摘要
目的 IgG4相关性疾病(IgG4-related disease,IgG4-RD)是2010年才正式命名的疾病,本病极为罕见,为引起关注报道1例。方法将我院诊断的1例IgG4相关性硬脊膜炎致脊髓压迫的患者从临床表现、病理、辅助检查、治疗等方面对其进行阐述并对相关文献进行复习。结果该患者以双下肢无力伴排尿困难起病,MRI:颈5至胸4椎水平脊髓前后缘条状高信号。手术组织病理学检查:免疫组化标记显示多量浆细胞表达IgG及IgG4,局部IgG4阳性数>10个/10HEP;考虑为IgG4相关的硬化性疾病,伴组织细胞增生。诊断为IgG4相关性硬脊膜炎致脊髓压迫症。给予激素冲击治疗有所好转。结论 IgG4-RD可以引起硬脊膜炎并可出现脊髓压迫症状,应该值得注意。
Objective To report a case of spinal cord compression caused by IgG4-related sclerosing pachymeningitis. Methods Take the case of spinal cord compression caused by IgG4-related sclerosing pachymeningitis as example,we demonstrated the clinical manifestations,pathology,auxiliary examination and treatment,and reviewed the related literature.Results The 39-year old male patient had pain in his back for 15 days,accompanying bladder dysfunction for 3 days and weakness in his lower limbs for 2 days. Spinal cord MRI showed rapid-progressing compression from C5 to T4. Emergency operation was performed and the immunohistochemical staining showed that IgG and IgG4 were expressed in majority plasma cells,and the number of positive IgG4 in the area was 〉10/10 HEP,considered as IgG4 related sclerosing disease and histiocytic hyperplasia. Given glucocorticoid pulse therapy,he made a slight improvement. Conclusion IgG4-RD could induce spinal cord compression of,which is worthy of note.
出处
《中风与神经疾病杂志》
CAS
2018年第1期40-43,共4页
Journal of Apoplexy and Nervous Diseases
基金
吉林大学优秀青年教师培养计划(No:419080500582)
