Open AccessVertebral artery dissection associated with familial Mediterranean fever and Behçet's disease
Author(s) -
Ishikawa Hidehiro,
Shindo Akihiro,
Ii Yuichiro,
Sakano Shoko,
Asahi Masaru,
Matsuura Keita,
Kishida Dai,
Umino Maki,
Maeda Masayuki,
Tomimoto Hidekazu
Publication year - 2019
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.773
Subject(s) - familial mediterranean fever , medicine , mefv , pyrin domain , vertebral artery dissection , meningitis , dissection (medical) , vertebral artery , behcet's disease , disease , surgery , pathology , gene mutation , immunology , gene , inflammation , mutation , inflammasome , genetics , biology
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.