Department of Neurology, Université Paris Descartes, Pôle neuroscience, services de Neurologie et de Neuroradiologie, Hôpital Sainte-Anne, Paris, France.
Int J Stroke. 2010 Aug;5(4):296-305. doi: 10.1111/j.1747-4949.2010.00445.x.
Fibromuscular dysplasia is an uncommon, segmental, nonatherosclerotic arterial disease of unknown aetiology. The disease primarily affects women and involves intermediate-sized arteries in many areas of the body, including cervical and intracranial arteries. Although often asymptomatic, fibromuscular dysplasia can also be associated with spontaneous dissection, severe stenosis that compromises the distal circulation, or intracranial aneurysm, and is therefore responsible for cerebral ischaemia or subarachnoid haemorrhage. Fibromuscular dysplasia affects middle and distal portions of the internal carotid and vertebral arteries, and occasionally, intracranial arteries. Several pathological and angiographic patterns exist. The most frequent pathological type is medial fibromuscular dysplasia, which is associated with the 'string of beads' angiographic pattern. Unifocal lesions are less common and can be associated with several pathological subtypes. The pathophysiology of the disease is widely unknown. Fibromuscular dysplasia may in fact result from various causes and reflect a non-specific response to different insults. The poor knowledge of the natural history and the lack of randomised trials that compared the different treatment options do not allow any satisfactory judgement to be made regarding the need for or the efficacy of any treatment.
纤维肌性发育不良是一种病因不明的非粥样硬化性、节段性、中动脉疾病。该疾病主要影响女性，涉及身体多个部位的中等大小动脉，包括颈内动脉和颅内动脉。虽然纤维肌性发育不良通常无症状，但也可能与自发性夹层、严重狭窄导致的远端循环受损或颅内动脉瘤有关，因此会导致脑缺血或蛛网膜下腔出血。纤维肌性发育不良影响颈内动脉和椎动脉的中部和远端，偶尔也会影响颅内动脉。存在几种病理和血管造影模式。最常见的病理类型是中层纤维肌性发育不良，与“串珠”血管造影模式相关。单发病变较少见，可能与几种病理亚型相关。该疾病的病理生理学广泛未知。纤维肌性发育不良实际上可能由多种原因引起，反映了对不同损伤的非特异性反应。对自然病史的了解不足，以及缺乏比较不同治疗选择的随机试验，使得对任何治疗的必要性或疗效都无法做出任何令人满意的判断。
