Ortiz-Fandiño J, Terré-Boliart R, Orient-López F, Guevara-Espinosa D, Ramón-Rona S, Bernabeu-Guitart M
Unidad de Daño Cerebral, Institut Guttmann, Hospital de Rehabilitació, Badalona, España.
Rev Neurol. 2004;38(1):34-7.
Fibromuscular dysplasia is an infrequent angiopathy that occurs more often in young women. It consists in a series of heterogeneous histological changes that finally lead to the narrowing of arteries. Clinical manifestations depend on the vessel involved, but high blood pressure (renal artery involvement) and stroke (carotid artery involvement) are the most common. Little has been published about this process in recent years.
A 30-year-old female with extensive infarction of the right middle cerebral artery caused by obstruction of the internal carotid artery. Arteriographical examination revealed the dissection of the four supra-aortic trunks together with alterations to the renal artery. The patient's age and the involvement of the four cerebral arteries with irregular stenoses led us to consider a fibromuscular dysplasia (FMD) as the first diagnostic option. A conservative attitude was adopted towards the vascular lesions and therapy was carried out with a view to rehabilitating the neurological deficits.
Brain FMD can be asymptomatic and is often diagnosed by chance. The usual clinical presentation is a stenosis-related ischemic stroke, arterial obstruction or arterio-arterial thromboembolism. Arteriography or magnetic resonance angiography are useful for diagnosis; the involvement of different supra-aortic vessels is characteristic. Despite the good results shown by percutaneous transluminal angioplasty, there is still a need for prospective studies that determine the optimal treatment for this pathology.
纤维肌发育不良是一种罕见的血管病,在年轻女性中更为常见。它表现为一系列异质性组织学改变,最终导致动脉狭窄。临床表现取决于受累血管,但高血压(肾动脉受累)和中风(颈动脉受累)最为常见。近年来关于这个过程的报道很少。
一名30岁女性,因颈内动脉阻塞导致右侧大脑中动脉广泛梗死。血管造影检查显示主动脉弓上四大分支夹层形成以及肾动脉改变。患者的年龄以及四条脑动脉出现不规则狭窄使我们将纤维肌发育不良(FMD)作为首要诊断选项。对血管病变采取保守态度,并针对神经功能缺损进行康复治疗。
脑部FMD可能无症状,常偶然被诊断出来。常见的临床表现是与狭窄相关的缺血性中风、动脉阻塞或动脉-动脉血栓栓塞。血管造影或磁共振血管造影对诊断有帮助;不同主动脉弓上血管受累是其特征。尽管经皮腔内血管成形术显示出良好效果,但仍需要前瞻性研究来确定针对这种病症的最佳治疗方法。