Annals of Clinical and Translational Neurology (May 2019)

Vertebral artery dissection associated with familial Mediterranean fever and Behçet's disease

  • Hidehiro Ishikawa,
  • Akihiro Shindo,
  • Yuichiro Ii,
  • Shoko Sakano,
  • Masaru Asahi,
  • Keita Matsuura,
  • Dai Kishida,
  • Maki Umino,
  • Masayuki Maeda,
  • Hidekazu Tomimoto

DOI
https://doi.org/10.1002/acn3.773
Journal volume & issue
Vol. 6, no. 5
pp. 974 – 978

Abstract

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Abstract Vertebral artery dissection and recurrent meningitis are rare complications in Behçet's disease. Behçet's disease may be associated with familial Mediterranean fever. Here, we describe a 52‐year‐old woman with severe headache who exhibited recurrent meningitis and vertebral artery dissection. Cerebrospinal fluid showed high levels of interleukin‐6. Magnetic resonance imaging revealed right vertebral artery dissection. The patient had three heterozygous mutations in the familial Mediterranean fever gene (MEFV) gene. She fulfilled criteria for diagnosis of Behçet's disease and familial Mediterranean fever. In conclusion, mutations of the MEFV gene may cause neuro‐inflammatory disorders and cerebrovascular disorders by reducing anti‐inflammatory activity of pyrin.