Miralles F, Reñé R, Rubio F, Pons L, Vila M J, Peres J
Servicio de Neurología, Hospital de Bellvitge-Prínceps d'Espanya, Ciutat Sanitària de Bellvitge, L'Hospitalet de Llobregat, Barcelona.
Neurologia. 1992 Oct;7(8):230-3.
According to the diagnostic criteria currently in use, the so-called "unilateral" forms of the moyamoya disease, or rather those in which the disorders of the disease itself--such as occlusion of the internal supraclinoid carotid artery or its terminal branches and development of abnormal collateral circulation in the region of the basal ganglia--are only found in one hemisphere and should be considered as "probable" forms of the disease with a much lower incidence than the bilateral or "defined" forms of the same. One patient with a primary intraventricular hemorrhage (PIVH) in whom an occlusion of the right internal carotid artery was angiographically demonstrated and in whom collateral type moyamoya circulation was found as was the presence of an aneurysm in the right coroid territory is presented. The association of PIVH, unilateral moyamoya disease and aneurysm is infrequent in the literature, with the origin of the bleeding, in some cases, having been attributed to rupture of the aneurysm. Since angiographic control was not available in the patient presented it cannot be excluded that the aneurysm was really a pseudoaneurysm therefore being a consequence, rather than a cause, of the arterial rupture. Thus one of the other mechanisms proposed must be invoked to explain the pathogenesis of the PIVH: rupture of a perforating artery or of a microaneurysm located in the subependimary periventricular region. The treatment recommended for these case of PIVH associated to aneurysm is chirurgical if persistence is demonstrated in successive arteriographies.
根据目前使用的诊断标准，烟雾病的所谓“单侧”形式，或者更确切地说，那些疾病本身的病变——如床突上段颈内动脉或其终末分支闭塞以及基底节区异常侧支循环形成——仅在一个半球出现，应被视为该病的“可能”形式，其发病率远低于双侧或“明确”形式。本文报告一例原发性脑室内出血（PIVH）患者，血管造影显示右侧颈内动脉闭塞，发现侧支型烟雾病循环，且右侧脉络膜区域存在动脉瘤。PIVH、单侧烟雾病和动脉瘤的关联在文献中并不常见，在某些情况下，出血的起源归因于动脉瘤破裂。由于所报告的患者未进行血管造影对照，不能排除该动脉瘤实际上是假性动脉瘤，因此是动脉破裂的结果而非原因。因此，必须援引提出的其他机制之一来解释PIVH的发病机制：位于室管膜下脑室周围区域的穿支动脉或微动脉瘤破裂。对于这些与动脉瘤相关的PIVH病例，如果在连续血管造影中显示持续存在，推荐的治疗方法是手术治疗。
