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在自身免疫性肝炎类固醇治疗期间出现显著脑室周围白质病变的神经结节病

Neurosarcoidosis Presenting with Prominent Periventricular White-Matter Lesions during Steroid Treatment for Autoimmune Hepatitis.

作者信息

Shibahara Tomoya, Yoshino Fumitaka, Matsuoka Mikiaki, Tachibana Masaki, Nakamura Kuniyuki, Ago Tetsuro, Kuroda Junya, Nakane Hiroshi

机构信息

Cerebrovascular and Neurology Center, National Hospital Organization Fukuokahigashi Medical Center, Fukuoka, Japan.

Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

出版信息

Case Rep Neurol. 2022 Aug 19;14(2):334-340. doi: 10.1159/000526223. eCollection 2022 May-Aug.

Abstract

A 63-year-old woman under treatment of autoimmune hepatitis presented with headache, memory loss, and somnolence. Three months before admission, the patient experienced liver inflammation relapse after prednisolone (PSL) cessation. Consequently, PSL was resumed and then tapered. Cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis with remarkably reduced glucose and elevated angiotensin-converting enzyme and soluble interleukin-2 receptor levels. Magnetic resonance imaging (MRI) revealed prominent bilateral periventricular white-matter lesions, hydrocephalus, ischemic stroke with gadolinium enhancement of frontoparietal and basilar meninges on contrast-enhanced fluid-attenuated inversion recovery. Magnetic resonance angiography (MRA) showed narrowing of the bilateral middle cerebral arteries. Based on these findings, we diagnosed the patient with neurosarcoidosis. Re-increment of PSL improved the neurological symptoms, CSF findings, and abnormalities found on MRI and MRA. This case suggests that neurosarcoidosis may occur as a complication of some autoimmune diseases during immunotherapy administration.

摘要

一名正在接受自身免疫性肝炎治疗的63岁女性出现头痛、记忆力减退和嗜睡症状。入院前三个月,患者在停用泼尼松龙(PSL)后出现肝脏炎症复发。因此,重新开始使用PSL,然后逐渐减量。脑脊液(CSF)检查显示淋巴细胞增多,葡萄糖显著降低,血管紧张素转换酶和可溶性白细胞介素-2受体水平升高。磁共振成像(MRI)显示双侧脑室周围白质病变明显、脑积水、缺血性中风,在对比增强液体衰减反转恢复序列上额顶叶和基底脑膜有钆增强。磁共振血管造影(MRA)显示双侧大脑中动脉狭窄。基于这些发现,我们诊断该患者患有神经结节病。PSL再次增量改善了神经症状、CSF检查结果以及MRI和MRA发现的异常。该病例提示神经结节病可能作为某些自身免疫性疾病在免疫治疗期间的并发症发生。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/faed/9459521/b15872b0ed35/crn-0014-0334-g01.jpg

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