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水通道蛋白4(AQP-4)免疫球蛋白G血清阳性视神经脊髓炎:综述与病例报告

AQUAPORIN-4 (AQP-4) IMMUNOGLOBULIN G SEROPOSITIVE NEUROMYELITIS OPTICA: A REVIEW AND CASE REPORT.

作者信息

Nwaze C, Eghwrudjakpor Y, Chinedu-Anunaso N

机构信息

Regions Stroke and Neuroscience Hospital, Owerri, Imo State, Nigeria.

出版信息

Ann Ib Postgrad Med. 2023 Dec;21(3):79-84. Epub 2024 Jan 30.

PMID:38706629
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11065186/
Abstract

BACKGROUND

Neuromyelitis optica spectrum disorder (NMOSD) is a relatively new terminology composed to encompass patients with neuromyelitis optica (NMO) and related immunological conditions. The diagnosis of this condition requires a seropositive aquaporin-4 immunoglobulin G (AQP-4 IgG), the presence of at least one core clinical characteristic and the exclusion of alternative diagnoses. Very few cases have been reported in sub-Saharan Africa.

OBJECTIVE

The aim of this article is to report a classical case of NMOSD with AQP-4 IgG seropositivity and normal brain, cervical and thoracic MRI findings.

RESULT

We report a 25-year-old Nigerian woman who presented with recurrent and alternating weakness, pain and numbness of all limbs, associated with episodic painful left-sided tonic spasms and urinary incontinence. She had earlier had symptoms of recurrent, episodic and alternating loss of vision in both eyes, associated with ocular pain.Examination findings revealed an intact mental status, no cranial nerve deficit and no focal limb weakness. Right-sided deep tendon reflexes were exaggerated. Vital signs were within normal limits. Brain MRI, Cervical spine MRI and Thoracic spine MRI all revealed normal findings. Serum aquaporin-4 IgG assay returned positive with a titer of 1:32.She was commenced on high dose steroids and there was gradual improvement of symptoms.

CONCLUSION

These findings confirmed the diagnosis of neuromyelitis optica spectrum disorder, and satisfies the diagnostic criteria published in 2015 by the International Panel for NMO Diagnosis (IPND).

摘要

背景

视神经脊髓炎谱系障碍(NMOSD)是一个相对较新的术语,用于涵盖视神经脊髓炎(NMO)患者及相关免疫性疾病。该疾病的诊断需要水通道蛋白4免疫球蛋白G(AQP - 4 IgG)血清学阳性、至少存在一项核心临床特征以及排除其他诊断。撒哈拉以南非洲地区报告的病例极少。

目的

本文旨在报告一例具有AQP - 4 IgG血清学阳性且脑、颈段和胸段MRI检查结果正常的经典NMOSD病例。

结果

我们报告了一名25岁的尼日利亚女性,她出现反复交替性的四肢无力、疼痛和麻木,并伴有发作性左侧疼痛性强直性痉挛和尿失禁。她早些时候曾有双眼反复、发作性和交替性视力丧失的症状,并伴有眼痛。检查发现精神状态正常,无颅神经功能缺损,无局灶性肢体无力。右侧深腱反射亢进。生命体征在正常范围内。脑部MRI、颈椎MRI和胸椎MRI均显示正常结果。血清水通道蛋白4 IgG检测呈阳性,滴度为1:32。她开始接受大剂量类固醇治疗,症状逐渐改善。

结论

这些发现证实了视神经脊髓炎谱系障碍的诊断,并符合国际NMO诊断小组(IPND)2015年发布的诊断标准。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5c3/11065186/7e37371150ca/AIPM-21-79_F1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5c3/11065186/7e37371150ca/AIPM-21-79_F1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a5c3/11065186/7e37371150ca/AIPM-21-79_F1.jpg

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Mult Scler Relat Disord. 2021 Aug;53:103030. doi: 10.1016/j.msard.2021.103030. Epub 2021 May 28.
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Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access.东非地区视神经脊髓炎谱系障碍病例,凸显诊断及医疗服务可及性方面的挑战。
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Worldwide Incidence and Prevalence of Neuromyelitis Optica: A Systematic Review.
全球视神经脊髓炎的发病率和患病率:一项系统评价。
Neurology. 2021 Jan 12;96(2):59-77. doi: 10.1212/WNL.0000000000011153. Epub 2020 Dec 11.
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Lancet Neurol. 2020 May;19(5):391-401. doi: 10.1016/S1474-4422(20)30070-3.
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