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间隔26年的多相性急性播散性脑脊髓炎:一例具有新表现的病例报告

Multiphasic Acute Disseminated Encephalomyelitis with a Temporal Gap of 26 Years: A Case Report with Novel Presentation.

作者信息

Asif Muhammad Hamza, Butt Armeen, Riaz Khadija, Shahzad Faizan, Asif Maimoonah, Farhan Muhammad, Ahmed Waqas, Shami Besher

机构信息

Rawalpindi Medical University, Rawalpindi, Pakistan.

Department of Medicine, Shalalamr Hospital, Lahore, Pakistan.

出版信息

Case Rep Neurol. 2024 Aug 19;16(1):242-248. doi: 10.1159/000540951. eCollection 2024 Jan-Dec.

Abstract

INTRODUCTION

Acute disseminated encephalomyelitis (ADEM) is an acute autoimmune demyelinating disease of the central nervous system that typically follows an acute viral infection or post-vaccination. It is more common in children than in adults and is characterized by widespread demyelination of the white matter of the brain and spinal cord. ADEM typically presents as a monophasic illness. Multiphasic ADEM has been an emerging topic with case reports highlighting the importance of the need for further investigation and literature on this topic. Multiphasic ADEM in adults is a rare presentation with an atypical course that can cause a delay in diagnosis leading to disability in patients.

CASE PRESENTATION

Multiple case reports have shown the presence of multiphasic ADEM without an established cause, emphasizing the importance of additional insight and data in this context. This is a unique case of multiphasic ADEM in a 59-year-old male with a temporal gap of 26 years with an idiopathic cause. He was suffering from many neurologic symptoms involving his upper and lower extremities and level of consciousness, and after he was diagnosed with ADEM, he was commenced on steroids and achieved a marked improvement in his clinical status.

CONCLUSION

Rarely, ADEM can present with recurrence or multiple phases, which requires prompt diagnosis and treatment. These phases may or may not be preceded by an identifiable trigger. Most patients respond well to steroids. However, further studies are required to achieve a good understanding and guidelines when dealing with such cases.

摘要

引言

急性播散性脑脊髓炎(ADEM)是一种中枢神经系统的急性自身免疫性脱髓鞘疾病,通常发生在急性病毒感染或接种疫苗之后。它在儿童中比在成人中更常见,其特征是脑和脊髓白质广泛脱髓鞘。ADEM通常表现为单相疾病。多相性ADEM一直是一个新兴话题,病例报告强调了对此进行进一步研究和相关文献的必要性。成人多相性ADEM是一种罕见的表现形式,病程不典型,可能导致诊断延迟,进而使患者致残。

病例介绍

多项病例报告显示存在病因不明的多相性ADEM,强调了在这种情况下深入了解和获取数据的重要性。这是一例59岁男性的独特多相性ADEM病例,两次发病间隔26年,病因不明。他出现了许多涉及上下肢及意识水平的神经症状,在被诊断为ADEM后,开始使用类固醇治疗,临床状况有显著改善。

结论

罕见情况下,ADEM可表现为复发或多相性,这需要及时诊断和治疗。这些阶段可能有或没有可识别的触发因素。大多数患者对类固醇治疗反应良好。然而,在处理此类病例时,还需要进一步研究以加深理解并制定指导原则。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51c8/11521480/47f0e29579e8/crn-2024-0016-0001-540951_F01.jpg

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