An autopsy case with non‐herpetic acute limbic encephalitis (NHALE)
An autopsy case with non‐herpetic acute limbic encephalitis (NHALE)
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非疱疹性急性边缘脑炎（NHALE）尸检一例
DOI：
10.1111/j.1440-1789.2008.00872.x
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发表时间：
2008
期刊：
影响因子：
2.3
通讯作者：
S. Kuzuhara
中科院分区：
文献类型：
--
作者：
T. Maki;Y. Kokubo;S. Nishida;Hideo Suzuki;S. Kuzuhara
非疱疹性急性边缘脑炎（NHALE）是一种病因不明的炎症性疾病，限制影响边缘系统。NHALE的神经病理改变只在一篇报道中被描述过。一位53岁的日本妇女出现高热、意识障碍和难治性全身抽搐。她的症状对包括皮质类固醇、无环鸟苷、γ -球蛋白和抗惊厥药在内的积极治疗具有耐药性，并急剧恶化，导致发病后6天出现癫痫持续状态。脑部核磁共振显示炎性改变局限于边缘系统。经实验室检查排除疱疹性脑炎。虽然不能完全排除副肿瘤边缘脑炎，但胸部CT及腹部超声检查均未发现肿瘤病变。患者在发病后34天死于多器官衰竭。组织学上，有神经元丢失和严重的胶质细胞增生，肥厚的星形胶质细胞增加，并且在局限于海马和杏仁核的几条小血管周围有血管周围的结扎。免疫组化结果显示，巨噬细胞和活化的星形胶质细胞更广泛地分布于边缘系统，即内囊、丘脑、尾状核和黑质。单纯疱疹病毒I(1)和II(2)免疫组化阴性。本病例的神经病理表现与先前报道相似。NHALE在神经病理学上被认为是一种独特的疾病实体，并且存在暴发性NHALE。
Non‐herpetic acute limbic encephalitis (NHALE) is a cause‐unknown, inflammatory disease entity that affects the limbic system restrictedly. Neuropathological changes of NHALE have been described in only one report. A 53‐year‐old Japanese woman developed high fever, disturbance of consciousness and intractable generalized convulsions. Her symptoms were resistant to aggressive therapy including corticosteroid, acyclovir, γ‐globulin and anticonvulsants, and progressed acutely, leading to status epilepticus at 6 days after the onset. Brain MRI revealed inflammatory changes restricted to the limbic system. Herpes encephalitis was excluded by laboratory tests. Although paraneoplastic limbic encephalitis could not be excluded completely, no tumor lesions were detected on chest CT and abdominal ultrasonography. The patient died of multiple organ failure at 34 days after the onset of the disease. Histologically, there were neuronal loss and severe gliosis with an increase in hypertrophic astrocytes and with perivascular cuffings around several small blood vessels restricted to the hippocampus and the amygdala. Immunohistochemically, macrophages and activated astrocytes were distributed more widely over the limbic system, namely internal capsule, thalamus, caudate nucleus and substantia nigra. Herpes simplex virus I(1)and II(2) were negative immunohistochemically. The neuropathological findings of this case were similar to that of the previous report. NHALE is supposed to be a distinct disease entity neuropathologically and the fulminant form of NHALE exists.