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

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Abstract

Background

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.

Conclusion

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

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Authors and Affiliations

  1. Department of Neurosurgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, 466-8550, Japan

    Yoshio Araki & Toshihiko Wakabayashi

  2. Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto, Japan

    Yasushi Takagi, Yohei Mineharu & Susumu Miyamoto

  3. Department of Health and Environmental Sciences, Kyoto University Graduate School of Medicine, Kyoto, Japan

    Hatasu Kobayashi

Authors
  1. Yoshio Araki
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  2. Yasushi Takagi
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  3. Yohei Mineharu
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  4. Hatasu Kobayashi
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  5. Susumu Miyamoto
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  6. Toshihiko Wakabayashi
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Corresponding author

Correspondence to Yoshio Araki.

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

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The authors declare that they have no conflict of interest.

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

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Araki, Y., Takagi, Y., Mineharu, Y. et al. Rapid contralateral progression of focal cerebral arteriopathy distinguished from RNF213-related moyamoya disease and fibromuscular dysplasia. Childs Nerv Syst 33, 1405–1409 (2017). https://doi.org/10.1007/s00381-017-3451-9

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  • DOI: https://doi.org/10.1007/s00381-017-3451-9

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