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目的报道1例以进行性脑萎缩和颅内多发病变起病的原发性中枢神经系统淋巴瘤。方法收集1例经病理确诊为原发性中枢神经系统淋巴瘤患者的病史及体征、实验室检查、影像学检查、颅内立体定向穿刺活检病理结果,并结合文献复习进行分析。结果患者男性,29岁,因“进行性四肢震颤伴反应迟钝2年,双下肢无力1个月”入院。入院前MRI提示进行性加重的脑萎缩及颅内皮质下、侧脑室旁、基底节多发长T1长T2病灶。外院予甲泼尼龙冲击治疗,2个月后复查头颅MRI示原病灶缓解,左顶叶新发长T1短T2病灶,有占位效应。穿刺活检病理证实左顶叶病灶为弥漫大B细胞淋巴瘤。结论非占位性白质病变及脑萎缩在原发性中枢神经系统淋巴瘤中鲜见,肿瘤实质病灶出现前,其临床特点、影像学及激素治疗效果往往与脱髓鞘病变难以鉴别。因此临床医生需结合病史及影像学检查综合考量诊断,合理选择检查与治疗时机,重视临床随访。
Objective To report a case of primary central nervous system lymphoma with progressive brain atrophy and multiple intracranial lesions. Methods One patient with pathologically diagnosed primary central nervous system lymphoma was enrolled in the study. The history and signs, laboratory tests, imaging findings and intracranial stereotactic biopsy results were collected and analyzed according to the literature review. Results The patient was male, 29 years old, “admitted to the hospital because of” limb tremor accompanied by unresponsiveness for 2 years and weakness in both lower limbs for 1 month. " Pre-admission MRI prompted progressive worsening of brain atrophy and cerebral cortex, lateral ventricle, basal ganglia multiple long T1 long T2 lesions. Outside the hospital to treat the impact of methylprednisolone, 2 months after the review of cranial MRI showed the original lesion relief, the new left long parietal T1 short T2 lesions, with mass effect. Biopsy biopsy confirmed that the left parietal lobe lesions were diffuse large B cell lymphoma. Conclusion Non-occupying white matter lesions and brain atrophy are rare in patients with primary central nervous system lymphoma. Before the appearance of tumor parenchyma, the clinical features, imaging and hormone therapy effects are often difficult to identify with demyelinating lesions. Therefore, clinicians need to combine medical history and imaging examination comprehensive consideration of diagnosis, a reasonable choice of examination and treatment timing, attention to clinical follow-up.