中枢神经系统炎性脱髓鞘性假瘤的诊断和治疗

目的:描述中枢神经系统炎性脱髓鞘性假瘤的临床表现,探讨该病的病理学特征以及MRI诊断价值,以期加强对本病的认识并提高诊断的正确性。方法:对2例经手术病理证实中枢神经系统炎性脱髓鞘性假瘤进行MRI检查,对其临床表现、影像学表现进行了回顾性分析。结果:MRI显示2例病变均表现为局限性肿块呈均匀长T1、长T2信号,临近未见伴有多发病灶。术前均被误诊为胶质瘤,经手术切除病变,病理证实为中枢神经系统炎性脱髓鞘性假瘤。结论:中枢神经系统炎性脱髓鞘性假瘤的临床和影像学诊断较为困难,常将本病误诊为肿瘤,此时可以先使用激素试验治疗,以避免手术或放射治疗造成的严重损伤。

Diagnosis and Treatment of Demyelinating Pseudotumor of the Central Nervous System

Aim: To describe the clinical manifestation of demyelinating pseudotumor of thecentral nervous system (CNS), and to discuss the pathological features and MRI diagnostic value,the knowledge and the diagnostic rightness about the disease shoud be further strengthened andimproved. Methods: Two pathologically proved cases of demyelinating pseudotumor of CNS werestudied with MRI, the clinical manifestation and the MRI imaging features were retrospectivelyanalyzed. Results: MRI imaging demonstrated localized mass without adjacent multiple accompanyinglesions in 2 cases, T1WI and T2WI showed homogenous low signal. After surgical resection, thepathologic diagnosis of the disease was central nervous systematic demyelinated inflammatorypseudotumor which was all misdiagnosed as glioma preoperatively. Conclusion: It is a difficulttask to make the correct diagnosis of CNS demyelinating pseudotumor based on the clinical informationand MR imaging findings, and this disease entity is often misdiagnosed as tumors by MR imaging,in such circumstances, providing test steroid therapy, thus avoiding the devastating injury causedby surgery or radiation therapy.