Sudden death in toddlers associated with developmental abnormalities of the hippocampus: A report of five cases
Sudden death in toddlers associated with developmental abnormalities of the hippocampus: A report of five cases
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DOI：
10.2350/06-08-0144.1
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发表时间：
2007-05-01
影响因子：
1.9
通讯作者：
Krous, Henry F.
中科院分区：
文献类型：
--
作者：
Kinney, Hannah C.;Armstrong, Dawna L.;Krous, Henry F.
儿童期不明原因猝死(SUDC)是指年龄大于1岁的儿童在回顾临床病史、死亡情况、尸检和适当的辅助测试后仍无法解释的猝死。我们在此报告了5例幼儿SUDC病例，我们认为这些病例定义了一个与尸检中的海马区异常有关的新实体。所有蹒跚学步的孩子都在夜间意外死亡，显然是在睡觉时。在死亡前48小时内，有2名幼儿发烧，3名有轻微上呼吸道感染，3名有轻微头部创伤。2例(40%)有热性惊厥病史，2例(40%)有家族热性惊厥病史。海马区的表现包括外部不对称和2个或更多微发育异常特征。与尸检确定死因的39名幼儿(对照组)相比，这5例患儿的颞叶中某些微发育异常的发生率显著增加(P<；0.01)。我们建议这5个病例定义了一个潜在的SUDC亚型，其猝死是由睡眠时异常海马区出现的无症状癫痫引起的，并导致心肺骤停。诱发因素可能是发烧、感染和/或轻微的头部创伤。提示的危险因素是热性惊厥病史和/或热性惊厥家族史。需要进一步的研究来证实这些初步发现，并确定幼儿猝死、海马体异常和发热性癫痫之间的可能联系。
Sudden unexplained death in childhood (SUDC) is the sudden death of a child older than I year of age that remains unexplained after review of the clinical history, circumstances of death, and autopsy with appropriate ancillary testing. We report here 5 cases of SUDC in toddlers that we believe define a new entity associated with hippocampal anomalies at autopsy. All of the toddlers died unexpectedly during the night, apparently during sleep. Within 48 hours before death, 2 toddlers had fever, 3 had a minor upper respiratory tract infection, and 3 experienced minor head trauma. There was a history of febrile seizures in 2 (40%) and a family history of febrile seizures in 2 (40%). Hippocampal findings included external asymmetry and 2 or more microdysgenetic features. The incidence of certain microdysgenetic features was substantially increased in the temporal lobes of these 5 cases compared with the temporal lobes of 39 (control) toddlers with the causes of death established at autopsy (P < 0.01). We propose that these 5 cases define a potential subset of SUDC whose sudden death is caused by an unwitnessed seizure arising during sleep in the anomalous hippocampus and producing cardiopulmonary arrest. Precipitating factors may be fever, infection, and/or minor head trauma. Suggested risk factors are a history of febrile seizures and/or a family history of febrile seizures. Future studies are needed to confirm these initial findings and to define the putative links between sudden death, hippocampal anomalies, and febrile seizures in toddlers.