Gubanova M V, Kalashnikova L A, Dobrynina L A
Research Center of Neurology, Moscow, Russia.
Zh Nevrol Psikhiatr Im S S Korsakova. 2020;120(11):116-123. doi: 10.17116/jnevro2020120111116.
The authors present the current data on the classification, epidemiology, etiology, neurological manifestations, prognosis, diagnosis, and treatment of patients with fibromuscular dysplasia (FMD). The review is based on the selection of publications by searching PubMed for keywords from the first sources until March 2019. FMD is a segmental non-atherosclerotic and non-inflammatory disease of large- and medium-caliber arteries leading to their stenosis. The disease occurs mostly in women (90%), and manifests itself in the 5th decade of life. In the cerebrovascular form of FMD, the extracranial internal carotid artery and the vertebral artery are usually affected. Diagnosis is based on the identification of alternation of narrowing and dilation of arteries using angiography (the string of beads sign (multifocal form)). Neurological manifestations include headache, tinnitus, and ischemic stroke, usually due to the dissection or stenosis, rarely, intracerebral or subarachnoid hemorrhages. The prognosis in most cases is favorable, relapses of strokes are rare. Treatment includes antiplatelet agents, if they are ineffective to prevent recurrence of ischemic stroke, endovascular treatment is carried out. Approaches to the treatment of intracranial aneurysms do not differ from those in patients without FMD.
作者介绍了纤维肌性发育不良（FMD）患者在分类、流行病学、病因、神经学表现、预后、诊断及治疗方面的现有数据。本综述基于通过在PubMed中检索关键词，从最初的文献来源至2019年3月筛选出版物。FMD是一种累及大中口径动脉导致其狭窄的节段性非动脉粥样硬化、非炎性疾病。该病多见于女性（90%），发病高峰在50岁左右。在FMD的脑血管型中，通常累及颈外段颈内动脉和椎动脉。诊断基于血管造影术发现动脉狭窄与扩张交替出现（串珠征（多灶型））。神经学表现包括头痛、耳鸣及缺血性卒中，通常由动脉夹层或狭窄所致，很少出现脑出血或蛛网膜下腔出血。多数情况下预后良好，卒中复发罕见。治疗包括使用抗血小板药物，若预防缺血性卒中复发无效，则进行血管内治疗。颅内动脉瘤的治疗方法与无FMD患者无异。
