颅内瘤样脱髓鞘的诊治分析.DOC

颅内瘤样脱髓鞘病变的诊治分析 程 刚1 ,黄 楹2(300070天津,天津医科大学研究生院1；300060天津,天津市环湖医院颅底外科2) [摘要] 目的 探讨颅内瘤样脱髓鞘病变的诊治。方法 回顾性分析10例颅内瘤样脱髓鞘患者的临床资料。6例行显微外科手术，4例行立体定向活检，经病理证实后部分患者给予药物治疗；其中6例患者术前未考虑脱髓鞘病变。结果 MRI显示单发病变8例，多发病变2例；肿物直径20～54mm；10位患者均有轻中度的周围水肿；8位患者存在脑回、脑室轻中度受压的占位效应，无中线移位；2例行MRS检查示NAA减低、CHO略减低、倒置LAC峰；4例弥散加权成像（DWI）显示病灶中间低信号，周围高信号；6例患者病变MRI强化部分CT为低密度影。本病的病理特征为神经纤维的髓鞘脱失，而轴索和神经纤维保持完整。 术后仍有急性期表现患者给予激素治疗，2位患者术后出现肢体运动障碍较前加重，1例颞顶部病变术后出现命名性失语，其余患者症状均明显改善。所有患者行电话或门诊随访3～84个月，平均34个月，8例患者症状明显好转或完全恢复，其中1例病灶变化不明显，其余明显缩小或消失；另外2例患者出院后症状反复出现，且有精神症状，后经内科诊断为MS。结论 颅内脱髓鞘临床表现无特征性及影像学不典型是导致术前难以诊断的主要原因。病理检查是确诊该病的最可靠方法，有时需要借助特殊染色同低级别肿瘤相鉴别。类固醇激素治疗后绝大多数患者预后较好，少数情况下还要结合其他治疗方案。 [关键词] 脱髓鞘；诊断；治疗；分析 [通信作者]黄楹，E-mail：Yinghuang00@ The diagnosis and treatment analysis of intracranial tumefactive demyelinating lesions Cheng Gang1，Huang Ying2（1Graduate School,Tianjin Medical University,，Tianjin，300070,2Department of Neurosurgery,Huan Hu Hosipital ,Tianjin，300060,China) [Abstract] Objective To explore the diagnosis, treatment reasons of intracranial tumefactive demyelinating lesions. Method The clinical data of 10 cases with intracranial tumefactive demyelinating lesions treated in our department from January 2006 to May 2013 were analyzed retrospectively. Results In all 10 cases，6 cases underwent surgical treatment，4 cases underwent stereotactic biopsy and were confirmed by Pathological examinations.6 cases of intracranial tumefactive demyelinating Lesions did not be diagnosed before operation. Conclusions intracranial tumefactive demyelinating lesions is usually diagnosed based on auxiliary inspections and clinical feature. With steroid consolidate treatment after operation, the prognosis is good，but in a few cases need to combined with other treatments. Non-characteristic clinical features，non-characteristic radiographic manifestations contribute to misdiagnosis. [Key Word] Tumefactive demyelinating lesions；Diagnosis ；Therapy ；analysis Corresponding author：Huang Ying，E-mail：Yinghuang00@ 多发性硬化(multiple sclero