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目的探讨颅内瘤样脱髓鞘病变的影像学表现、病理特征、治疗及预后。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20~54 mm;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,后经内科诊断为多发性硬化(multiple sclerosis,MS)。结论颅内瘤样脱髓鞘病变的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。
Objective To investigate the imaging features, pathological features, treatment and prognosis of intracranial aneurysmal demyelinating lesions. Methods The clinical data of 10 patients with intracranial intratumoral demyelination were retrospectively analyzed. 6 cases underwent microsurgery and 4 cases underwent stereotactic biopsy. Some of them were treated with drugs after pathological examination. Among them, 6 cases did not consider demyelinating lesions preoperatively. Results MRI showed single lesion in 8 cases, multiple lesions in 2 cases, tumor diameter of 20 ~ 54 mm; 10 patients had mild to moderate peripheral edema; 8 patients with brain back, 2 patients underwent MRS examination showed NAA decreased, CHO slightly reduced, inverted LAC peak; 4 cases of DWI showed low signal in the middle of the lesion, the surrounding high signal; 6 cases of lesion MRI enhanced part of the CT For low density shadow. The pathological features of the disease is the demyelination of nerve fibers, and axonal and nerve fibers remain intact. Hormone therapy was still used in patients with acute phase after operation. Two patients had aggravated extremity dysfunction after operation. One patient had a nominal aphasia after operation and the symptoms of other patients were significantly improved. All patients were followed up for 3 to 84 months by telephone or outpatient, with an average of 34 months. Eight patients showed significant improvement or complete recovery of symptoms. One of the lesions showed no significant change and the others contracted or disappeared. The other two patients recovered from symptoms Appeared, and there are mental symptoms, after medical diagnosis of multiple sclerosis (multiple sclerosis, MS). Conclusion The clinical manifestations of intracranial aneurysmal demyelinating disease without characteristic and imaging atypical is the leading cause of preoperative diagnosis difficult. Pathological examination is the most reliable method of diagnosis of the disease, and sometimes need to use special staining with low-grade tumor phase identification. The vast majority of patients treated with steroid hormone have a better prognosis and in rare cases combined with other treatment regimens.