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一名7岁患者以急性播散性脑脊髓炎为表现的急性起病性构音障碍:一种罕见的临床和影像学实体。

Acute onset anarthria in a 7-year-old patient as a presentation of acute disseminated encephalomyelitis: A rare clinical and radiological entity.

作者信息

Belfkih Rachid, Khayat Omar Ghomari

机构信息

Abdelmalek Saadi University, Faculty of medicine and pharmacy of Tangier, Tangier, Morocco.

Department of neurology, University Hospital Center of Tangier-Tetouan-Al hoceima, Tangier, Morocco.

出版信息

Radiol Case Rep. 2021 Sep 4;16(11):3418-3421. doi: 10.1016/j.radcr.2021.08.016. eCollection 2021 Nov.

DOI:10.1016/j.radcr.2021.08.016
PMID:34522279
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8426168/
Abstract

Acute disseminated encephalomyelitis (ADEM), also called post-infectious encephalomyelitis, is defined as an immune-mediated inflammatory and demyelinating event that involves the central nervous system. It usually follows an infectious episode or an active immunization several weeks prior the disease onset. ADEM is typically presented with an encephalopathy associated to focal neurological signs. Cerebrospinal fluid analysis is usually nonspecific, showing signs of central nervous system inflammation with negative viral and bacterial cultures. The diagnosis is based on clinical and MRI findings. Patients with ADEM respond well to anti-inflammatory and immunosuppressive agents, with high-dose of intravenous steroids administered as first-line treatment. Herein, we present the case of a 7-year-old male patient diagnosed of acute disseminated encephalomyelitis most likely secondary to a viral upper respiratory tract infection. It is a unique case, as the inaugurating sign is an acute onset anarthria. The patient responded favorably to the first-line therapy with an almost full recovery within the first week of treatment.

摘要

急性播散性脑脊髓炎(ADEM),也称为感染后脑脊髓炎,被定义为一种涉及中枢神经系统的免疫介导的炎症性和脱髓鞘性疾病。它通常在疾病发作前几周出现感染性发作或主动免疫后发生。ADEM通常表现为伴有局灶性神经体征的脑病。脑脊液分析通常无特异性,显示中枢神经系统炎症迹象,病毒和细菌培养均为阴性。诊断基于临床和MRI表现。ADEM患者对抗炎和免疫抑制剂反应良好,高剂量静脉注射类固醇作为一线治疗。在此,我们报告一例7岁男性患者,诊断为急性播散性脑脊髓炎,最可能继发于病毒性上呼吸道感染。这是一个独特的病例,因为首发症状是急性构音障碍。患者对一线治疗反应良好,在治疗的第一周内几乎完全康复。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ad9/8426168/68b8521030b5/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ad9/8426168/9081c1e2e2d7/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ad9/8426168/b86f5d03c7b8/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ad9/8426168/68b8521030b5/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ad9/8426168/9081c1e2e2d7/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ad9/8426168/b86f5d03c7b8/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ad9/8426168/68b8521030b5/gr3.jpg

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本文引用的文献

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Neurology. 2011 Aug 9;77(6):580-8. doi: 10.1212/WNL.0b013e318228c0b1. Epub 2011 Jul 27.
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Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children.儿童急性播散性脑脊髓炎的临床及神经放射学特征
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