Objective:Individuals with sickle cell disease (SCD) are at increased risk for developing impairment across cognitive domains, although the most common deficits are thought to be related to processing speed and executive functions. One of the most common ways of evaluating cognitive functioning is through the administration of intellectual tests. While lower overall intellectual functioning in individuals with SCD compared to healthy controls has been found, the specific pattern of strengths and weaknesses across indices is not well known. Anecdotally, it has been observed at our clinic that individuals with SCD are more likely to show relative or significant weaknesses in visuospatial abilities, but this has not been formally investigated. Further, based on the extant research, individuals with SCD would likely demonstrate lower working memory and processing speed indices, but, as far as we are aware, this has not been investigated either. The purpose of the present study is to examine the intellectual profiles, including areas of relative and significant strengths and weaknesses, of children and adolescents with SCD.Participants and Methods:Participants are children and adolescents (age 6-16) with SCD who were referred for a neuropsychological evaluation at Montefiore Medical Center’s Neuropsychological Assessment Service from 2015 to 2022. These participants (N=54) were identified through a thorough review of patients seen through this service and were administered the Wechsler Intelligence Scale for Children, Fifth Edition (WISC-V; Wechsler, 2014). Mean scores were calculated for WISC-V indices. In addition, differences were calculated between WISC-V indices (e.g., VCI-VSI, etc.), and a discrepancy analysis was conducted comparing the base rates of these differences in the present sample to the WISC-V standardization sample.Results:In our sample, the mean total FSIQ of our sample was 85 (SD=14.5). The following mean scores were obtained across indices: VCI, SS=90 (SD=14.5); VSI, SS=86.5 (SD=14.9); FRI, SS=90 (15.5); WMI, SS=89 (SD=15.6); and PSI, SS=82 (SD=17.4). Many of the index score discrepancy base rates were similar to the standardization sample. However, our sample had greater discrepancies between several indices compared to the standardization sample. In particular, the following base rate discrepancies between index scores emerged as being different in our sample compared to the standardization sample: VCI>VSI and VCI>PSI. Notably, a 30+ point difference VCI>VSI was found in 6% of our sample (compared to 1.6% of the standardization sample) and a 30+ point difference between VCI>PSI was found in 12% of our sample (compared to 4.6% of the standardization sample). In addition, a 10+ point difference found between VCI>PSI was found in 50% of our sample (compared to 29% of standardization sample).Conclusions:In our sample, FSIQ and index scores fell approximately 0.5-1.33 SD below the standardization sample means, with the lowest index scores being PSI and VSI. Consistent with the literature, the PSI (but not WMI) emerged as an area most discrepant to other indices (particularly VCI). In line with our observations, the VSI emerged as an area of relative difficulty as compared to the VCI. These results suggest that, in addition to processing speed, visuospatial/constructional ability is an area that warrants consideration in the assessment of individuals with SCD.

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83 儿童镰状细胞病人群的 WISC-V 概况

目的：尽管最常见的缺陷被认为与处理速度和执行功能有关，但镰状细胞病 (SCD) 患者出现跨认知领域障碍的风险增加。评估认知功能的最常见方法之一是进行智力测试。虽然与健康对照组相比，SCD 患者的整体智力功能较低，但各指数的优势和劣势的具体模式尚不清楚。有趣的是，我们诊所观察到患有 SCD 的人更有可能在视觉空间能力方面表现出相对或显着的弱点，但这尚未得到正式调查。此外，根据现有研究，患有 SCD 的人可能会表现出较低的工作记忆和处理速度指数，但据我们所知，这也尚未得到调查。本研究的目的是检查患有 SCD 的儿童和青少年的智力概况，包括相对和显着的优势和劣势领域。 参与者和方法：参与者是被转诊的患有 SCD 的儿童和青少年（6-16 岁） 2015 年至 2022 年，蒙蒂菲奥里医疗中心的神经心理评估服务进行了神经心理学评估。这些参与者 (N=54) 通过对通过该服务看到的患者进行彻底审查来确定，并接受韦克斯勒儿童智力量表第五版 (WISC- V；韦克斯勒，2014）。计算 WISC-V 指数的平均分。此外，还计算了 WISC-V 指数（例如 VCI-VSI 等）之间的差异，并进行差异分析，将本样本中这些差异的基准率与 WISC-V 标准化样本进行比较。 结果：我们的样本，我们样本的平均总 FSIQ 为 85 (SD=14.5)。跨指数获得以下平均分数：VCI，SS=90（SD=14.5）；VSI，SS=86.5（SD=14.9）；FRI，SS=90 (15.5)；WMI，SS=89​​（SD=15.6）；和PSI，SS=82（SD=17.4）。许多指数得分差异基准率与标准化样本相似。然而，与标准化样本相比，我们的样本在几个指标之间存在更大的差异。特别是，与标准化样本相比，我们的样本中指数得分之间的以下基本比率差异有所不同：VCI>VSI 和 VCI>PSI。值得注意的是，在 6% 的样本中发现了 30+ 点的 VCI>VSI 差异（相比之下，标准化样本为 1.6%），在 12% 的样本中发现了 VCI>PSI 30+ 点的差异（相比之下，标准化样本为 4.6%）。标准化样本的%）。此外，在我们的 50% 样本中发现 VCI>PSI 之间存在 10+ 分的差异（标准化样本为 29%）。 结论：在我们的样本中，FSIQ 和指数得分比标准化值下降了约 0.5-1.33 SD样本意味着，指数得分最低的是 PSI 和 VSI。与文献一致，PSI（但不是 WMI）是与其他指数（特别是 VCI）差异最大的领域。根据我们的观察，与 VCI 相比，VSI 是一个相对困难的领域。这些结果表明，除了处理速度之外，视觉空间/构建能力也是评估 SCD 患者时值得考虑的一个领域。