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系统性红斑狼疮伴纵向广泛横贯性脊髓炎:病例报告及文献复习

Longitudinally extensive transverse myelitis in systemic lupus erythematosus: case report and review of the literature.

作者信息

Nardone Raffaele, Fitzgerald Ryan T, Bailey Ariel, Zuccoli Giulio

机构信息

Department of Neurology, Christian Doppler Klinik, Paracelsus Medical University, Salzburg, Austria; Spinal Cord Injury and Tissue Regeneration Center, Paracelsus Medical University, Salzburg, Austria; Department of Neurology, Franz Tappeiner Hospital, Merano, Italy.

Department of Radiology, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

出版信息

Clin Neurol Neurosurg. 2015 Feb;129:57-61. doi: 10.1016/j.clineuro.2014.11.014. Epub 2014 Dec 13.

Abstract

OBJECTIVE

To report a case of longitudinally extensive transverse myelitis (LETM), a rare but disabling condition defined as a lesion of the spinal cord that extends over four or more vertebrae on MRI, in association with systemic lupus erythematosus (SLE).

METHODS

We present a rare case of LETM involving the cervical and thoracic spinal cord in a patient with SLE and review the existing literature on the association of lupus-associated myelitis.

RESULTS

LETM is included within the diagnostic criteria for Neuromyelitis Optica (NMO), but is also known to be associated with a wide range of auto-immune diseases. Only 37 cases of LETM in patients with SLE have been previously described. We performed an updated review on epidemiology, pathophysiology, clinical features, diagnosis, management, and prognosis of LETM in the setting of SLE.

CONCLUSION

Due to the generally poor prognosis of LETM in SLE patients, prompt diagnosis and treatment is of critical importance for a positive clinical outcome. We provide a comprehensive perspective of past and current literature in order to aid diagnosis and management of this rare phenomenon.

摘要

目的

报告一例纵向广泛横贯性脊髓炎(LETM),这是一种罕见但致残的疾病,在磁共振成像(MRI)上定义为脊髓病变延伸超过四个或更多椎体,并与系统性红斑狼疮(SLE)相关。

方法

我们报告一例罕见的LETM病例,累及一名SLE患者的颈髓和胸髓,并回顾了关于狼疮相关性脊髓炎关联的现有文献。

结果

LETM包含在视神经脊髓炎(NMO)的诊断标准内,但也已知与多种自身免疫性疾病相关。此前仅描述过37例SLE患者的LETM病例。我们对SLE背景下LETM的流行病学、病理生理学、临床特征、诊断、管理和预后进行了更新回顾。

结论

由于SLE患者中LETM的总体预后通常较差,及时诊断和治疗对于良好的临床结果至关重要。我们提供过去和当前文献的全面观点,以帮助诊断和管理这一罕见现象。

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