表现为进行性认知功能减退和白质脑病的原发性中枢神经系统淋巴瘤一例

A case of primary central nervous system lymphoma presenting with progressive cognitive impairment and leukoencephalopathy

目的提高对原发中枢神经系统淋巴瘤(PCNL)的临床影像学及其少见表现的认识,有利于早期诊断。方法分析1例PCNL的患者,其临床主要表现为头痛、进行性认知功能减退和症状性癫痫,影像学特点是以白质受累为主,病理证实为弥漫性大B细胞淋巴瘤。结果本例患者亚急性病程,逐渐进展,激素治疗无效。头颅MRI显示双侧侧脑室后角旁和透明隔长T1,长T2异常信号,可见小片状强化。与典型PCNL的影像学表现不同。也不符合典型的脑淋巴瘤病的影像学表现。结论白质脑病的鉴别诊断应考虑PCNL的可能。激素治疗无效不能排除PCNL。

Objective To improve the recognition of clinical and neuroimaging findings and rare presentation of primary central nervous system lymphoma（PCNL）.Methods Analyzing one case of PCNL,which presented with headache,progressive cognitive impairment and symptomatic epilepsy,leukoencephalopathy on MRI,neoplastic lymphoid cells with B-cell lineage on brain biopsy.Result This patient with subacute onset and progressive course was unresponsive to steroid treatment.MRI showed bilateral paraventricular abnormal signal with partial enhancement which is different from typical PCNL or lymphomatosis cerebri.Conclusion The differential diagnosis of leukoencephalopathy should take PCNL into account,and PCNL shouldn＇t be excluded when steroid treatment is ineffective.