Marcinkevicius Egidijus, Liutkus Danius, Gvazdaitis Antanas
Clinic of Neurosurgery, Kaunas University of Medicine, Kaunas, Lithuania.
Medicina (Kaunas). 2006;42(2):130-6.
Moyamoya disease was first described in Japan and represents characteristic appearance on cerebral angiography an abnormal network ("puff of smoke") of collaterals around stenotic arteries. This disease is characterized by progressive intracranial vascular obliterations of the circle of Willis, resulting in successive ischemic or hemorrhagic events. Moyamoya disease primarily occurs among orient people (Japanese, Koreans, Caucasians) and is very rare in Lithuania.
To evaluate long-term effectiveness of cerebral revascularization in order to prevent cerebral ischemic and hemorrhagic events in patients with moyamoya disease.
Since 1995, 14 patients with moyamoya disease have been treated at the Clinic of Neurosurgery of Kaunas University of Medicine. All patients underwent neurological examination, computed tomography or/and magnetic resonance imaging, transcranial Doppler sonography, single photon emission computed tomography and four vessels cerebral angiography investigations. The diagnosis of moyamoya disease was confirmed by cerebral angiography. Cerebral ischemia was observed in 7 cases and cerebral hemorrhage was detected in the other 7 moyamoya disease patients. Neurological disability was evaluated using the Scandinavian Stroke Scale, and performance in the active daily life using the Barthel Index. The degree of handicap was assessed with the Rankin Scale. Different surgical procedures (superficial temporal artery to middle cerebral artery anastomosis, encephalosynangiosis, bifrontal free omental flap) were applied for cerebral revascularization of moyamoya disease patients.
The diagnosis of moyamoya disease was confirmed by cerebral angiography in all our patients. Ten patients were selected for surgical brain revascularization. The main criterion for selection of patients for surgery was hypoperfusion of the brain on single photon emission computed tomography. During follow-up period (mean follow-up period was 36 months) there were no rebleeding or ischemic events in both surgical groups.
Cerebral angiography is the main diagnostic procedure which confirms the diagnosis of moyamoya disease. Cerebral hypoperfusion on single photon emission computed tomography is the main criterion for selection of patients for cerebral revascularization. Extra-intracranial anastomosis is an effective procedure for preventing both ischemic and hemorrhagic events in moyamoya patients.
烟雾病最早在日本被描述，其特征是在脑血管造影上呈现出狭窄动脉周围异常的侧支循环网络（“烟雾”）。这种疾病的特点是 Willis 环进行性颅内血管闭塞，导致相继发生缺血性或出血性事件。烟雾病主要发生在东方人群（日本人、韩国人、高加索人）中，在立陶宛非常罕见。
评估脑血运重建的长期效果，以预防烟雾病患者发生脑缺血和出血事件。
自 1995 年以来，考纳斯医科大学神经外科诊所治疗了 14 例烟雾病患者。所有患者均接受了神经学检查、计算机断层扫描或/和磁共振成像、经颅多普勒超声检查、单光子发射计算机断层扫描和四血管脑血管造影检查。烟雾病的诊断通过脑血管造影得以证实。7 例患者观察到脑缺血，另外 7 例烟雾病患者检测到脑出血。使用斯堪的纳维亚卒中量表评估神经功能障碍，使用 Barthel 指数评估日常生活活动能力。用 Rankin 量表评估残疾程度。对烟雾病患者采用不同的外科手术方法（颞浅动脉-大脑中动脉吻合术、脑-硬脑膜-动脉-血管融合术、双额游离大网膜瓣）进行脑血运重建。
我们所有患者的烟雾病诊断均通过脑血管造影得以证实。10 例患者被选进行脑血运重建手术。选择患者进行手术的主要标准是单光子发射计算机断层扫描显示脑灌注不足。在随访期间（平均随访期为 36 个月），两个手术组均未发生再出血或缺血事件。
脑血管造影是确诊烟雾病的主要诊断方法。单光子发射计算机断层扫描显示脑灌注不足是选择患者进行脑血运重建的主要标准。颅外吻合术是预防烟雾病患者缺血性和出血性事件的有效方法。
