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中年埃塞俄比亚女性合并脑干受累的视神经脊髓炎:病例报告及文献复习。

Neuromyelitis optica with brain stem involvement in a middle-aged Ethiopian woman: a case report and review of literature.

机构信息

Department of Neuro-surgery, St. Paul's Hospital Millennium Medical College, Addis Ababa, Ethiopia.

Department of Internal Medicine, St. Paul's Hospital Millennium Medical College, Addis Ababa, Ethiopia.

出版信息

J Med Case Rep. 2021 Oct 1;15(1):489. doi: 10.1186/s13256-021-03019-6.

Abstract

INTRODUCTION

Neuromyelitis optica is a demyelinating disease of the central nervous system that predominantly affects the optic nerves and spinal cord. In neuromyelitis optica, white blood cells and antibodies primarily attack the optic nerves and the spinal cord, but may also attack the brain. Brainstem manifestation has been described recently. So far, neuromyelitis optica is very rare in Ethiopia and there were only two case reports, but this is the first case report of neuromyelitis optica with brainstem involvement.

CASE PRESENTATION

A 47-year-old Addis Ababa woman presented to Saint Paul's Hospital Millennium Medical College with a history of visual loss of 7 years and bilateral lower limb weakness of 4 days duration. She had bilateral oculomotor nerve palsy. Her past medical history showed systemic hypertension for 18 years and dyslipidemia for 1 year. The objective evaluation of the patient revealed right optic nerve atrophy suggesting optic neuritis and flaccid paraplegia with sensory level at the fourth thoracic vertebra. Diagnostic work-up using electromyography and spinal magnetic resonance imaging revealed demyelinating anterior visual pathway dysfunction and signs of extensive cervicothoracic transverse myelitis from the third cervical to lower thoracic vertebrae, respectively. Then a diagnosis of neuromyelitis optica was established. After treatment with high-dose systemic steroid followed by azathioprine, the patient was stable for several months with significant improvement of vision and lower-extremity weakness with no relapse of symptoms.

CONCLUSION

The case described here is a rare inflammatory demyelinating disorder of the central nervous system occurring in East Africa. It reminds clinicians to suspect neuromyelitis optica in a patient who presented with unexplained recurrent optic neuritis to make a timely diagnosis and prevention of permanent neuronal damage. Neuromyelitis optica can also be associated with oculomotor nerve involvement.

摘要

介绍

视神经脊髓炎是一种中枢神经系统脱髓鞘疾病,主要影响视神经和脊髓。在视神经脊髓炎中,白细胞和抗体主要攻击视神经和脊髓,但也可能攻击大脑。最近描述了脑干表现。到目前为止,视神经脊髓炎在埃塞俄比亚非常罕见,仅有两例病例报告,但这是首例伴有脑干受累的视神经脊髓炎病例报告。

病例介绍

一名 47 岁的亚的斯亚贝巴女性因 7 年的视力丧失和 4 天的双侧下肢无力到圣保禄医院千年医科大学就诊。她有双侧动眼神经麻痹。她的既往病史显示 18 年的系统性高血压和 1 年的血脂异常。患者的客观评估显示右侧视神经萎缩,提示视神经炎和弛缓性截瘫,感觉平面位于第四胸椎。使用肌电图和脊髓磁共振成像进行的诊断性检查分别显示脱髓鞘性前视觉通路功能障碍和从第三颈椎到下胸椎的广泛颈胸横贯性脊髓炎的迹象。然后诊断为视神经脊髓炎。在高剂量全身类固醇治疗后,再用硫唑嘌呤治疗,患者病情稳定数月,视力和下肢无力明显改善,无症状复发。

结论

这里描述的病例是一种罕见的中枢神经系统炎症性脱髓鞘疾病,发生在东非。它提醒临床医生在出现不明原因的复发性视神经炎的患者中怀疑视神经脊髓炎,以便及时诊断和预防永久性神经元损伤。视神经脊髓炎也可能与动眼神经受累有关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3f4d/8485543/326fd3871b03/13256_2021_3019_Fig1_HTML.jpg

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