Wasserman A L, Wilimas J A, Fairclough D L, Mulhern R K, Wang W
Department of Psychiatry and Psychology, St. Jude Children's Research Hospital, Memphis, Tennessee.
Am J Pediatr Hematol Oncol. 1991 Spring;13(1):14-20. doi: 10.1097/00043426-199121000-00004.
Forty-three children and adolescents (8-16 years old) with sickle cell disease (SCD) and no overt neurologic dysfunction were evaluated with the Wechsler Intelligence Scale for Children-Revised (WISC-R), the Wide Range Achievement Test (WRAT), and an age-appropriate version of the Luria-Nebraska Neuropsychological Battery (LNNB). Thirty similar-aged siblings were controls. WISC-R performance and full-scale IQs were lower (p less than 0.05) for patients than sibling controls. No significant differences were seen on the WRAT. Patients with SCD missed more school days than controls (p less than 0.001) but did not differ from controls in academic performance or the proportion in special education. On the LNNB, scores for patients and controls 13 years and older did not differ. However, young patients had significantly elevated (abnormal) scores compared with controls on the following scales: expressive speech, writing, reading, arithmetic, memory, and pathognomonic. The possibility that subtle neuropsychological deficits in younger patients with SCD reflect either subclinical strokes or a predisposition for strokes merits further investigation in a large multi-institution study.
对43名患有镰状细胞病（SCD）且无明显神经功能障碍的儿童和青少年（8至16岁），使用韦氏儿童智力量表修订版（WISC-R）、广泛成就测验（WRAT）以及适合其年龄的鲁利亚-内布拉斯加神经心理成套测验（LNNB）进行评估。30名年龄相仿的兄弟姐妹作为对照。患者的WISC-R表现和全量表智商低于兄弟姐妹对照组（p<0.05）。WRAT方面未观察到显著差异。SCD患者缺课天数多于对照组（p<0.001），但在学业成绩或接受特殊教育的比例上与对照组无差异。在LNNB上，13岁及以上患者和对照组的得分无差异。然而，年轻患者在以下量表上的得分与对照组相比显著升高（异常）：表达性言语、书写、阅读、算术、记忆和确诊量表。SCD年轻患者存在细微神经心理缺陷，这可能反映亚临床中风或中风易感性，这一可能性值得在一项大型多机构研究中进一步探究。
