中枢神经系统脱髓鞘假瘤与胶质瘤的鉴别诊断和临床分析

Differential diagnosis and clinical analysis of central nervous system demyelinating pseudotumor and Glioma

目的探讨CNS脱髓鞘假瘤(DPT)的临床特点,并与胶质瘤进行鉴别,以提高对DPT的认识。方法收集均经病理证实的13例DPT和17例胶质瘤患者的临床资料,对比二者之间的临床、影像学、实验室检查及病理特点,并分析DPT的治疗与转归。结果 DPT组平均发病年龄高于胶质瘤组(P<0.05)。DPT组女性多见,胶质瘤组无明显性别差异。DPT组急性或亚急性起病(76.9%)常见,胶质瘤组慢性起病常见(70.5%)。DPT组首发症状以头痛(38.4%)多见,胶质瘤组以头痛(41.1%)、痫性发作(23.5%)多见。DPT组影像学部分病灶可见"垂直征"及特异性"开环征",DWI高b值呈高信号,胶质瘤DWI高b值多呈低信号。MRS检查出现特异性β,γ-谷氨酸复合物(Glx)峰升高可与胶质瘤进行鉴别。两组CSF压力、蛋白相比差异有统计学意义(均P<0.05)。病理活组织检查出现特异性核分裂状的Creutzfeldt细胞及CD68免疫组化染色阳性有助于两者鉴别诊断。经激素治疗后DPT大部分病灶明显缩小或消失,部分可发展为多发性硬化。结论DPT在临床表现及影像学等方面与胶质瘤相似。影像学检查出现"垂直征"、"开环征"及β,γ-Glx峰升高可能具有特异性,DWI/MRS检查有助于鉴别胶质瘤。激素实验性治疗可能有助于鉴别病变性质,但最终确诊仍需病理结果,部分可发展为多发性硬化。

Objective To investigate the clinical features of CNS demyelinating pseudotumor （DPT）, and to identify the glioma, in order to improve the understanding of DPT. Methods The clinical data of pathologically confirmed 13 cases of DPT and 17 cases of glioma patients were collected. Clinical, radiological and pathological features were compared, and treatment and outcome of DPT were analyzed. Results The mean age of onset in the DPT group was significantly higher than that in the glioma group （ P 〈 0. 05 ）. DPT group was predominatly females, while there was no significant gender difference in glioma group. DPT primarily had acute or subacute onset （76.9%） ,while glioma group had chronic onset in common（70.5% ）. Headache （38.4%） was the most common as the first symptom, but in glioma group headache （41.1% ）, seizures （23.5%） more were more common. DPT group imaging part of the lesion can show ＂vertieal sign＂ and speeific＂ open-loop sign＂, DWI high b value was high signal, while in glioma group DWI high b value was mostly low signal. MRS examination showed specific [3, ^-Glx peak elevation can be identified with glioma. There were significant difference in the CSF pressure and protein between the two groups （all P 〈 0. 05 ）. Pathological biopsy of specific mitotic Creutzfeldt cells and CD68 immunohistoehemical staining positive were helpful for the differential diagnosis of both. After hormone therapy, most of the DPT lesions were significantly reduced or disappeared, some could be developed as multiple sclerosis. Conclusions DPT is similar with glioma in clinical manifestations and imaging and other aspects. The presence of ＂vertical sign＂, ＂open-loop sign＂ and β,γ-Glx peak elevation may be specific in imaging studies, DWI/MRS may help to identify glioma. Hormone experimental treatment may help to identify the nature of the lesion, but the final diagnosis still needs pathological results, some DPT can be developed into multiple sclerosis.