A Case Report Of IgG4-related Benign Intracranial Tumor And Literature Review

Objective:Through the analysis of a case of IgG4 related intracranial benign tumor（bilateral parasellar and left frame）which recurred 15 months after surgical resection and was significantly improved and relieved by glucocorticoid combined with immunosuppressant treatment,we can explore the occurrence and development mechanism,clinical manifestation,auxiliary examination and treatment status of IgG4related diseases.Methods:A patient who was diagnosed as intracranial space occupying lesion in an external hospital and treated with lesion excision was confirmed as IgG4 related disease by postoperative pathology and immunohistochemistry.The condition recurred again more than 15 months after operation,and the patient admitted to the rheumatology department of our hospital with malignant exophthalmos and obvious visual impairment underwent serological and morphological examination.The patient was diagnosed as IgG4 related diseases,IgG4 related orbital lesions,IgG4 related hypophysitis and IgG4 related ophthalmopathy.Glucocorticoids were given in combination with immunosuppressants such as methotrexate,thalidomide and hydroxychloroquine.The recovery of malignant exophthalmos,headache,visual acuity,IgG4 level and intracranial space occupying were observed.The current situation of clinical diagnosis and treatment of this kind of disease was reviewed.Results:This patient had clinical manifestations of IgG4 related diseases and intracranial space occupying lesions,and was accompanied by malignant exophthalmos.The serum IgG4 level increased significantly（10 times higher than the normal value）,Ig G and leukocyte levels increased,and the orbital CT plain scan reported swelling of the left extraocular muscle and exophthalmos.It was diagnosed as IgG4 related diseases,IgG4related orbital lesions,IgG4 related hypophysitis and IgG4 related ophthalmopathy.After treatment with glucocorticoid and immunosuppressant,the level of serum IgG4decreased rapidly,and the symptoms of malignant exophthalmos and headache were relieved quickly.There were no obvious adverse reactions during the treatment.Conclusion:Because IgG4 related diseases are relatively rare,they have been gradually recognized in recent years.IgG4 related central nervous system diseases are rarely reported at home and abroad,and have no obvious common with the clinical manifestations of other IgG4 related diseases.Patients usually see a doctor with nerve tissue compression symptoms,which is very easy to miss diagnosis,misdiagnosis and over treatment,and is easy to relapse after surgical treatment.Pathological examination found that tissue lymphocytes,IgG4 plasma cell infiltration and fibrosis can be diagnosed^[1],but pathological biopsy of intracranial lesions is difficult.Therefore,it is urgent to find new serological indexes or imaging features to improve the early diagnosis of IgG4 related intracranial space occupying diseases.