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病例报告:伴有可逆性胼胝体压部病变的临床轻度脑炎/脑病:一例尸检病例

Case report: Clinically mild encephalitis/encephalopathy with a reversible splenial lesion: an autopsy case.

作者信息

Hayashi Maho, Ueda Midori, Hayashi Koji, Kawahara Ei, Azuma Shin-Ichiro, Suzuki Asuka, Nakaya Yuka, Asano Rei, Sato Mamiko, Miura Toyoaki, Hayashi Hiromi, Hayashi Kouji, Kobayashi Yasutaka

机构信息

Department of Diabetes and Endocrinology, Fukui General Hospital, Egami-cho, Fukui, Japan.

Department of Rehabilitation Medicine, Fukui General Hospital, Egami-cho, Fukui, Japan.

出版信息

Front Neurol. 2024 Jan 4;14:1322302. doi: 10.3389/fneur.2023.1322302. eCollection 2023.

Abstract

Clinically mild encephalitis/encephalopathy with a reversible splenial lesion is a clinicoradiological syndrome characterized by transient neuropsychiatric symptoms and hyperintensity of the splenium of the corpus callosum on diffusion-weighted MRI. Although intramyelinic edema and inflammatory cell infiltration can be predicted by MRI, the pathology of the splenium of the corpus callosum remains unknown. We encountered a case of clinically mild encephalitis/encephalopathy with a reversible splenial lesion and hypoglycemia in a patient who died of sepsis, and an autopsy was performed. The postmortem pathological findings included intramyelinic edema, myelin pallor, loss of fibrous astrocytes, microglial reactions, and minimal lymphocytic infiltration in the parenchyma. Based on these findings, transient demyelination following cytotoxic edema in the splenium of corpus callosum was strongly considered a pathogenesis of "clinically mild encephalitis/encephalopathy with a reversible splenial lesion" associated with hypoglycemia, and it could be generalized for the disease associated with the other causes. As cytotoxic edema could be the central pathology of the disease, the recently proposed term cytotoxic lesions of the corpus callosum may be applicable to this syndrome.

摘要

临床轻度脑炎/脑病伴可逆性胼胝体压部病变是一种临床放射学综合征,其特征为短暂的神经精神症状以及磁共振成像扩散加权像上胼胝体压部高信号。尽管磁共振成像可预测髓鞘内水肿和炎性细胞浸润,但胼胝体压部的病理学仍不清楚。我们遇到一例临床轻度脑炎/脑病伴可逆性胼胝体压部病变且伴有低血糖的患者,该患者死于败血症,并进行了尸检。尸检病理结果包括髓鞘内水肿、髓鞘苍白、纤维型星形胶质细胞丢失、小胶质细胞反应以及实质内少量淋巴细胞浸润。基于这些发现,强烈认为胼胝体压部细胞毒性水肿后的短暂性脱髓鞘是与低血糖相关的“临床轻度脑炎/脑病伴可逆性胼胝体压部病变”的发病机制,并且这可能适用于与其他病因相关的该疾病。由于细胞毒性水肿可能是该疾病的核心病理学改变,最近提出的胼胝体细胞毒性病变这一术语可能适用于此综合征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1978/10794512/f3e7e44ea4fa/fneur-14-1322302-g001.jpg

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