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一例罕见的小头畸形病例。

An interesting case of extreme microcephaly.

作者信息

Horácková L

机构信息

Department of Anatomy, Medical Faculty, J. E. Purkynĕ University, Brno, Czechoslovakia.

出版信息

Anat Anz. 1989;169(3):179-92.

PMID:2610372
Abstract

The collections of the Museum in the Department of Anatomy, Faculty of Medicine, J. E. Purkynĕ University in Brno, include a skull of about 25 to 30-year-old individual with extreme microcephaly that has been so far described rarely in the morphological literature. The skull capacity is 355 cm3, which is only 26.1% of the skull capacity in a "normal" individual. While the facial skeleton is reduced only by 10-15% if compared with the norm, the cerebral part is striking by its extraordinarily small dimensions (smaller by 30-40% in comparison with the norm), particularly in the area of the frontal bone squama. The size of the skull is characterized best by the values of the basic metric measurements: maximal length of the skull 122 mm (norm: 172 mm), maximal breadth of the skull 94 mm (norm: 140 mm), height of the skull 96 mm (norm: 130 mm), circumference of the skull through the glabella 351 mm (norm: 510 mm). Foramen occipitale magnum is shifted strikingly to the dorsal direction. The cause of the microcephaly described cannot be explained explicitly just on the basis of the findings in the skull. For finding the actual cause a number of other data should be known. Authors' hypothesis that the problem is primary microcephaly has been supported even by roentgenological finding.

摘要

布尔诺的扬·埃万杰利斯塔·普尔基涅大学医学院解剖学系博物馆的藏品中,有一个约25至30岁个体的颅骨,其患有极端小头畸形,这在形态学文献中迄今鲜有描述。颅骨容量为355立方厘米,仅为“正常”个体颅骨容量的26.1%。与正常情况相比,面部骨骼仅缩小了10 - 15%,而脑部部分则因其尺寸异常小(与正常情况相比小30 - 40%)而引人注目,尤其是额骨鳞部区域。颅骨大小最能通过基本测量值来表征:颅骨最大长度122毫米(正常:172毫米),颅骨最大宽度94毫米(正常:140毫米),颅骨高度96毫米(正常:130毫米),通过眉间的颅骨周长351毫米(正常:510毫米)。枕骨大孔明显向背侧移位。仅根据颅骨的发现无法明确解释所描述的小头畸形的原因。为了找出实际原因,还需要了解许多其他数据。作者关于该问题是原发性小头畸形的假设甚至得到了放射学检查结果的支持。

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