Chen Chih-Ping, Lin Shuan-Pei, Tsai Fuu-Jen, Chern Schu-Rern, Lee Chen-Chi, Wang Wayseen
Department of Obstetrics and Gynecology, Mackay Memorial Hospital, 92, Section 2, Chung-Shan North Road, Taipei 104, Taiwan.
Eur J Med Genet. 2008 Jul-Aug;51(4):368-72. doi: 10.1016/j.ejmg.2008.02.011. Epub 2008 Mar 29.
We report a male patient with speech and language disorder, cleft palate, epilepsy, a ventricular septal defect, mental retardation and developmental delay. Characteristic facial features include low-set ears, a beak-like nose, a prominent nasal bridge, a long philtrum, a narrow forehead, a long face, a pointed chin and dental position abnormalities. Array-comparative genomic hybridization (CGH) analysis demonstrated the presence of a 5.6-Mb deletion in 15q14 (chromosome 15: 3,18,33,000-3,74,77,000bp). The present case provides the evidence that 15q14 deletion outside the region encompassing the CHRNA7 gene can cause generalized epilepsy, and a locus in 15q14 is associated with speech and language disorder.
我们报告了一名患有言语和语言障碍、腭裂、癫痫、室间隔缺损、智力障碍和发育迟缓的男性患者。其特征性面部特征包括低位耳、喙状鼻、鼻梁突出、人中长、额头窄、脸长、下巴尖以及牙齿位置异常。阵列比较基因组杂交(CGH)分析显示15q14(染色体15:31833000 - 37477000bp)存在一个5.6Mb的缺失。本病例提供了证据表明,包含CHRNA7基因区域之外的15q14缺失可导致全身性癫痫,且15q14中的一个基因座与言语和语言障碍相关。