Liu Hui-Miao, Dong Ci, Zhang Yong-Zhi, Tian Ya-Yun, Chen Hong-Xu, Zhang Sai, Li Na, Gu Ping
Department of Neurology, the First Hospital of Hebei Medical University Brain Ageing and Cognitive Neuroscience Laboratory of Hebei, Shijiazhuang, Hebei, China.
Medicine (Baltimore). 2017 Oct;96(40):e7958. doi: 10.1097/MD.0000000000007958.
To investigate the clinical and MRI characteristics of spinal cord nerve Behçet's disease.
One patient with spinal cord nerve Behçet's disease was admitted to our hospital at October 20, 2015.
Spinal cord nerve Behçet's disease.
Retrospective analysis was performed on such case as well as 16 cases of spinal cord nerve Behçet's disease reported in China or abroad.
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.
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.
探讨脊髓型神经白塞病的临床及磁共振成像(MRI)特征。
2015年10月20日,一名脊髓型神经白塞病患者入住我院。
脊髓型神经白塞病。
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。
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。
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。