Graham J F, Matoba A
Neurosurgery Service, Hawaii Permanente Medical Group, Honolulu 96819, USA.
Clin Neurol Neurosurg. 1997 Oct;99 Suppl 2:S31-5. doi: 10.1016/s0303-8467(97)00037-1.
Moyamoya disease (MMD) is a rare entity in the US with a few retrospective series and sporadic cases reported in the US and North America. Although it is known that MMD exists in all races, there is a predilection for people of Asian origin. Because of the relatively high percentage of Asians living in Hawaii, it was hypothesized that the estimated prevalence of MMD would be higher in Hawaii than the remaining US. All practicing neurologists, neurosurgeons, neuroradiologists and major hospitals in Hawaii were surveyed for MMD patients treated during the past 10 years. Medical records and angiograms (when available) were reviewed and the diagnostic guidelines for MMD from the Ministry of Health and Welfare of Japan applied. There were 53 records reviewed in 42 patients; 21 fulfilled the criteria for definite Moyamoya disease which were the focus of this study. In Hawaii, the prevalence of MMD was higher in patients of Japanese descent compared to Caucasians (P = 0.012) and higher than in the remaining US (P < 0.001). Non-Japanese Asians and Pacific Islanders had a higher incidence of MMD than Caucasians that was not statistically significant. There was no difference in MMD among Japanese living in Hawaii or Japan. Males had an equal percentage of hemorrhage and infarcts; females tended to have a higher incidence of ischemic events rather than hemorrhage. Age and sex distribution of our series were similar to larger reported MMD studies. Our results suggest that: (1) Moyamoya disease in Hawaii has a higher incidence and prevalence than the rest of the US, largely due to the larger percentage of Asians, particularly Japanese, living in Hawaii; and (2) genetic rather than environmental factors may explain the increased MMD in Hawaii.
烟雾病（MMD）在美国是一种罕见疾病，美国和北美仅有少数回顾性系列研究及散发病例报道。尽管已知烟雾病在所有种族中均有存在，但亚洲裔人群更易患病。由于居住在夏威夷的亚洲人比例相对较高，因此推测夏威夷烟雾病的估计患病率会高于美国其他地区。对夏威夷所有执业神经科医生、神经外科医生、神经放射科医生以及各大医院进行了调查，以了解过去10年中治疗的烟雾病患者情况。查阅了病历和血管造影照片（如有），并应用了日本厚生省的烟雾病诊断指南。共查阅了42例患者的53份记录；其中21例符合确诊烟雾病的标准，这也是本研究的重点。在夏威夷，日裔患者的烟雾病患病率高于白种人（P = 0.012），且高于美国其他地区（P < 0.001）。非日裔亚洲人和太平洋岛民的烟雾病发病率高于白种人，但无统计学意义。居住在夏威夷或日本的日本人之间烟雾病患病率无差异。男性出血和梗死的比例相同；女性缺血性事件的发生率往往高于出血性事件。我们系列研究的年龄和性别分布与已报道的较大规模烟雾病研究相似。我们的结果表明：（1）夏威夷的烟雾病发病率和患病率高于美国其他地区，这主要归因于居住在夏威夷的亚洲人，尤其是日本人的比例较高；（2）遗传因素而非环境因素可能解释了夏威夷烟雾病患病率的增加。
