皮肌炎合并颅内肿瘤12例分析

目的提高对皮肌炎(DM)合并颅内肿瘤的认识,探讨其发病机制,总结诊断和治疗此类病例的经验.方法对12例DM合并颅内肿瘤的病例做回顾性分析,并复习23年相关文献.结果p53基因种系突变与DM合并颅内肿瘤有关.DM合并颅内肿瘤相对少见是由于:①脑组织有血脑屏障:②脑组织中淋巴细胞较少,免疫应答及免疫交叉的概率相对较少.由于DM有比较典型的临床表现,结合实验室检查,一般能明确诊断,如在发病或治疗过程中出现了头痛、呕吐、视力减退等高颅压症状,出现了偏瘫、失语等定位症状可做头颅CT或磁共振成像(MRI)即可明确.治疗首选类固醇激素,同时处理肿瘤十分必要.结论DM合并颅内肿瘤是一种少见疾病,应及时明确诊断,恰当选择治疗措施.

The analysis of twelve patients with dermatomyositis complicated with neoplasma

Objective To improve the recognition of dermatomyositis (DM) complicated with intracranial neoplasma and to explore its pathogenesis aimed to collect the experience of diagnosis and treatment. Methods Twelve DM patients complicated with intracranial neoplasma were analysed and the literature of recent 23 years were reviewed. Results DM complicated with intracranial neoplasma was associated with the p53 genic mutation and it was rare because of blood-brain barrier and scanty of lymphocytes in brain tissue. Correct diagnosis depended on clinical manifestations and the computerized tomography or the magnetic resonance imaging of brain. Glucocorticoid was the first choice treatment.However, removal the neoplasma was necessary. Conclusion DM complicated with intracranial neoplasma is rare. Clinicians should make the diagnosis promptly and chosen the appropriate treatment.