Concentric structure of thalamic lesions in acute necrotizing encephalopathy

Concentric structure of thalamic lesions in acute necrotizing encephalopathy
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DOI:
10.1007/s00234-002-0773-3
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发表时间:
2002-06-01
期刊:
影响因子:
2.8
通讯作者:
Takashima, S
Takashima, S
中科院分区:
医学3区
文献类型:
--
作者:
Mizuguchi, M;Hayashi, M;Takashima, S

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儿童急性坏死性脑病(acute necrotizing encephalopathy of children,ANE)是一种以双侧丘脑、壳核和大脑白色物质为主要表现的对称性多发性脑损害,CT和MRI常表现为同心圆状结构。为了揭示这一发现的病理基础,对三例ANE死亡病例的CT和尸检结果进行了比较。头颅CT显示1例患者在脑病发作后3.5天死亡,但在30小时内死亡的其他2例患者中未显示丘脑病变的同心圆结构。神经病理学检查显示,所有病例的血管和实质病理改变呈层状。血管的过度渗透性和由此产生的血管源性水肿随着大脑表面深度的增加而变得更加突出。1例CT表现为病灶深部血管周围严重出血,中央高密度。
Acute necrotizing encephalopathy of childhood (ANE) is characterized by multiple, symmetrical brain lesions affecting the bilateral thalami, putamina and cerebral white matter, which often show a concentric structure on CT and MRI. To reveal the pathological substrate of this finding, comparison was made between CT and necropsy findings of three fatal cases of ANE. Cranial CT demonstrated a concentric structure of the thalamocerebral lesions in one patient who died 3.5 days after the onset of encephalopathy, but not in the other two patients who died within 30 h. Neuropathological examination of postmortem brains revealed laminar changes of vascular and parenchymal pathology in all the cases. Excessive permeability of blood vessels and resultant vasogenic edema became more prominent with increasing depth from the cerebral surface. The deep portion of the lesions showed severe perivascular hemorrhage, accounting for the central high density on the CT images of one patient.