Enlarged Sylvian fissures in infants with interstitial deletion of chromosome 22q11
Enlarged Sylvian fissures in infants with interstitial deletion of chromosome 22q11
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DOI：
10.1002/(sici)1096-8628(19970919)74:5
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发表时间：
1997-09-19
期刊：
影响因子：
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通讯作者：
Zackai, E
中科院分区：
文献类型：
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作者：
Bingham, PM;Zimmerman, RA;Zackai, E
两名患有染色体22 q11缺失综合征的婴儿在头颅MRI上发现对称性脑裂扩大。我们在神经影像学研究中比较了17名del 22 q11受试者与年龄匹配的疾病对照组的大脑侧裂的大小。平均前眼间距被用作侧裂扩大的指标。对称性扩大的大脑外侧裂存在于10的17名受试者与del 22 q11。年龄发病模式，以及在2例患者的后续扫描，表明在这些患者的鳃盖区的延迟增长。与右侧相比，del 22 q11受试者的左侧外侧裂始终不成比例地扩大。这一观察结果表明，缺失区域中的一个基因（或多个基因）以不同的方式影响左右侧裂周皮层的发育。鳃盖的异常发育可以解释22 q11缺失综合征患者中常见的一些神经发育特征。(C)1997 Wiley-Liss，Inc.
Two infants with chromosome 22q11 deletion syndrome were noted to have symmetrically enlarged Sylvian fissures on cranial MRI. We compared the size of the Sylvian fissures in neuroimaging studies from 17 other subjects with del 22q11 to age-matched disease controls. The mean anterior interopercular distance was used as an index of Sylvian fissure enlargement. Symmetric enlargement of the Sylvian fissures was present in 10 of 17 subjects with del 22q11. The age-incidence pattern, as well as follow-up scans in 2 patients, suggests delayed growth of the opercular region in these patients. Subjects with del 22q11 consistently had disproportionate enlargement of the left Sylvian fissure compared to the right. This observation suggests that a gene (or genes) in the deleted region affects the development of the left and right perisylvian cortex in different ways. Abnormal development of the operculum may explain some of the neurodevelopmental features that are common among individuals with 22q11 deletion syndrome. (C) 1997 Wiley-Liss, Inc.