单侧烟雾病的临床特征

摘要/Abstract

摘要：

【摘要】目的探讨单侧烟雾病的临床特征及预后。方法回顾性分析2003年1月～2010年1月在中国人民解放军第307医院神经外科就诊的单侧烟雾病患者，对患者的发病情况、影像学检查结果、治疗结果及预后情况进行分析。结果 78例患者中男52例，女26例，成人52例，儿童26例，其中2例患儿合并家族性烟雾病。首发症状中24例患者表现为脑梗死，短暂性脑缺血发作（transient ischemic attack，TIA）18例，头痛17例，9例表现为脑出血（8例为成人），其他表现共10例。46例患者为右侧病变，32例患者为左侧病变。73例患者接受手术，69例患者接受随访。术后6例出现TIA，2例出现新发脑梗死，9例脑出血患者未再发生脑出血。32例患者接受血管造影复查，29例显示颅内外已建立良好的侧支循环。2例患者术后对侧血管出现狭窄或闭塞，按复查血管病变是否存在对侧进展，分为进展组和非进展组，其中进展比例在本组为6.3%（2/32）。在进展组儿童及伴有家族史所占比例均为100%，与非进展组这两项比较P值分别为0.043和0.002，差异具有显著性（P<0.05）。结论具有家族史的儿童患者可能是对侧进展的预测因素；手术后能建立明确的侧支循环，改善临床症状。

文章导读： 通过回顾性分析探讨了单侧烟雾病的特征及演变，丰富并细化了烟雾病研究的内容。

关键词: 烟雾病; 单侧; 脑缺血; 脑出血; 演变

Abstract:

【Abstract】 Objective To investigate the clinical features and prognosis of unilateral moyamoya disease. Methods A retrospective study was conducted to analyze the incidence, imaging data, treatment and prognosis of patients admitted to 307 Hospital of Chinese People's Liberation Army with unilateral moyamoya disease from January 2003 to January 2010. Results In 78 patients, 52 were male and 26 were female, 52 were adult, 26 were pediatric patients and 2 with familial history. As to the initial symptoms, 24 cases manifested cerebral infarction, 18 cases showed transient ischemic attack (TIA), 9 cases (8 adult patients) suffered hemorrhage, 2 cases showed syncope, 2 cases suffered dizziness and the other 6 cases were asymptomatic. Among the 78 patients, 46 cases were located in the right hemispheres and 32 cases were in the left sides. All the patients received surgery and 69 cases were followed-up. During the follow-up, 6 cases complained of TIA, 2 cases came up with new cerebral infarction. The 9 hemorrhage-onset cases suffered no recurrent bleeding episodes. Thirty-two cases conducted postoperative angiography for re-examination and 29 cases showed a great deal of compensatory blood supply from extracranial to intracranial arteries. Two cases showed progression in the contralateral hemispheres during follow-up and the progression rate in this group accounted for 6.3% (2/32). The proportion of pediatric patient and accompanied family history are all one hundred percent in the progression group. Statistical difference can be seen in age and family history between progression and non-progression group and the P value are 0.043 and 0.002 respectively. Conclusion The familial pediatric cases may be the predictive factor for the contralateral progression of unilateral moyamoya disease. Surgical treatment can establish effective collateral circulation and improve the clinical symptoms.

Key words: Moyamoya disease, unilateral; Cerebral ischemia; Cerebral hemorrhage; Progression

李德生，咸鹏，杨伟中，刘鹏，宗睿，暴向阳，张正善，段炼. 单侧烟雾病的临床特征[J]. 中国卒中杂志, 2013, 8(07): 511-516.

LI De-Sheng, XIAN Peng, YANG Wei-Zhong, LIU Peng, ZONG Rui, BAO Xiang-Yang, ZHANG Zheng-Shan, DUAN Lian.. Clinical Features of Unilateral Moyamoya Disease[J]. Chinese Journal of Stroke, 2013, 8(07): 511-516.

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