Marín-Padilla Miguel, Parisi Joseph E, Armstrong Dawna L, Sargent Steve K, Kaplan James A
Molecular Neuroscience Program, Mayo Clinic, Guggenheim 1521A, 200 First Ave. SW, Rochester, MN 55905, USA.
Acta Neuropathol. 2002 Apr;103(4):321-32. doi: 10.1007/s00401-001-0470-z. Epub 2001 Nov 21.
This study describes the developmental neuropathology of two infants who survived 7 and 9 years, respectively, an episode of violent shaking (shaken infant syndrome) early in their lives. The shaking injuries include cortical and subcortical contusions, hemorrhages, hypoxic/ischemic and axonal damage, and severe edema. The types, distribution, and resolution of these shaking injuries are detailed by sequential radiographic studies and by pathologic examination at postmortem. Despite their severity and extent, these injuries resolved in a relatively short period of time. By 6 months, the original injuries are repaired and the resultant encephaloclastic encephalopathies (e.g., multicystic encephalomalacia, porencephaly, generalized white matter attenuation, diffuse cortical atrophy, microgyria, ulegyria, and hydrocephalus ex vacuo) are well established. No appreciable pathologic differences are detected when radiographic findings at 6 months of age are compared to postmortem observations. On the other hand, undamaged and/or partially damaged cortical regions survive the original insult and undergo post-injury reorganization that transforms the residual cortex structural and presumably functional organization. Prominent features of this post-injury reorganization include progressive cortical dysplasia with cytoarchitectural disorganization, laminar obliteration, morphologic and functional (synaptic reorganization) transformation of some neurons, preservation of layer 1 intrinsic fibers and Cajal-Retzius cells, and the presence of large (hypertrophic) intrinsic neurons with intense neurofilament immunoreactivity. We propose that this progressive dysplastic process modifies the residual cortex structural and functional organization, influences the child's neurological and psychological maturation, and may play a significant role in the pathogenesis of ensuing neurological and/or psychological sequelae.
本研究描述了两名分别存活了7年和9年的婴儿的发育性神经病理学情况，他们在生命早期都经历过一次剧烈摇晃（婴儿摇晃综合征）。摇晃造成的损伤包括皮质和皮质下挫伤、出血、缺氧/缺血性损伤和轴突损伤，以及严重水肿。通过系列影像学研究和死后病理检查详细记录了这些摇晃损伤的类型、分布和恢复情况。尽管这些损伤严重且范围广泛，但在相对较短的时间内得到了恢复。到6个月时，原来的损伤已修复，由此产生的脑破坏性脑病（如多囊性脑软化、脑穿通畸形、弥漫性白质萎缩、弥漫性皮质萎缩、微小脑回、脑回发育不全和脑外积水）已形成。将6个月大时的影像学检查结果与死后观察结果进行比较，未发现明显的病理差异。另一方面，未受损和/或部分受损的皮质区域在最初的损伤中存活下来，并在损伤后进行重组，从而改变了残余皮质的结构以及可能的功能组织。损伤后重组的突出特征包括渐进性皮质发育异常伴细胞结构紊乱、分层消失、一些神经元的形态和功能（突触重组）转变、第1层固有纤维和Cajal-Retzius细胞的保留，以及存在具有强烈神经丝免疫反应性的大型（肥大）固有神经元。我们认为，这种渐进性发育异常过程改变了残余皮质的结构和功能组织，影响儿童的神经和心理成熟，并可能在随后的神经和/或心理后遗症的发病机制中起重要作用。
