以进行性脑萎缩及非占位性脑白质病变起病的原发性中枢神经系统淋巴瘤(附1例报道及文献复习)

A Case Report and Review of Primary Central Nervous System Lymphoma Preceded by Multifocal White Matter Lesions and Brain Atrophy

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。

Aim To discuss clinical, radiological and pathological of primary CNS lymphoma（PCNSL） accompanied by sentinel lesions and brain atrophy and review the literature retrospectively. Methods The clinical manifestations, laboratory tests results, brain MRI and intracranial lesion pathology of a Chinese patient with PCNSL accompanied by sentinel lesions and brain atrophy were collected and reviewed. Results A 29-year old male complained of progressive tremor and cognitive impairment for 2 years and weakness in both legs for 1 month. Previous brain MRI revealed progressive multiple white matter lesionsin superficial locations, periventricular white matter and basal ganglia and brain atrophy. Treatment of methylprednisolone led to radiologic remission of previous lesions but a new space occupying lesion in his left parietal lobe which was proved to be diffuse large B cell lymphoma. Conclusion Sentinel lesions and brain atrophy are rare presentations of primary CNS lymphoma. Sometimes the clinical features, radiological appearance and response to corticosteroids are indistinguishable from multiple sclerosis. It is suggested that this pattern of PCNSL should be paid attention and followed up.