Moyamoya disease among young patients: Its aggressive clinical course and the role of active surgical treatment
Moyamoya disease among young patients: Its aggressive clinical course and the role of active surgical treatment
复制标题
DOI：
10.1227/01.neu.0000114140.41509.14
复制
发表时间：
2004-04-01
期刊：
影响因子：
4.8
通讯作者：
Wang, KC
中科院分区：
文献类型：
--
作者：
Kim, SK;Seol, HJ;Wang, KC
目的：青年烟雾病(MMD)患者的预后比老年患者差。本研究的目的是探讨年轻MMD患者的临床特点和治疗结果。方法：204例MMD患者，根据手术时的年龄分为三组，A组23例，年龄6岁。对每组患者的临床表现模式和随后的术前或手术相关梗塞的发生情况进行评估。结果：首次发病时，A组(87%)和B组(58%)的脑梗塞发生率明显高于C组(46%)。随后的术前脑梗塞发生率A组(39%)明显高于B组(6%)或C组(0.8%)。出现症状和随后的术前脑梗塞之间的中位间隔为3个月(范围1-14个月)。与手术相关的梗死率在三组之间没有显著差异。A组的临床结果优良率(58%)明显低于B组(84%)或C组(86%)，尽管两组术后血流动力学改善率相似。A组临床转归差的主要原因是术前脑梗塞。结论：青年MMD病情进展快，临床转归差。这些发现表明了年轻MMD患者早期手术的必要性；然而，实际的益处应该通过额外的对照研究和长期随访监测来验证。
OBJECTIVE: The prognosis for moyamoya disease (MMD) among young patients is known to be worse than that among older patients. The aim of this study was to investigate the clinical features and treatment outcomes of young patients with MMD.METHODS: A total of 204 patients with MMD who underwent encephaloduroarteriosynangiosis, with or without bifrontal encephalogaleosynangiosis, were categorized into three groups according to their ages at the time of surgery, i.e., Group A (n = 23, 6 yr of age). For each group, patterns of presentation and the occurrence of subsequent preoperative or surgery-related infarctions were assessed. Clinical outcomes and postoperative hemodynamic status were analyzed.RESULTS: At initial presentation, infarctions were significantly more frequent in Group A (87%) and Group B (58%) than in Group C (46%). Subsequent preoperative infarctions occurred significantly more frequently in Group A (39%) than in Group B (6%) or Group C (0.8%). The median interval between the onset of symptoms and a subsequent preoperative infarction was 3 months (range, 1-14 mo). No significant difference in the rates of surgery-related infarctions among the three groups was observed. The rate of favourable clinical outcomes was significantly lower in Group A (58%) than in Group B (84%) or Group C (86%), although the rates of postoperative hemodynamic improvements were similar among the groups. The poor clinical outcomes for Group A were caused mainly by preoperative infarctions.CONCLUSION: Young-age MMD demonstrates rapid disease progression and results in poor clinical outcomes. These findings indicate the necessity of early surgery for young patients with MMD; however, the actual benefits should be verified with additional controlled studies, with long-term follow-up monitoring.