Changes in globus pallidus with (Pre) term kernicterus
Changes in globus pallidus with (Pre) term kernicterus
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DOI：
10.1542/peds.112.6.1256
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发表时间：
2003-12-01
期刊：
影响因子：
8
通讯作者：
Barkovich, J
中科院分区：
文献类型：
--
作者：
Govaert, P;Lequin, M;Barkovich, J
Objective.我们报告了5例核黄疸早产儿和3例足月儿苍白球的系列磁共振（MR）和超声表现，并描述了极低出生体重早产儿的临床背景。在此信息的基础上，我们建议的诊断和预防手段。我们回顾了5名早产儿和3名足月儿疑似胆红素相关脑损伤的病历、MR和超声图像。其中包括患有严重听力损失、四肢瘫痪张力过高和T2加权MR成像（MRI）苍白球异常高信号的早产儿。在第150天死亡的1例婴儿中，在新生儿期确诊。其他人作为门诊病人被挑选出来，在12或22个月的校正年龄进行扫描。我们纳入了三例足月核黄疸的病例，以比较新生儿期和婴儿早期的系列MRI：它们是由葡萄糖-6-磷酸脱氢酶缺乏、尿脓毒症和脱水加果糖1 - 6二磷酸酶缺乏引起的。5例胎龄25 ~ 29周的早产儿血清总胆红素（TSB）水平低于通常建议的换血阈值。混合性酸中毒存在于3名婴儿周围的TSB峰。在没有置换药物的情况下，胆红素/白蛋白摩尔比全部> 0.5。所有人都未能通过床边听力筛查，并且在听觉脑反应测试中有严重的听力损失。1例患儿苍白球呈对称性均匀强回声，为警戒特征。苍白球在T1加权像上呈高信号。其他婴儿由于运动障碍性四肢瘫痪和听力损失而出现严重的发育迟缓。在12或22个月的校正年龄时，苍白球在T1加权像上正常，但在T2加权像上高信号。2例婴儿在冠状位液体衰减反转恢复MRI中记录到丘脑底受累。在新生儿期有典型临床表现的足月儿的MR表现与早产儿相似，但靶向超声检查未发现苍白球损伤。新生儿MRI表现为苍白球T1高信号和轻度T2高信号。血清白蛋白水平低的酸性极低出生体重早产儿发生MR证实的苍白球损伤和听力损失，面临“可接受的”TSB水平。系列MRI记录了新生儿晚期苍白球从急性主要T1高信号到永久性T2高信号的转变。丘脑底而非丘脑受累有助于与缺血性或代谢性疾病鉴别。新生儿时，这些婴儿僵硬，有严重的呼吸暂停，然后在婴儿期发展为高渗性四肢瘫痪。
Objective. We report serial magnetic resonance (MR) and sonographic behavior of globus pallidus in 5 preterm and 3 term infants with kernicterus and describe the clinical context in very low birth weight preterm infants. On the basis of this information, we suggest means of diagnosis and prevention.Methods. Charts and MR and ultrasound images of 5 preterm infants and 3 term infants with suspected bilirubin-associated brain damage were reviewed. Included were preterm infants with severe hearing loss, quadriplegic hypertonia, and abnormal hypersignal of globus pallidus on T2-weighted MR imaging (MRI). In 1 infant who died on day 150, the diagnosis was confirmed during the neonatal period. The others were picked up as outpatients and scanned at 12 or 22 months' corrected age. Three instances of term kernicterus were included for comparison of serial MRI in the neonatal period and early infancy: they were caused by glucose-6-phosphate dehydrogenase deficiency, urosepsis, and dehydration plus fructose 1 - 6 biphosphatase deficiency.Results. Five preterm infants of 25 to 29 weeks' gestational age presented with total serum bilirubin (TSB) levels below exchange transfusion thresholds commonly advised. Mixed acidosis was present in 3 infants around the TSB peak. The bilirubin/ albumin molar ratio was > 0.5 in all, in the absence of displacing drugs. All failed to pass bedside hearing screen tests and had severe hearing loss on auditory brain response testing. Symmetrical homogeneous hyperechogenicity of globus pallidus was the alerting feature in 1 infant. Globus pallidus was hyperintense on T1-weighted MR images in this child. The other infants presented with severe developmental delay as a result of dyskinetic quadriplegia and hearing loss. Globus pallidus was normal on T1- but hyperintense on T2-weighted MR images at 12 or 22 months' corrected age. Subthalamic involvement was documented in coronal fluid attenuated inversion recovery MRI in 2 infants. The term infants with classical clinical presentation in the neonatal period had MR behavior similar to the preterms, but pallidal injury was not recognized with targeted sonographic examination. Their neonatal MR images demonstrated pallidal T1 hyperintensity and mild T2 hyperintensity.Conclusion. Acidotic very low birth weight preterm infants with low serum albumin levels develop MR-confirmed pallidal injury and hearing loss facing "accepted" TSB levels. Serial MRI documents a shift from acute mainly T1 hypersignal to permanent T2 hypersignal in globus pallidus within the late neonatal period. Subthalamic and not thalamic involvement helps to differentiate from ischemic or metabolic disorder. As newborns, these infants are rigid and have severe apnea, before developing hypertonic quadriplegia in infancy.