Wei Yi-Chia, Liu Chi-Hung, Chang Ting-Yu, Chin Shy-Chyi, Chang Chien-Hung, Huang Kuo-Lun, Chang Yeu-Jhy, Peng Tsung-I, Lee Tsong-Hai
Department of Neurology, Keelung Medical Center, Chang Gung Memorial Hospital, Keelung, Taiwan.
Department of Neurology, Linkou Medical Center, Chang Gung Memorial Hospital and College of Medicine, Chang Gung University, Taoyuan, Taiwan.
J Stroke Cerebrovasc Dis. 2014 Jul;23(6):1344-50. doi: 10.1016/j.jstrokecerebrovasdis.2013.11.008. Epub 2014 Jan 25.
Several coexisting diseases have been reported in patients with moyamoya vasculopathy (MMV), but studies of quasi-moyamoya disease (quasi-MMD) are rare. This study aims to investigate the frequency of known coexisting diseases in patients with quasi-MMD and to compare quasi-MMD with moyamoya disease (MMD).
Between 2000 and 2011, we retrospectively screened patients with International Classification of Diseases, Ninth Revision, code of 4375 (MMD) in the Health Information System of our hospital. The vascular images of each patient were confirmed by 2 neurologists and 1 neuroradiologist based on the diagnostic criteria of Japan Ministry of Health and Welfare. We excluded the patients with missing images and erroneous diagnosis. Demographics, coexisting diseases, laboratory data, treatment, and recurrent strokes were recorded. The eligible patients were divided into quasi-MMD and MMD groups according to the presence or absence of coexisting diseases.
MMV was found in 90 patients including 37 (41.1%) quasi-MMD and 53 (58.9%) MMD. Atherosclerosis (32.4%) and thyroid disease (29.7%) were the leading coexisting diseases in quasi-MMD. Patients with MMD became symptomatic in a bimodal age distribution, whereas patients with quasi-MMD became symptomatic in a single-peak distribution. The prognosis of recurrent strokes was similar between quasi-MMD and MMD based on Kaplan-Meier analysis.
A bimodal distribution of onset age was noted in MMD, whereas a single-peak distribution was found in quasi-MMD. Coexisting diseases were usually underevaluated but were more common than expected in patients with MMV. Atherosclerosis and thyroid diseases were the leading coexisting diseases in different preferential age.
烟雾病血管病变（MMV）患者中已报告有几种并存疾病，但关于类烟雾病（quasi-MMD）的研究很少。本研究旨在调查quasi-MMD患者中已知并存疾病的发生率，并将quasi-MMD与烟雾病（MMD）进行比较。
2000年至2011年期间，我们在我院健康信息系统中回顾性筛查了国际疾病分类第九版编码为4375（MMD）的患者。每位患者的血管图像由2名神经科医生和1名神经放射科医生根据日本厚生省的诊断标准进行确认。我们排除了图像缺失和诊断错误的患者。记录人口统计学、并存疾病、实验室数据、治疗和复发性中风情况。根据是否存在并存疾病，将符合条件的患者分为quasi-MMD组和MMD组。
在90例患者中发现了MMV，其中37例（41.1%）为quasi-MMD，53例（58.9%）为MMD。动脉粥样硬化（32.4%）和甲状腺疾病（29.7%）是quasi-MMD中主要的并存疾病。MMD患者症状出现呈双峰年龄分布，而quasi-MMD患者症状出现呈单峰分布。根据Kaplan-Meier分析，quasi-MMD和MMD复发性中风的预后相似。
MMD发病年龄呈双峰分布，而quasi-MMD呈单峰分布。并存疾病通常未得到充分评估，但在MMV患者中比预期更常见。动脉粥样硬化和甲状腺疾病是不同偏好年龄中主要的并存疾病。
