Increased incidence of a midline brain anomaly in patients with nonsyndromic clefts of the lip and/or palate
Increased incidence of a midline brain anomaly in patients with nonsyndromic clefts of the lip and/or palate
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DOI：
10.1111/j.1552-6569.2001.tb00072.x
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发表时间：
2001-10-01
影响因子：
2.4
通讯作者：
Canady, J
中科院分区：
文献类型：
--
作者：
Nopoulos, P;Berg, S;Canady, J
背景和目的。非综合征性唇腭裂（CLP）是一种发育性颅面畸形，通常与认知功能障碍有关。本研究旨在评价CLP患者是否存在特定的中线脑异常（透明隔腔扩大[CSP]），该异常在其他发育综合征中已被证明与认知功能不良相关。方法.使用磁共振成像对49名CLIP成年男性和75名健康对照者进行脑成像。使用连续冠状图像测量CSP的大小。结果CLP组中大CSP的发生率为8%（4/49），显著高于对照组。在这4例受试者中的2例中，异常是隔叶完全不融合，称为CSP和海绵窦联合。此外，有一个显着的负相关的智商和CSP在CLP患者，而不存在于对照组。也就是说，在患有CLP的个体中，CSP越大，智商越低。结论.患有CLP的成年男性的CSP扩大的患病率增加。此外，这种异常与认知缺陷直接相关。这项研究提供了进一步的证据，表明面部的发育和大脑的发育密切相关，颅面发育的缺陷很可能与大脑发育的缺陷有关。
Background and Purpose. Nonsyndromic clefts of the lip and palate (CLP) are developmental craniofacial abnormalities that are often associated with cognitive dysfunction. This study was designed to evaluate, in patients with CLP, the presence of a specific midline brain anomaly (enlarged cavum septi pellucidi [CSP]) that has been shown in other developmental syndromes to be related to poor cognitive function. Methods. Brain images were obtained using magnetic resonance imaging on 49 adult men with CLIP and 75 healthy controls. Size of CSP was measured using consecutive coronal images. Results. The incidence of large CSP in the CLP group was 8% (4 of 49), significantly higher than that found in the control group. In 2 of these 4 subjects, the anomaly was complete nonfusion of the septal leaflets, known as a combined CSP and cavurn vergae. Furthermore, there was a significant inverse relationship of IQ and CSP in CLP patients that was not present in controls. That is, in individuals with CLP, the larger the CSP, the lower the IQ. Conclusions. Adult men with CLP have an increased prevalence of enlarged CSP. Moreover, this anomaly is directly related to cognitive deficits. This study provides further evidence that the development of the face and the development of the brain are intimately related and that defects in craniofacial development are most likely associated with defects in brain development.