysplasia

  • Yoshio ArakiEmail author
  • Yasushi Takagi
  • Yohei Mineharu
  • Hatasu Kobayashi
  • Susumu Miyamoto
  • Toshihiko Wakabayashi
Case Report



Focal cerebral arteriopathy includes unifocal or multifocal lesions that are unilateral or bilateral. Large- and/or medium-sized vessels are involved and can be visualized on angiography.

Case report

We report a case of cerebral infarction in a 9-year-old Japanese female who presented with a transient ischemic attack. Steno-occlusion involving the distal part of the internal carotid artery, proximal middle cerebral artery, and anterior cerebral artery was observed. Digital subtraction angiography demonstrated a beaded appearance in the cervical portion of the diseased internal carotid artery. Revascularization surgery was performed 45 days after the onset. A new infarction appeared on the other side of the anterior cerebral artery territory 7 months after the 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 revealed no characteristics of fibromuscular dysplasia.


The radiological and genetic features coincided with focal cerebral arteriopathy, which is a distinct entity from fibromuscular dysplasia and RNF213-related moyamoya disease.


Focal cerebral arteriopathy RNF213 Moyamoya disease Fibromuscular dysplasia 


Compliance with ethical standards


No sources of financial or material support were gained for conducting this study.

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

Informed consent was obtained from the participant included in this case report.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Department of NeurosurgeryNagoya University Graduate School of MedicineNagoyaJapan
  2. 2.Department of NeurosurgeryKyoto University Graduate School of MedicineKyotoJapan
  3. 3.Department of Health and Environmental SciencesKyoto University Graduate School of MedicineKyotoJapan

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