Adams R J
Department of Neurology, Medical College of Georgia, 1467 Harper St, HB-2060, Augusta, GA 30912, USA.
Arch Neurol. 2001 Apr;58(4):565-8. doi: 10.1001/archneur.58.4.565.
While the problem of stroke in the patients with sickle cell disease (SCD) has been known for more than 75 years, adequate preventive and treatment strategies are just now being tested. Recent data on prevalence and incidence have been obtained from the Cooperative Study of Sickle Cell Disease of more than 4000 patients with SCD observed in 23 US clinical centers over a 10-year period.1 The overall age-specific incidence of first stroke in SCD (homozygous sickle cell anemia) is low (0.13%) at ages younger than 24 months, increasing to just over 1% at ages 2 to 5 years, with only a slight decrement to 0.79% at ages 6 to 9 years. The risk of brain infarction declines until a second peak is seen at ages older than 50 years, when the incidence again increases to nearly 1.3%. Although intracranial hemorrhage does occur in young children with SCD, the risk is low compared with older children and adults. The Cooperative Study of Sickle Cell Disease reported risk factors for infarction to be prior transient ischemic attack, low steady-state hemoglobin values, and rate and recency of episodes of acute chest syndrome, as well as elevated systolic blood pressure. Risk factors for intracranial hemorrhage included low steady-state hemoglobin values and a high leukocyte count. The burden of cerebrovascular disease is even higher if subclinical magnetic resonance imaging (MRI) lesions, presumed to be ischemic, are included. The prevalence of such lesions is more than 22% in patients with SCD, and most of these patients have not reported symptoms, although specialized neuropsychological testing shows lower scores in children with silent lesions on MRI scans. Patients with a history of clinical stroke typically have infarcts in the cortex and deep white matter, whereas silent infarcts tend to be more limited to deep white matter. Common infarction patterns are characterized by wedge-shaped lesions of large-vessel territories; border zone infarctions, particularly of the middle and cerebral artery watershed region; and small punctate lesions of the deep white matter. Fat embolism to the brain and venous thromboses are encountered rarely.
虽然镰状细胞病（SCD）患者的中风问题已被知晓超过75年，但足够的预防和治疗策略目前才刚刚开始进行测试。关于患病率和发病率的最新数据来自镰状细胞病合作研究，该研究在10年期间对美国23个临床中心观察的4000多名SCD患者进行了研究。1 SCD（纯合子镰状细胞贫血）首次中风的总体年龄特异性发病率在24个月以下时较低（0.13%），在2至5岁时增至略高于1%，在6至9岁时仅略有下降至0.79%。脑梗死风险下降，直到50岁以上出现第二个高峰，此时发病率再次增至近1.3%。虽然SCD幼儿中确实会发生颅内出血，但与大龄儿童和成人相比，风险较低。镰状细胞病合作研究报告称，梗死的危险因素包括既往短暂性脑缺血发作、低稳态血红蛋白值、急性胸部综合征发作的频率和近期情况，以及收缩压升高。颅内出血的危险因素包括低稳态血红蛋白值和高白细胞计数。如果将假定为缺血性的亚临床磁共振成像（MRI）病变包括在内，脑血管疾病的负担会更高。此类病变在SCD患者中的患病率超过22%，而且这些患者大多未报告有症状，尽管专门的神经心理学测试显示，MRI扫描有隐匿性病变的儿童得分较低。有临床中风病史的患者通常在皮质和深部白质有梗死，而隐匿性梗死往往更局限于深部白质。常见的梗死模式的特征是大血管区域的楔形病变；边缘带梗死，特别是大脑中动脉和大脑分水岭区域；以及深部白质的小斑点状病变。脑脂肪栓塞和静脉血栓形成很少见。
