Yoshida Shunichi, Eguchi Kaoru, Onodera Kazunari, Suzuki Kengo, Fujishiro Kenichiro, Riku Shigeo
Department of Neurology, Social Insurance Chukyo Hospital.
Rinsho Shinkeigaku. 2013;53(6):439-45. doi: 10.5692/clinicalneurol.53.439.
Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory arterial disease of unknown etiology. We report a 26-year-old woman who presented with transient ischemic attack (TIA) due to bilateral internal carotid artery (ICA) occlusion and severe basilar artery stenosis, as FMD was diagnosed by a biopsy specimen of right ICA. Imaging investigations included magnetic resonance angiography and catheter angiogram without characteristic "string of beads" pattern, before reaching a definitive diagnosis by pathologist. Anti-platelet therapy and bypass surgery of superficial temporal artery-middle cerebral artery revealed no more clinical symptoms. This case of intra- and extra-cranial FMD gives a consideration of such rare disease in the differential diagnosis of TIA or stroke in healthy young patients. The literature of FMD is reviewed including pathological findings.
纤维肌发育不良(FMD)是一种病因不明的非动脉粥样硬化性、非炎性动脉疾病。我们报告一名26岁女性,因双侧颈内动脉(ICA)闭塞和严重基底动脉狭窄出现短暂性脑缺血发作(TIA),通过右侧ICA活检标本确诊为FMD。在病理学家做出明确诊断之前,影像学检查包括磁共振血管造影和导管血管造影,未发现特征性的“串珠样”表现。抗血小板治疗和颞浅动脉-大脑中动脉搭桥手术使临床症状消失。这例颅内和颅外FMD病例提示,在健康年轻患者TIA或卒中的鉴别诊断中应考虑到这种罕见疾病。本文回顾了FMD的相关文献,包括病理表现。