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结节性多动脉炎伴发后部可逆性脑病综合征。

Polyarteritis nodosa presenting with posterior reversible leukoencephalopathy syndrome.

机构信息

Neurology, The Walton Centre NHS Foundation Trust, Liverpool, UK

Neurology, The Walton Centre NHS Foundation Trust, Liverpool, UK.

出版信息

BMJ Case Rep. 2022 Aug 31;15(8):e250867. doi: 10.1136/bcr-2022-250867.

Abstract

A man in his 20s presented following a generalised tonic-clonic seizure on a background of a recent diagnosis of hepatitis B (HBV). During admission, he was severely hypertensive and imaging findings confirmed a diagnosis of posterior reversible leukoencephalopathy syndrome (PRES). The patient subsequently developed multiorgan involvement with an axonal sensorimotor neuropathy, vascular cutaneous lesions and multiple bilateral renal and splenic infarcts. Based on the 2012 Revised International Chapel Hill Consensus Criteria, a diagnosis of polyarteritis nodosa (PAN) with secondary PRES was made. The patient was given intravenous methylprednisolone, followed by a prolonged course of oral prednisolone, and tenofovir antiviral therapy to target HBV seroconversion. He made a good neurological recovery with resolution of imaging changes. This case highlights the importance of a low threshold for systemic screening for young patients presenting with PRES secondary to uncontrolled hypertension and the importance of viral screening, particularly for HBV.

摘要

一位 20 多岁的男性,在近期被诊断为乙型肝炎(HBV)的背景下出现全身性强直阵挛性发作。住院期间,他血压严重升高,影像学检查结果确诊为后部可逆性脑病综合征(PRES)。随后,该患者出现多器官受累,表现为轴索性感觉运动神经病、血管性皮肤损伤以及多发双侧肾和脾梗死。根据 2012 年修订的国际夏佩特山会议共识标准,诊断为伴继发性 PRES 的结节性多动脉炎(PAN)。患者接受了静脉注射甲基强的松龙治疗,随后进行了长时间的口服泼尼松龙治疗,并进行了抗病毒治疗以实现 HBV 血清转换。他的神经功能恢复良好,影像学改变也得到了缓解。本病例强调了对出现 PRES 且血压控制不佳的年轻患者进行系统性筛查的重要性,以及病毒筛查的重要性,特别是乙型肝炎病毒筛查。

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本文引用的文献

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Neurological Involvement in Primary Systemic Vasculitis.原发性系统性血管炎的神经系统受累
Front Neurol. 2019 Apr 26;10:430. doi: 10.3389/fneur.2019.00430. eCollection 2019.
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Polyarteritis Nodosa Neurologic Manifestations.结节性多动脉炎神经系统表现。
Neurol Clin. 2019 May;37(2):345-357. doi: 10.1016/j.ncl.2019.01.007. Epub 2019 Mar 16.
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Diagnosis and classification of polyarteritis nodosa.结节性多动脉炎的诊断和分类。
J Autoimmun. 2014 Feb-Mar;48-49:84-9. doi: 10.1016/j.jaut.2014.01.029. Epub 2014 Jan 28.

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