Neuropsychological Evaluation Of Patients

在过去的五年中，这项工作的主要重点是比较癫痫患者（全身性和局灶性）与他们的一级亲属的神经心理学测试概况。在早期的一项研究中，12个家庭的先证者被诊断为儿童失神癫痫（CAE），结果表明，母亲，但不是患者的父亲共享轻度形式的一些行为障碍，在先证者中看到的（Levav，1991）。这在持续注意力的测试中是明显的（持续表现测试，CPT; Rosvold等人，1956年）。这一结果与先前关于该疾病遗传的研究一致。在本研究中，我们评估了另外60个家庭中的先证者及其健康的一级亲属，以及健康对照对在神经心理学测试中的表现。我们评估了与青少年肌阵挛性癫痫（JME），颞叶癫痫（TLE）和儿童失神癫痫（CAE）的先证者亲属对。注意力，记忆力和解决问题的行为进行了研究，以评估可能的认知特征的家族聚集。到目前为止的分析表明，有一个强大的家族聚集的特征与控制冲动和警惕性的持续注意力的任务，尤其是在JME家庭。这在TLE家族中未见，在CAE家族中仅在一定程度上。这项研究发表在Journal of Clinical & Experimental Neuropsychology。我们目前正在与有兴趣定位与JME相关的癫痫基因的遗传学家合作-最有可能包括染色体6p上的位置21.3-并且他们在识别JME的表型方面的工作与我们自己的神经心理学数据很好地结合起来。在与约翰霍普金斯大学的同事合作进行的另一项研究中，我们发现骨铅含量（通过K X射线技术测量）与持续注意力持续表现测试（CPT）得分之间存在高度显著的相关性。这些研究结果是基于对市中心年轻人的研究，最近（2001年8月）在加州州弗朗西斯科举行的美国心理学协会大会的海报会议上发表的。此外，在约翰霍普金斯大学孙惠芳（Huey-Fang Sun，2000）的博士论文中，发现教师评定的课堂攻击与CPT分数显著相关。那些在CPT（和其他注意力测试）中得分较低的孩子比那些在CPT中表现较好的孩子更具攻击性。这种情况持续了至少五个年级（2-6年级）。

During the past five years, the main thrust of this work has involved a comparison of the neuropsychological test profiles of patients with seizure disorders (generalized as well as focal) with those of their first-degree relatives. In an earlier study of 12 families with probands diagnosed with childhood absence epilepsy (CAE), the results indicated that the mothers, but not the fathers of the patients share in mild form some of the behavioral impairment seen in the probands (Levav, 1991). This was evident in tests of sustained attention (the Continuous Performance Test, CPT; Rosvold et al., 1956). This result is consistent with prior research on the inheritance of the disorder. In the present study we assessed probands and their healthy first- degree relatives in an additional 60 families, as well as healthy control pairs, in their performance on neuropsychological tests. We assessed proband-relative pairs with Juvenile Myoclonic Epilepsy (JME), Temporal Lobe Epilepsy (TLE) and Childhood Absence Epilepsy (CAE). Attention, memory and problem-solving behavior were studied in order to evaluate possible familial aggregation of cognitive traits. The analysis thus far indicates that there is a strong familial aggregation of traits associated with control of impulsivity and vigilance in the sustained attention tasks, seen especially in JME families. This was not seen in the TLE families, and only to a modest extent in the CAE families. This work has been published in the Journal of Clinical & Experimental Neuropsychology. We are currently collaborating with geneticists who are interested in localizing the epilepsy gene(s) associated with JME--most likely including location 21.3 on chromosome 6p--and whose work on identifying the phenotype of JME dovetails well with our own neuropsychological data. In another study conducted in collaboration with colleagues at Johns Hopkins, we have found highly significant correlations between assays of bone lead, as measured by the K X-ray technique, and scores on the Continuous Performance Test of sustained attention (CPT). These findings were based on studies of an inner-city population of young adults and were recently presented (August 2001) at a poster session at the American Psychological Association convention in San Francisco, California. In addition, in a doctoral dissertation at Johns Hopkins by Huey-Fang Sun (2000), it was found that teacher-rated classroom aggression correlated significantly with scores on the CPT. Those children who scored poorly on the CPT (and on other attention tests) were more aggressive than children who performed better on the CPT. This persisted over at least five grades (2-6).