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先天性呼吸暂停伴延髓和橄榄体发育不全:一项计算机重建的病理学研究

Congenital apnea with medullary and olivary hypoplasia: a pathologic study with computer reconstructions.

作者信息

Kinney H C, Filiano J J, Brazy J E, Burger P C, Sidman R L

机构信息

Division of Neuroscience, Children's Hospital, Boston, MA 02115.

出版信息

Clin Neuropathol. 1989 Jul-Aug;8(4):163-73.

PMID:2776382
Abstract

We report a three-month-old infant with congenital central apnea who was ventilator-dependent throughout his brief life. At autopsy the most significant findings were localized to the medulla and included severe tegmental necrosis involving respiratory-related sites and olivary hypoplasia. Golgi impregnations of the cerebellum demonstrated several Purkinje cells with changes consistent with experimental olivary ablation. The majority of Purkinje cells, however, were normal; this observation suggests that in an early and subtotal olivary lesion, the incomplete complement of olivary neurons maintains sufficient collateral axonal branches to compensate for decreased cell number. Computer graphics enabled us to dissect the components of the complex medullary pathology and examine them individually and in selected combinations in three dimensions. Computer reconstruction aided the identification and dating of a malformative lesion (first trimester) from a later (second/third trimester), superimposed destructive lesion. This report suggests that the synthesis of complex morphologic data in human neuropathology into meaningful three-dimensional visual displays by computer reconstruction facilitates their comprehension. Computer reconstruction is especially valuable in the elucidation of 3-D topographic relationships in functionally and architecturally complex regions such as the brain stem.

摘要

我们报告了一名3个月大的先天性中枢性呼吸暂停婴儿,在其短暂的生命中一直依赖呼吸机。尸检时,最显著的发现局限于延髓,包括涉及呼吸相关部位的严重被盖坏死和橄榄体发育不全。小脑的高尔基染色显示几个浦肯野细胞有与实验性橄榄体切除一致的变化。然而,大多数浦肯野细胞是正常的;这一观察结果表明,在早期和部分橄榄体病变中,橄榄体神经元的不完全补充保持了足够的侧支轴突分支,以补偿细胞数量的减少。计算机图形学使我们能够剖析复杂的延髓病理成分,并在三维空间中单独和以选定的组合方式对其进行检查。计算机重建有助于从后期(妊娠中期/晚期)叠加的破坏性病变中识别和确定畸形病变(妊娠早期)的时间。本报告表明,通过计算机重建将人类神经病理学中复杂的形态学数据合成为有意义的三维视觉显示有助于对其的理解。计算机重建在阐明脑干等功能和结构复杂区域的三维地形关系方面特别有价值。

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