PDF(Pubmed)
Abstract：
Moyamoya disease (MMD) is a rare, progressive cerebrovascular disorder that occurs when the major arteries supplying the brain become narrowed or obstructed. Because of this, small and delicate collateral vessels develop to compensate for the decrease in blood flow. Unfortunately, these vessels are insufficient to meet the brain\'s metabolic demands. Though initially described in Japan, MMD occurs in a variety of ethnicities around the world. The clinical manifestations of the disease can be devastating, with patients often presenting with symptoms of a stroke or transient ischemic attack. The long history of insults and chronic changes to the brain makes these individuals susceptible to alterations in their mental status. We describe a case of a young African American female with a history of sickle cell anemia (SCA) and undiagnosed MMD who presented to the emergency department with community-acquired pneumonia (CAP). In addition to her medical derangements, she also presented with paranoia, delusional guilt, and refusal to speak.
摘要：
烟雾病（MMD）是一种罕见的，当供应大脑的主要动脉变窄或阻塞时发生的进行性脑血管疾病。正因为如此,小而脆弱的侧支血管发育以补偿血流量的减少。不幸的是,这些血管不足以满足大脑的代谢需求。虽然最初在日本描述，MMD发生在世界各地的各种种族中。该疾病的临床表现可能是毁灭性的，患者通常表现为中风或短暂性脑缺血发作的症状。长期的侮辱和大脑的慢性变化使这些人容易受到精神状态的改变。我们描述了一例年轻的非洲裔美国女性，有镰状细胞性贫血（SCA）和未诊断的MMD病史，她因社区获得性肺炎（CAP）向急诊科就诊。除了她的医疗混乱，她还表现出偏执狂，妄想内疚,拒绝说话。
