Anna Maria Lavezzi, "Lino Rossi" Research Centre for the study and prevention of unexpected perinatal death and SIDS, Department of Biomedical, Surgical and Dental Sciences, University of Milan, Via della Commenda 19, 20122 Milano, Italy, phone: +39-02-50320821, fax: +39-02-50320823, e-mail:
Folia Neuropathol. 2013;51(4):290-301. doi: 10.5114/fn.2013.39718.
The present study was aimed at supplementing our previous investigations on the morphological features of the Purkinje cells during the autonomic nervous system development, particularly in victims of sudden perinatal death (Sudden Intrauterine Unexplained Death Syndrome and Sudden Infant Death Syndrome), given their crucial role in determining connectivity patterns in the brain as well as in the control of autonomic functions. We highlighted in these pathologies, and precisely in 21 cases of sudden foetal death and 26 cases of sudden infant death, a high percentage of developmental defects of the Purkinje cells such as heterotopia, hypoplasia, hyperplasia, mitotic and/or shrunken features and abnormal neuronal nuclear antigen expression. These alterations can be interpreted as a result of a defective maturation and/or migration of Purkinje cells in foetal cerebellum, likely consequence of exposure to injuries, particularly to maternal cigarette smoke. Interestingly, we observed in sudden perinatal deaths an association with similar developmental defects of both the dentate and the inferior olivary nuclei. This suggests the existence of a Purkinje-Olivo-Dentate network playing a fundamental role in triggering a sudden death mechanism in perinatal life in the presence of specific risk factors.
本研究旨在补充我们之前关于自主神经系统发育过程中浦肯野细胞形态特征的研究，特别是在突然围产期死亡（突然宫内原因不明死亡综合征和婴儿猝死综合征）的受害者中，因为它们在确定大脑连接模式以及控制自主功能方面起着至关重要的作用。我们在这些病理学中发现，尤其是在 21 例胎儿死亡和 26 例婴儿猝死病例中，浦肯野细胞发育缺陷的比例很高，如异位、发育不良、增生、有丝分裂和/或萎缩特征以及异常神经元核抗原表达。这些改变可以解释为胎儿小脑浦肯野细胞成熟和/或迁移缺陷的结果，可能是由于暴露于损伤，特别是母体吸烟。有趣的是，我们在围产期突然死亡中观察到齿状核和下橄榄核的类似发育缺陷之间存在关联。这表明存在浦肯野-橄榄-齿状核网络，在存在特定危险因素的情况下，该网络在围产期生命中触发突然死亡机制中起着基础性作用。
