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神经白塞病脊髓型的临床及影像学特征:一例病例报告及系统综述

Clinical and imaging features of spinal cord type of neuro Behçet disease: A case report and systematic review.

作者信息

Liu Hui-Miao, Dong Ci, Zhang Yong-Zhi, Tian Ya-Yun, Chen Hong-Xu, Zhang Sai, Li Na, Gu Ping

机构信息

Department of Neurology, the First Hospital of Hebei Medical University Brain Ageing and Cognitive Neuroscience Laboratory of Hebei, Shijiazhuang, Hebei, China.

出版信息

Medicine (Baltimore). 2017 Oct;96(40):e7958. doi: 10.1097/MD.0000000000007958.

DOI:10.1097/MD.0000000000007958
PMID:28984755
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5737991/
Abstract

RATIONAL

To investigate the clinical and MRI characteristics of spinal cord nerve Behçet's disease.

PATIENT CONCERNS

One patient with spinal cord nerve Behçet's disease was admitted to our hospital at October 20, 2015.

DIAGNOSE

Spinal cord nerve Behçet's disease.

INTERVENTIONS

Retrospective analysis was performed on such case as well as 16 cases of spinal cord nerve Behçet's disease reported in China or abroad.

OUTCOMES

Seventeen cases of spinal cord type of neuro Behçet's disease include 13 men and 4 women, with an average age of onset of 34.8 years old. The mean time from Behçet's disease symptoms to spinal cord involvement were 10.8 years. The initial symptom in one case was spinal cord injury, and another 4 cases had a recurrence course. The most common performance of spinal cord injury was sensory disturbance (82.4%), following by weakness (76.5%), sphincter or sexual dysfunction (58.8%), and pain in back, backside of neck or lower chest (29.4%). The number of cells was slightly increased or the protein level was increased in cerebrospinal fluid test. And the water channel protein antibody and oligoclonal band of serum levels were all negative. The spinal cord injury involved more than 3 vertebral bodies in 10 cases, and involved more than half of spinal cord in sagittal plane in 8 cases. In acute stage, shock therapy with large dose of glucocorticoid was generally applied both in China and abroad.

LESSONS

The clinical features of spinal cord nerve Behçet's disease were various, making it easily misdiagnosed. Longitudinal extensive transverse myelitis performs as a characteristic manifestation.

摘要

目的

探讨脊髓型神经白塞病的临床及磁共振成像(MRI)特征。

患者情况

2015年10月20日,一名脊髓型神经白塞病患者入住我院。

诊断

脊髓型神经白塞病。

干预措施

对该病例以及国内外报道的16例脊髓型神经白塞病病例进行回顾性分析。

结果

17例脊髓型神经白塞病患者中,男性13例,女性4例,平均发病年龄34.8岁。白塞病症状出现至脊髓受累的平均时间为10.8年。1例首发症状为脊髓损伤,4例有复发病程。脊髓损伤最常见的表现为感觉障碍(82.4%),其次为无力(76.5%)、括约肌或性功能障碍(58.8%)以及背部、颈后部或下胸部疼痛(29.4%)。脑脊液检查细胞数轻度增加或蛋白水平升高,血清水通道蛋白抗体及寡克隆带均为阴性。10例脊髓损伤累及3个以上椎体,8例矢状面累及脊髓超过一半。急性期国内外普遍采用大剂量糖皮质激素冲击治疗。

经验教训

脊髓型神经白塞病临床特征多样,易误诊,纵向广泛横贯性脊髓炎为其特征性表现。

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A Major Review: Current Aspects of Ocular Behçet's Disease in Japan.一项重大综述:日本眼部白塞病的当前状况
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Magnetic resonance imaging in neuromyelitis optica.视神经脊髓炎的磁共振成像
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